The Aestheticians Journal Oct'23 issue

Procedural Dermatology: My Experiences

Tofacitinib for the Treatment of Alopecia Totalis: A Case Presentation

Dermatoscopy of Cutaneous

Amyloidosis: A Case Report

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Published for the period of October - 2023

Wrinkles and Skin Laxity

Wrinkles and skin laxity are common concerns related to the aging process and the overall health and condition of the skin. The appearance of the skin is a reflection of the inner health and wellbeing. With time, skin loses its volume, elasticity and firmness, leading to the development of fine lines, wrinkles and sagging skin. The natural aging process, combined with variety of factors like sun exposure, stress, smoking, poor nutrition, genetics and lifestyle habits, can take a toll on the skin. Additionally, visible marks, whether from acne, accidents or procedures, can also impact the overall appearance of the skin and make feel self-conscious.

The natural aging process is one of the primary causes of wrinkles and skin laxity. As we get older, the skin produces less collagen and elastin, which are proteins that give the skin its elasticity and firmness. This leads to the formation of wrinkles and sagging skin. Excessive exposure to ultraviolet radiation from the sun can accelerate the aging of the skin and lead to the development of wrinkles. UV rays damage collagen fibers and elastin, causing the skin to lose its ability to bounce back. A diet lacking in essential nutrients, particularly antioxidants and vitamins, can negatively impact skin health and contribute to premature aging. Many other factors indirectly can contribute to skin aging and the development of wrinkles.

Dermatologists are always ready to address and help to improve the appearance of individual’s skin wrinkles and skin laxity with using various preventive measures and treatment options like non-invasive treatments, cosmetic procedures or with skincare products containing antioxidants, retinoids and moisturizers.

In this issue we have clinical case presentations on Tofacitinib for Alopecia Totalis, Procedural Dermatology, Dermatoscopy of Cutaneous Amyloidosis.

HOPE YOU HAVE A GREAT READ

Thanks & Cheers

- Dom Daniel Executive Editor & Publisher

Procedural Dermatology: My Experiences

Dr. Prerna, M.D. (Dermatology)

Tofacitinib for the Treatment of Alopecia Totalis: A Case Presentation

Dr. Stuti Vyas, MBBS, DDV

Dermatoscopy of Cutaneous Amyloidosis: A Case Report

Dr. Logamoorthy Ramamoorthy, MBBS, MD, DNB

08

twin studies shown concordancerate 55%, indicating a significant genetic influence on developmentofthecondition whereas familial studies forshowsfamilyhistoryispositive about20% patientswith alopeciatotalis. Genome-wide association studieshaveidentifiedcertain human leukocyte antigen (HLA) genes associated with the development of alopecia areata and its subtypes. HLA-DRB1 polymorphisms, particularly HLA-DRB104 and HLA-DRB116, have 08 20 28

can be present differentsub-types Oct

e.g. macular amyloidosis (MA), lichen amyloidosis (LA) and nodular amyloidosis (NA). Macular amyloidosis is most common subtype primary cutaneous amyloidosis. typically presents flat, brownish macules on the upper back, shoulders and upper chest which may itchy and show rippled reticulated pattern. Lichen amyloidosis small, itchy, raised papules often appear on the shins, lower legs and other sites and may have lichenified (thickened and rough) surface that can form linear grouped patterns. Nodularamyloidosisisarare form of primary cutaneous amyloidosis that presents as nodules tumors the skin which firm, raised and vary size can occur anypartofthebody may be associated withsystemicamyloidosisinsome Procedural Dermatology: My ExperiencesDr.PrernaM.D. Fellow(Dermatology) inAesthetics,LasersandDermatosurgery Dermatologist Purnia,Bihar SummaryThe dermatologicaladvancesprocedures have been very promising last two decades. The demand dermatologicalbothconventional surgeries andminimallyinvasiveprocedures havebeenincreasing.Energy based devices, cryosprays, botulunium toxins, fillers, threads,radiofrequencyhave added ourarmamentarium.Introduction Procedural dermatology is a specialized field within dermatology that focuses on the diagnosis, treatment, and management of skin conditions through various minimally invasiveproceduresandtechniques. encompassesawiderangeof procedures,includingbut limited to surgical, cosmetic, and laser interventions. The field procedural dermatologyhasexperienced significant advancements over the past decades, leading to promising outcomes for patients. Thedemandforbothconventional dermatological surgeries andhasminimallyinvasiveprocedures been on the rise, driven

Dermatology: Experiences byfactorssuchasincreased awareness, technologicaladvancementsand desire foreffective lessinvasive aretreatments.Theseprocedures typically performed in an outpatient setting, providing patients with convenience shorter recovery times compared to traditional surgical interventions. The introduction of energy-based devices, such as lasers, intense pulsed light (IPL), and radiofrequency devices, has revolutionized the waydermatologicalconditionsare treated. These devices precise targeting of specific skin concerns, including hair removal, pigmentation disorders, vascular lesions, acne scars, and signs of aging. They provide effective results minimaldowntime and fewer risks compared traditional surgical approaches. Procedural dermatology requires understandingcomprehensive dermatologic conditions, excellent clinical skills, and meticulous approach to procedural techniques.

Editorial Board

Dr. Prerna

M.D. (Dermatology)

Fellow in Aesthetics, Lasers and Dermatosurgery

Dermatologist

Purnia, Bihar

Dr. Logamoorthy Ramamoorthy

MBBS, MD, DNB

Senior Resident

Department of Dermatology and Sexually Transmitted Diseases

Jawaharlal Institute of Postgraduate Medical Education and Research (JIPMER), Puducherry, India

Dr. Stuti Vyas

MBBS, DDV

Dermatologist and Cosmetologist Ahmedabad

Dr. Siva Elango Rajamani

Junior Resident

Department of Dermatology and Sexually Transmitted Diseases

Jawaharlal Institute of Postgraduate Medical Education and Research (JIPMER), Puducherry, India

Advisory Board

Dr. Ashwini

Dr. Rajendran R.

Dr. Sankeerth

Dr. Anvitha

Dr. Karthik

Dr. Harshwardhan

Dr. Pradhyumna Bhandari

Dr. Sohandas Shetty

Dr. Nithin S. U.

Dr. Revathi

Dr. Venkatesh V.

Dr. Vinayak

Procedural Dermatology: My Experiences

Dr. Prerna

M.D. (Dermatology)

Fellow in Aesthetics, Lasers and Dermatosurgery

Dermatologist

Purnia, Bihar

Summary

The advances in dermatological procedures have been very promising in last two decades. The demand for both conventional dermatological surgeries and minimally invasive procedures have been increasing. Energy based devices, cryosprays, botulunium toxins, fillers, threads, radiofrequency have added to our armamentarium.

Introduction

Procedural dermatology is a specialized field within dermatology that focuses on the diagnosis, treatment, and management of skin conditions through various minimally invasive procedures and techniques. It encompasses a wide range of procedures, including but not limited to surgical, cosmetic, and laser interventions. The field of procedural dermatology has experienced significant advancements over the past two decades, leading to promising outcomes for patients. The demand for both conventional dermatological surgeries and minimally invasive procedures has been on the rise, driven

by factors such as increased awareness, technological advancements and the desire for effective and less invasive treatments. These procedures are typically performed in an outpatient setting, providing patients with convenience and shorter recovery times compared to traditional surgical interventions. The introduction of energy-based devices, such as lasers, intense pulsed light (IPL), and radiofrequency devices, has revolutionized the way dermatological conditions are treated. These devices offer precise targeting of specific skin concerns, including hair removal, pigmentation disorders, vascular lesions, acne scars, and signs of aging. They provide effective results with minimal downtime and fewer risks compared to traditional surgical approaches.

Procedural dermatology requires a comprehensive understanding of dermatologic conditions, excellent clinical skills, and a meticulous approach to procedural techniques.

Procedural dermatologists often work in collaboration with other specialists, such as dermatopathologists, dermatologic surgeons and cosmetic dermatologists, to provide comprehensive care to their patients. Overall, procedural dermatology plays a crucial role in the diagnosis, treatment and management of various skin conditions, helping patients achieve healthier, functional and aesthetically pleasing skin through minimally invasive procedures. It is always important to completely understand the concerns and questions raised by patients regarding scars, pigmentation issues and their impact on self-esteem and body image. As dermatologists, it is important to address these concerns and provide the best possible solutions to help improve their confidence and overall well-being. By sharing some cases and the positive outcomes achieved through interventional dermatology, you can reassure your patients that there are viable options available to address their concerns. Each patient's situation is unique and a personalized treatment plan tailored to their specific needs and goals is crucial for achieving the best possible results. It is essential to have open and honest discussions with your patients, understanding their expectations and explaining the potential benefits and limitations of the available procedures. By providing compassionate care and utilizing the advancements in interventional dermatology, you can help alleviate their

anxiety, improve their selfesteem and contribute to their overall well-being.

Advancements in interventional dermatology, including the availability of lasers, energy-based devices, cryoguns, chemical peels, hair transplantation and vitiligo surgeries, have indeed brought about significant changes in the field. These procedures offer new options for addressing various dermatological concerns and have the potential to greatly improve patients' quality of life.

Here are a few examples of cases where interventional procedures have made a positive impact:

Scar revision: Scars can result from various causes, including injuries, surgeries or skin conditions. Interventional procedures such as laser treatments, microneedling, dermal fillers and scar revision surgeries can help improve the appearance of scars, minimize their visibility, and restore a more natural skin texture.

Pigmentation disorders: Pigmentation concerns like melasma, post-inflammatory hyperpigmentation and age spots can significantly affect a person's self-esteem. Dermatologists can utilize interventions such as chemical peels, laser treatments (such as Q-switched lasers) and topical medications to reduce pigmentation and achieve a more even skin tone.

Hair transplantation : Hair loss, whether due to genetics, hormonal changes,

or other factors, can have a significant impact on self-esteem and body image. Hair transplantation techniques, such as follicular unit transplantation (FUT) or follicular unit extraction (FUE), offer a long-term solution for hair restoration and can greatly enhance a patient's self-confidence.

Vitiligo surgeries: Vitiligo, a condition characterized by the loss of skin pigmentation, can be emotionally distressing for those affected. Surgical interventions like punch grafting, suction blister grafting and melanocyte transplantation can help repigment the affected areas and improve the appearance of vitiligo.

Doctor shall I have to live with this scar for my whole life? Doctor is there any solution for this pigmentation? These are the common questions asked in my daily OPD. Any scar, any minimal disfigurement, pigmentation can cause low self esteem and anxiety. We dermatologists are expected to deliver the best to allay their anxiety. Earlier a conservative approach was favoured because of limited availability and experience of interventional procedures. But the availability of lasers, energy based devices, cryoguns, chemical peels, hair transplantation; vitiligo surgeries have brought a landmark change in interventional dermatology. I am sharing here few cases who were disappointed with the medical treatment and were suffering from negative body image and low self esteem.

Case 1

A 26 year old girl presented to me with lentigens all over her face for last 14 years. She was a professional and independent but was socially withdrawn. She had received lots of medical treatments over the years but had lost all the hope to get better. She was recommended Q switch Nd:YAG laser toning.

Case 2

After 4 sessions of laser toning

A young girl presented with severe grade 3 pustular acne and acne excoriae. Her hobby was singing and she used to give live performances. But she stopped giving all the performances since the onset of acne. At my clinic, she underwent two sessions of black peel at one month interval along with systemic and oral medications.

Before procedure

Before procedure

Case 3

A 18 year old boy presented with unremitting acne and post acne pigmentation. He was in 12th standard. His scholastic performance poorly dropped since the onset of acne and pigmentation. He had received treatments but it did not help him much. He was recommended black peel at my clinic.

After

Before

After 2 sessions of black peel

procedure

2 sessions of black peel

Case 4

A young girl of marriageable age was socially stigmatized since the onset of blotchy pigmentation all over her face. She had severe post inflammatory hyperpigmentation. She underwent one session of Q switch Nd:YAG laser toning.

Before procedure

After 1 session of Q switch laser toning

After 3 sessions of laser toning

Case 5

A 28 year old girl from a humble family was distraught with resistant non responding melasma, acneform eruptions and folliculitis. She had one session of black peel followed by a session of laser toning. Her folliculitis and acneform lesions resolved and there was an improvement noted in her skin texture. For her melasma, she had two sessions of combination peels.

Before procedure

After 1 session of laser toning and 3 sessions of chemical peel

Case 6

A 27 year old woman presented to me with recalcitrant acne and post inflammatory hyperpigmentation. She had received lots of oral and topical treatment from other doctors. She had lost all hopes of getting better. She was advised laser toning at monthly intervals. With each session, we noted a reduction in acne and reduction in pigmentation.

Before procedure

After procedure

Case 7

A 35 year old woman presented with a giant Café Au Lait mcaule extending from her neck to right cheek. She was advised to undergo laser toning. There was a marked reduction in pigmentation after one session of toning.

Before procedure

After procedure Discussion

There are numerous cases who benefited with these minimal procedures. Apart from the above mentioned cases, there are some amazing cases of resistant keloids and hypertrophic scars which responded to intralesional radiofrequency. The patients with hypertrichosis had their confidence back after having sessions with laser hair reduction. There are some vitiligo patients who were non responders to all types of medical management were benefited with excimer lamp. Procedural dermatology has indeed become an integral and essential part of dermatology including both surgical and non-surgical methods, reflecting the diverse approaches of procedures and techniques aimed at diagnosing, treating and improving various skin, hair, nail, and mucous membrane conditions. It encompasses the utilization of energy-based devices, minimally invasive cosmetic procedures (like botulinum toxin injections and fillers) and other non-surgical and semi-surgical techniques. Procedural dermatology has significantly advanced

in recent years. The minimal procedures in procedural dermatology offers a wide range of treatment options that have shown effectiveness in addressing various skin conditions, including resistant keloids, hypertrophic scars, hypertrichosis and vitiligo. Some notable procedures are; intralesional radiofrequency for keloids and hypertrophic scars, were controlled heat energy is delivered to the scar tissue which promotes collagen remodeling and helps flatten the scar tissue. Since keloids and hypertrophic scars can be challenging to treat, particularly when they are resistant to conventional therapies this is a promising method in reducing the size and improving the appearance of such scars. Further laser hair reduction is used for hypertrichosis (excessive hair growth) that utilizes specific wavelengths of light to target and destroy hair follicles and offers a longterm solution for reducing unwanted hair. Another method used is excimer lamp for vitiligo which emits narrow band ultraviolet B (NB-UVB)

light, that helps to stimulate melanocyte activity leading in repigmenting vitiligo patches. While medical management options exist, not all patients respond adequately. Patients undergoing such treatments gains self-esteem, improving their physical appearance due to positive impacts of such surgeries. However there are unmet needs in this field showing challenges and opportunities for improvement. One of the primary unmet needs is equitable access to these procedures as some advanced procedures may be costly, making them inaccessible to individuals with limited financial resources or inadequate insurance coverage. The increase affordability and accessibility, such as through insurance coverage expansion or innovative pricing models, can help to address this issue. Specialized training and expertise is required for the growing demand. Building a stronger evidence base will enhance patient outcomes and guide clinical decision-making which can be done by research and clinical studies which are needed to establish robust evidence-based guidelines. This includes comparative studies, long-term outcome assessments, and investigations into the optimal techniques, devices, and patient selection for specific procedures. Each patient's condition is unique, requiring personalized treatment plan by consider individual patient factors, including skin type, medical history and treatment

goals, when selecting and performing procedures. Continual efforts should be made to minimize risks and complications through advanced techniques, improved device safety profiles, standardized protocols and ongoing professional development and training. Advancement in technology and innovation is essential that includes the development of new devices, techniques and treatment modalities that further enhance safety, efficacy and patient comfort. By addressing these unmet needs, procedural dermatology can further improve patient outcomes, expand access to care and enhance the overall quality of procedural dermatological treatments. Sharing such cases and experiences helps spread awareness about the potential benefits of these procedures and encourages further exploration and research in the field. It also highlights the importance of personalized treatment approaches and the value of continuous learning and innovation in procedural dermatology.

Some common procedures performed include skin biopsy (removal of a small sample of skin tissue for microscopic examination to diagnose skin conditions, such as skin cancer or inflammatory disorders), excisions to surgically remove skin lesions, including skin cancer, mohs surgery; is a specialized technique for removing skin cancer layer by layer, laser

and light-based therapies to treat a wide range of skin conditions, such as unwanted hair, pigmentation disorders, vascular lesions, scars and wrinkles, cosmetic procedures includes a range of cosmetic treatments, including injectable fillers, neuromodulators (such as botox), chemical peels, microdermabrasion and non-surgical skin tightening procedures, dermatologic surgery to remove benign skin growths, cysts, lipomas, and other dermatologic conditions. Cryosprays and cryotherapy techniques have also contributed to the armamentarium of procedural dermatology which allow the controlled use of extreme cold, typically using liquid nitrogen, to treat various skin conditions, including warts, actinic keratoses and skin cancers. Cryosurgery is a versatile and minimally invasive option for the management of these conditions. Botulinum toxins, such as botox, have gained immense popularity in both cosmetic and therapeutic applications. These toxins work by temporarily relaxing specific muscles, making them an effective treatment for dynamic wrinkles and certain medical conditions such as hyperhidrosis and muscle disorders.

Fillers made of biocompatible materials, such as hyaluronic acid, have become widely used in cosmetic dermatology. They are used to restore volume, enhance facial contours, and minimize the appearance of

wrinkles and scars. Fillers provide immediate results with minimal downtime, making them a popular choice for non-surgical facial rejuvenation. Threads, often referred to as thread lifting or thread rejuvenation, have gained popularity as a minimally invasive alternative to surgical facelifts. These threads, typically made of biodegradable materials, are inserted beneath the skin to lift sagging tissues and improve facial contours. Thread lifting provides a non-surgical option for skin tightening and rejuvenation. Radiofrequency devices and techniques have also made significant contributions to procedural dermatology. They utilize controlled radiofrequency energy to stimulate collagen production and improve skin laxity and texture. Radiofrequency treatments can be used for facial and body contouring, skin tightening and cellulite reduction.

It's important to note that experiences can vary depending on the individual, the specific procedure, and the expertise of the dermatologist. The outcome and recovery time will depend on the procedure performed. Some procedures may show immediate results, while others may require multiple sessions or weeks to see the full effects. Follow-up appointments are often scheduled to monitor progress and address any concerns or complications if any faced.

Conclusion

The World Health Organization (WHO) defines health as “a state of complete physical, mental and social well-being and not merely the absence of disease and infirmity.” So this implies that ideally, to be healthy, patients must be free of mental and social distress which results from diseases leading to disfigurement. The lack of beauty and attractiveness is associated with a significant social disadvantage. Such patients undergo a remodelling of their emotional state and are more prone to depression and anxiety. This creates strain in social interactions and avoidant behaviour. Minimally invasive cosmetic procedures can improve quality of life and body image and reduce psychological distress and anxiety. The demand for both conventional dermatologic surgeries and minimally invasive cosmetic procedures is increasing. However, the supply of trained professionals with expertise in these procedures is limited. This has led to a situation where unqualified practitioners may attempt procedures, leading to complications and poor outcomes. Waiting lists for procedures like vitiligo surgeries can become lengthy, delaying necessary interventions for patients. One of the challenges in procedural dermatology is the lack of good quality research, resulting in a lack of standardization and evidencebased guidelines for various procedures. This can lead to variations in practice and a lack

of understanding regarding the efficacy and safety of different drug-procedure and procedure-procedure combinations. Additionally, the public may be exposed to costly procedures without sufficient evidence of their efficacy. For such issues it is important to have an individual institutional and broad policy directives, emphasis should be placed on formal handson procedural dermatology training during residency and beyond. This will ensure that dermatologists are adequately trained to perform procedures safely and effectively. Research efforts should focus on generating high-quality evidence and developing standardized protocols for different procedures. Meeting the increasing demand for minimally invasive procedures while delivering satisfactory results for patients.

Tofacitinib for the Treatment of Alopecia Totalis: A Case Presentation

Dr. Stuti Vyas MBBS, DDV

Dermatologist and Cosmetologist

Ahmedabad

Introduction

Alopecia totalis (AT) is a subtype of alopecia areata which is an autoimmune condition characterized by the complete loss of hair on the scalp. It is a condition where the immune system mistakenly attacks the hair follicles, leading to hair loss. While the exact cause of alopecia areata is unknown, it is believed to involve a combination of genetic and environmental factors. The evaluation and treatment of alopecia totalis involve several steps. The primary goals of treatment are to promote hair regrowth and address any emotional or psychological impact of hair loss.1

The patients with alopecia totalis have a poorer prognosis for hair regrowth due to the severity and extent of hair loss, as well as the involvement of a larger area of the scalp compared to focal alopecia areata (where hair loss is limited to specific patches) patients, tending to less responsive to treatment interventions. Exceptions can be made patients with alopecia totalis may still experience partial or complete

hair regrowth with appropriate interventions. The immunemediated inflammation in alopecia totalis may cause more profound damage to the hair follicles, leading to a reduced likelihood of regrowth hence early recognition and intervention, timely diagnosis and initiation of appropriate treatment are crucial. Being an autoimmune condition with the unknown etiology, genetic factors plays a crucial role as there is an increased risk of developing alopecia areata if a close family member has the condition. A supported identical twin studies have shown a concordance rate of 55%, indicating a significant genetic influence on the development of the condition whereas familial studies shows family history is positive for about 20% of patients with alopecia totalis.2

Genome-wide association studies have identified certain human leukocyte antigen (HLA) genes associated with the development of alopecia areata and its subtypes. HLA-DRB1 polymorphisms, particularly HLA-DRB104 and HLA-DRB116, have

been found to be associated with increased susceptibility to alopecia areata. HLADQB103 has also been linked to the development of alopecia areata. Some specific polymorphisms, such as HLA-DRB11104 and HLA-DQB1*0301, have been identified to be more highly associated with alopecia areata and AU compared to other forms of alopecia areata. In addition environmental triggers such as stress, infections or certain medications can contribute to flares or relapses of the disease and can lead the onset or exacerbation of alopecia areata, including alopecia totalis. These factors can potentially activate the immune system and lead to an autoimmune response against hair follicles.3

Evaluation can be based on either a thorough medical history to identify any underlying conditions or triggers that may contribute to hair loss which may include questions about recent illnesses, medication use or family history of autoimmune disorders or physical examination of the scalp to assess the extent of hair loss and rule out other possible causes. Scalp biopsy can be done in some cases to confirm the diagnosis and rule out other conditions that may cause hair loss. The individual responses to treatment provided such as topical corticosteroids, topical immunotherapy, systemic corticosteroids, immunomodulatory agents and hair transplantation, can

vary from person to person. The choice of treatment depends on various factors, including the patient's age, overall health, preferences and the extent of hair loss.1,2,4

Case Report

A 17 year old male patient was presented with near complete hair loss on scalp since 6 months in our clinic. Few patches were noticed even when he was 9 years old which recovered by its own. There was no history of similar illness in family members and also no history of any drug. At the age of 17 he started to notice few patches again which was in alopecia ophiasis pattern in the beginning but then increased and involved the full scalp within 2 months. Patient waited and tried ayurvedic remedies but it gave no visible results so patient decided to consult a Dermatologist. Eyebrows and other body hairs were intact. Nails were spared. There was no major illness in recent past.

The patient history stated no diabetes, asthama or any other illness. No history of any other skin related issues. The diagnosis of alopecia totalis was made. Initially the patient was started with low doses oral steroids for 3 months along with topical steroids and tacrolimus. We also tried multiple sessions of micro needling with triamconolone 10mg/ml in a diluted form. Because of no response, we then started cyclosporine 50mg BD for 3 months, But even with that, the recovery was poor. Finally, after thorough investigations which included CBC, HbA1c, LFT, CREATININE, TB GOLD TEST, TSH we started the treatment with tofacitanib 5mg twice a day with monthly follow up. During this time patient was asked to continue local application of fluticasone cream and tacrolimus ointment. Within 2 months hair started growing back and by the end of 6th month patient had recovered almost 90% of the hair back. Patient was on tofacitinib 5mg BD for 6 months and then on once a day dosage.

Tofacitinib for the Treatment of Alopecia Totalis: A Case Presentation

Diagnosis

After 6 months of treatment

The diagnosis of alopecia totalis is primarily based on the medical history and physical examination of patients which are as follows:

Most patients with alopecia totalis will have a history of discrete, patchy hair loss that has progressed to total scalp, only a small percentage of patients with alopecia areata progress to alopecia totalis or alopecia universalis with an average time of about one year and in 90% of patients, it occurs within four years. Initially alopecia totalis is often preceded by multifocal alopecia areata, defined as the presence of five or more discrete patches of hair loss. Comorbid conditions are common in patients with alopecia areata and may be slightly more frequent in those with alopecia totalis and are associated with autoimmune thyroid disease, vitiligo, type I diabetes mellitus, atopy and inflammatory bowel disease.

The hallmark of alopecia totalis is the complete loss of hair on the scalp, affected areas typically appear pale, shiny, smooth and devoid of any hair follicles without any signs of inflammation, scarring or other skin abnormalities. Other areas of the body, such as the eyebrows, eyelashes and body hair,

may also be affected in some individuals.2

Follicular orifices should be visible on the scalp, distinguishing alopecia totalis from scarring alopecia where they are absent. In areas of spontaneous hair regrowth, initially non-pigmented hair may be observed, but repigmentation typically occurs within weeks to months. Nail involvement such as pitting, brittle nails, trachyonychia (rough nails), and onycholysis (nail separation), is common in alopecia areata, particularly in patients with alopecia totalis, should be closely evaluated. With thorough history and comprehensive physical examination essential information to support the diagnosis of alopecia totalis and treatment of alopecia totalis can be provided.1,2

With differential diagnosis the healthcare provider can consider other conditions that can cause hair loss, such as trichotillomania, AGA, scarring alopecia, traction alopecia and non inflammatory tinea capitis. They will evaluate the scalp for signs of scarring or inflammation, which can help differentiate alopecia totalis from other conditions. In some cases, a scalp biopsy may be performed to confirm the diagnosis in case of uncertainty of the diagnosis. The biopsy involves removing a small sample of scalp tissue for examination under a microscope. Laboratory tests are typically not necessary for the diagnosis of alopecia totalis but it should be done to rather rule out an underlying

Tofacitinib for the Treatment of Alopecia Totalis: A Case Presentation

After 2 months of treatment

autoimmune or systemic comorbidity.

Treatment

The treatment of alopecia totalis has to be a combination of oral as well as topical medicines so as to stop the on going inflammatory process. The available treatment options include:

Topical corticosteroids/ topical calcineurin inhibitor/minoxidil: These are commonly used as a firstline medication treatment for alopecia totalis which can be prescribed as a combination therapy.

Systemic corticosteroids: Daily or weekly oral corticosteroids to be prescribed to suppress the immune system specially in fast progressing alopecia areata. However, longterm use of systemic corticosteroids may have significant side effects and is generally reserved for shortterm use.

Systemic calcineurin

Inhibitors : Oral cyclosporine can be tried as a steroid sparing agent.

JAK INHIBITORS : Medications like JAK inhibitors, such as tofacitinib or baricitinib, have shown promising result in treating alopecia areata in recent past. These drugs target specific immune pathways involved in the disease process.1,2

Tofacitinib is an oral Janus kinase (JAK) inhibitor that has shown promise result in the management of alopecia areata, including its more extensive forms like alopecia

totalis (AT) and alopecia universalis (AU). Here's an overview of the role of tofacitinib in the management of alopecia totalis:

Mechanism of Action:

Tofacitinib inhibits certain JAK enzymes, including JAK1 and JAK3, which are involved in the signaling pathways of various pro-inflammatory cytokines. By blocking these pathways, tofacitinib helps modulate the immune response involved in the pathogenesis of alopecia areata. Several clinical studies, case reports, case series and open-label studies have reported positive outcomes as significant hair regrowth in a substantial proportion of patients with tofacitinib in the treatment of alopecia areata, including alopecia totalis, even in cases that were unresponsive to other treatment modalities.

Dosage and Duration:

The optimal dosage of tofacitinib for the treatment of alopecia areata is still being investigated. However, most studies have used a dosage of 5 mg twice daily. The duration of treatment varies among individuals and may need to be continued for an extended period to maintain hair regrowth. Tofacitinib is associated with potential side effects, including an increased risk of opportunistic infections, liver function abnormalities, and reactivation of latent tuberculosis. Thorough investigations like TB GOLD TEST, blood counts, liver function tests are recommended before and during the treatment with tofacitinib. Since tofacitinib

has shown promising results in the management of alopecia totalis, it is important to consider the potential risks and benefits on an individual basis and hence the longterm safety and efficacy of tofacitinib for alopecia areata, including alopecia totalis, are still being studied and further research is needed to establish its place in therapy.

Thorough discussion with their healthcare provider regarding the potential benefits against the risks and considering individual factors such as the extent of hair loss, treatment history and comorbidities is essential for patients considering tofacitinib or any other systemic treatment for alopecia totalis.8,9, 10,11,12,13

Given the potential risks and the need for monitoring, the use of tofacitinib in the management of alopecia totalis requires an inter professional team approach. Dermatologists along with other specialists like chest physicians should collaborate to assess the suitability of treatment, monitor patients for side effects and adjust the treatment plan as necessary. Key aspects of collaboration include:

Accurate Diagnosis : Dermatologists play a central role in diagnosing alopecia totalis through physical examination, medical history, and possibly scalp biopsies. Effective communication and sharing of information among team members ensure accurate diagnosis and appropriate treatment planning.

Tofacitinib for the Treatment of Alopecia Totalis: A Case Presentation

Treatment Selection and Monitoring: Collaborative decision-making involving dermatologists, primary care physicians and other specialists are important in determining the most suitable treatment options for each patient. Regular monitoring of treatment progress and potential side effects is essential.

Psychological Support : Mental health specialists can provide emotional support, counselling and guidance to individuals with alopecia totalis, helping them cope with the psycho-social impact of hair loss. Collaborating with mental health professionals ensures a holistic approach to patient care.

Education and Patient Empowerment: The inter professional team can work together to educate patients about the condition, treatment options and selfcare strategies. Empowering patients with knowledge helps them actively participate in their own care and make informed decisions.

Long-term Follow-up : Collaborative care ensures that patients receive regular follow-up visits, allowing for continuous evaluation of treatment outcomes, management of potential relapses and adjustments to the treatment plan when needed.

By fostering collaboration and effective communication, the inter professional team can provide comprehensive care to patients with alopecia totalis, leading to improved

outcomes and a better quality of life for those affected by this condition.1,2,14

Discussion

Tofacitinib, although approved for rheumatoid arthritis (RA), has shown promise in the treatment of alopecia areata, including its more extensive forms like alopecia totalis and alopecia universalis (AU).

The use of tofacitinib for the treatment of alopecia totalis represents an off-label use, as it has not received regulatory approval specifically for this condition. The study mentioned also revealed that 6 out of 20 patients experienced interruptions in medication usage in which four of these were due to adverse effects, while the other two were caused by lapses in prescription insurance coverage. These challenges highlight the obstacles and potential barriers patients may encounter when trying to access and maintain treatment with tofacitinib for alopecia totalis. The study also reported that the durability of response among the patients treated was shorter than the 10 to 12 weeks reported in other studies. Hair shedding occurred approximately 4 to 5 weeks after discontinuation of the medication, indicating that a relatively short period of time may be sufficient for the inflammatory pathways involved in alopecia areata to reestablish themselves following the cessation of tofacitinib. Nonetheless, the results of this study, along with previous research, suggest that JAK inhibition, such as with tofacitinib, holds

a promise as an effective treatment option for alopecia areata and its more extensive forms like alopecia totalis. Further research and clinical trials will help provide more comprehensive evidence regarding the benefits, risks, and optimal usage of JAK inhibitors in the management of alopecia areata.8,10,15,16,17

The observation that the majority of patients experienced hair regrowth, ranging from less than 5%100%, during the study is consistent with previous research findings. It suggests that the response to treatment with tofacitinib in alopecia totalis may not be solely dependent on the duration of the disease but rather on underlying pathophysiological mechanisms. It is seen that the hair regrown in some patients differ from their natural hair color and texture which is not uncommon in alopecia areata and its subtypes. This awareness regarding the potential hair growth difference has to be brought to the patients. Additionally a study mentioned a patient demonstrated the regrowth of hair resembling female androgenetic alopecia, which is a pattern typically observed in males. This shows the hair growth complexity in alopecia totalis patients and emphasizes need for individualized assessment and treatment approaches for each patient.

Overall, the response to treatment with tofacitinib in alopecia totalis can lead to significant hair regrowth in many patients, regardless of

disease duration. However, variations in regrowth characteristics, such as color and texture, should be anticipated and individualized management is necessary to address the specific needs and expectations of each patients. Tofacitinib, although approved for rheumatoid arthritis, has shown promise in the treatment of alopecia areata, including its more extensive forms like alopecia totalis and alopecia universalis (AU).8,15,18,19,20,21

Conclusion

In conclusion, alopecia totalis is a chronic condition characterized by complete hair loss of the scalp. The evaluation of alopecia totalis should involve thorough medical history and physical examination. Treatment options for alopecia totalis are limited, and the prognosis for spontaneous hair regrowth is generally poor. However, emerging therapies such as tofacitinib, a Janus kinase (JAK) inhibitor, have shown promising results and gives potential treatment resulting in promoting hair regrowth in some patients with alopecia totalis. Tofacitinib is an offlabel treatment option that requires careful consideration of the potential risks and benefits, as well as the challenges associated with insurance coverage and monitoring.

However, despite these hurdles, many patients have experienced significant hair regrowth with tofacitinib treatment. It is important to note that the response to tofacitinib can vary among

Tofacitinib for the Treatment of Alopecia Totalis: A Case Presentation

individuals, with some experiencing partial regrowth and others achieving nearcomplete regrowth. The regrown hair may also differ in color and texture from the patient's natural hair. Also note that chances of recurrence are present after stopping the medicines. The management of alopecia totalis requires an inter professional team approach. Primary care providers play a crucial role in recognizing the condition and referring patients to dermatologists for further evaluation and treatment options. Collaboration with dermatologists, therapists, and psychiatrists may be necessary to provide comprehensive care, support patients emotionally, to address the psychological distress often associated with extensive hair loss and explore emerging therapies to enhance the management of alopecia totalis and improve patient outcomes.

References

1. Kassira S, Korta DZ, Chapman LW, Dann F. Review of treatment for alopecia totalis and alopecia universalis. Int J Dermatol. 2017 Aug;56(8):801810. doi: 10.1111/ ijd.13612. Epub 2017 Apr 5. PMID: 28378336.

2. Abbott J, Rapini RP. Totalis Alopecia. [Updated 2023 Feb 5]. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2023 Jan-. Available from: https://www.ncbi.nlm. nih.gov/books / NBK563225/

3. Jabbari, Ali et al. “Genetic basis of alopecia areata: a roadmap for translational research.” Dermatologic clinics vol. 31,1 (2013): 109-17. doi:10.1016/j.det.2012.08.014

4. Gupta, Sumit; Bisht, Priyanka Borde; Kannan, Charisma. Alopecia Totalis Successfully Treated with Modified Platelet-Rich Plasma Therapy in a Patient Recalcitrant to Traditional Treatment Modalities. Clinical Dermatology Review 5(1):p 120-122, Jan–Jun 2021. | DOI: 10.4103/CDR.CDR_50_19

5. Andrew G Messenger, Patient education: Alopecia areata (Beyond the Basics). May 2023.

6. Asz-Sigall, Daniel et al. “Differential Diagnosis of Female-Pattern Hair Loss.” Skin appendage disorders vol. 2,1-2 (2016): 18-21. doi:10.1159/000445806

7. Mounsey AL, Reed SW. Diagnosing and treating hair loss. Am Fam Physician. 2009 Aug 15;80(4):356-62. PMID: 19678603.

8. Hogan, Sara et al. “LONG-TERM TREATMENT WITH TOFACITINIB IN SEVERE ALOPECIA AREATA: AN UPDATE.” The Journal of clinical and aesthetic dermatology vol. 12,6 (2019): 12-14.

9. Liu, L. Y., & King, B. A. (2018). Tofacitinib for the Treatment of Severe Alopecia Areata in Adults and Adolescents. Journal of Investigative Dermatology Symposium Proceedings, 19(1), S18–S20. doi:10.1016/j. jisp.2017.10.003

10. Mariana Esteves, Sofia Lopes, Filomena Azevedo, Ana Pedrosa; Effectiveness of Oral Tofacitinib Dose Tapering in a Case of Alopecia Areata Universalis. Skin Appendage Disord 19 January 2021; 7 (1): 36–40. https://doi. org/10.1159/000510673

11. Yu L, Yu H, Zhang S, Hao Y and Zhang S (2022) Case Report: Successful Treatment of Alopecia Universalis With Tofacitinib and Increased Cytokine Levels: Normal Therapeutic Reaction or Danger Signal? Front. Immunol. 13:904156. doi: 10.3389/ fimmu.2022.904156

12. Alkhalifah A, Alsantali A, Wang

Tofacitinib for the Treatment of Alopecia Totalis: A Case Presentation

E, McElwee KJ, Shapiro J. Alopecia areata update: part I. Clinical picture, histopathology, and pathogenesis. J Am Acad Dermatol. 2010 Feb;62(2):17788, quiz 189-90. doi: 10.1016/j. jaad.2009.10.032. PMID: 20115945.

13. Liu, L. Y., Craiglow, B. G., & King, B. A. (2018). Tofacitinib 2% ointment, a topical Janus kinase inhibitor, for the treatment of alopecia areata: A pilot study of 10 patients. Journal of the American Academy of Dermatology, 78(2), 403–404.e1. doi:10.1016/j. jaad.2017.10.043

14. Lepe K and Zito P M. Alopecia Areata. March 7, 2023.

15. Sánchez-Díaz M et al. Tofacitinib for Treatment of Alopecia Areata: Realworld Evidence and Factors Associated with Therapeutic Response. Acta Derm Venereol 2022; 102: adv00736. DOI: 10.2340/actadv.v102.2036

16. Zhang Wenxin Z, Li Xiangqian, Chen Baifu, Zhang Jianzhong, Torres-Culala Kara Melissa T., Zhou Cheng. Oral Tofacitinib and Systemic Corticosteroids, Alone or in Combination, in Patients With Moderate-to-Severe Alopecia Areata: A Retrospective Study. Frontiers in Medicine, VOLUME=9, YEAR=2022, DOI=10.3389/fmed.2022.891434 URL=https://www.frontiersin.org/ articles/ 10.3389/ fmed.2022.891434

17. Ibrahim O, Bayart CB, Hogan S, Piliang M, Bergfeld WF. Treatment of Alopecia Areata With Tofacitinib. JAMA Dermatol. 2017;153(6):600–602. doi:10.1001/jamadermatol.2017.0001

18. Jabbari, A., Sansaricq, F., Cerise, J., Chen, J. C., Bitterman, A., Ulerio, G., … Mackay-Wiggan, J. (2018). An OpenLabel Pilot Study to Evaluate the Efficacy of Tofacitinib in Moderate to Severe Patch-Type Alopecia Areata, Totalis, and Universalis. Journal of Investigative Dermatology, 138(7), 1539–1545. doi:10.1016/j.jid.2018.01.032

19. Liu, L. Y., Craiglow, B. G., & King, B. A. Tofacitinib for the treatment of severe alopecia areata and variants: A study of 90 patients. J Am Acad Dermatol. 2017;76(1):22. Epub 2016 Nov 2.

20. Dai YX, Yeh CP, Chen CC. Efficacy and safety of tofacitinib therapy in Asian patients with severe alopecia areata. Dermatol Sin 2020;38:3-8

21. Florian Anzengruber, Julia-Tatjana Maul, Jivko Kamarachev, Ralph M. Trüeb, Lars E. French, Alexander A. Navarini; Transient Efficacy of Tofacitinib in Alopecia Areata Universalis. Case Rep Dermatol 28 April 2016; 8 (1): 102–106. https://doi.org/10.1159/000445182

Dermatoscopy of Cutaneous Amyloidosis: A Case Report

Dr. Logamoorthy Ramamoorthy

MBBS, MD, DNB

Senior Resident

Department of Dermatology and Sexually Transmitted Diseases

Jawaharlal Institute of Postgraduate Medical Education and Research (JIPMER), Puducherry, India

Dr. Siva Elango Rajamani

Junior Resident

Department of Dermatology and Sexually Transmitted Diseases

Jawaharlal Institute of Postgraduate Medical Education and Research (JIPMER), Puducherry, India

Introduction

Cutaneous amyloidosis is a group of rare disorders characterized by the deposition of amyloid protein in the skin. Amyloidosis is a term used for diseases caused by the extracellular accumulation of insoluble polymeric protein fibrils in various tissues and organs, leading to the impairment of their normal function. In cutaneous amyloidosis, the amyloid deposits specifically affect the skin, without involvement of internal organs. There are several subtypes of cutaneous amyloidosis, including primary localized cutaneous amyloidosis (PLCA), secondary cutaneous amyloidosis and familial/genetic forms. The most common form of cutaneous amyloidosis is primary localized cutaneous amyloidosis which can be present in different sub-types

e.g. macular amyloidosis (MA), lichen amyloidosis (LA) and nodular amyloidosis (NA). Macular amyloidosis is the most common subtype of primary cutaneous amyloidosis. It typically presents as flat, brownish macules on the upper back, shoulders and upper chest which may be itchy and show a rippled or reticulated pattern. Lichen amyloidosis are small, itchy, raised papules that often appear on the shins, lower legs and other sites and may have a lichenified (thickened and rough) surface that can form linear or grouped patterns. Nodular amyloidosis is a rare form of primary cutaneous amyloidosis that presents as nodules or tumors in the skin which are firm, raised and can vary in size and can occur on any part of the body or may be associated with systemic amyloidosis in some

cases. In addition to these three main subtypes, other variants of primary cutaneous amyloidosis include bullous, poikilodermic, vitiliginous and anosacral types which have distinct clinical presentations and may involve specific anatomical regions or have additional features such as blistering or depigmentation. The patients can present with a combination of macular and papular lesions, leading to the term "biphasic amyloidosis." Unknown being the cause it is assumed to occur sporadically or be associated with certain underlying conditions, such as chronic inflammatory skin diseases, genetic mutations or systemic amyloidosis. It's important to differentiate primary localized cutaneous amyloidosis from systemic forms of amyloidosis, as systemic amyloidosis typically involves multiple organs and can have more severe implications for overall health and organ function. The localized nature of primary cutaneous amyloidosis benefits for a more focused treatment approach, primarily aimed at managing symptoms and improving the quality of life for affected individuals. However, monitoring patients with primary cutaneous amyloidosis for any signs of systemic involvement or progression of the disease is crucial. If there are concerns about systemic involvement or if the diagnosis is unclear a comprehensive evaluation is recommended to ensure appropriate management and follow-up. One of the characteristic features of amyloid deposits is their ability

to exhibit congophilia (affinity for Congo Red staining) and green birefringence under polarized light. Clinically, it can be challenging to differentiate between different subtypes of primary cutaneous amyloidosis.1,2,3,4

Case report

A 59-year-old male was presented with multiple asymptomatic raised lesions

brown dots and globules at the periphery, “hub-andspoke” pattern, a scaling surrounding some white hubs and erosions (Figure 2) [DermLite DL4, contact, polarized, x10]. No clinical history of other diseases is present. Thus a call for final diagnosis of cutaneous lichen amyloidosis was made.

of cutaneous lichen amyloidosis showing central white hubs (blue arrows) with greyish-brown globules/streaks/peppering at the periphery (white arrows), erosion (red arrow), scaling (black arrow) and “hub-andspoke” pattern (yellow circle). [DermLite DL4, contact, polarized, x10].

Diagnosis

Figure 1: Multiple hyperpigmented to skin-colored papules symmetrically distributed over the bilateral lower legs.

Primary localized cutaneous amyloidosis is indeed a relatively rare chronic condition that leads to the deposition of amyloid protein in the dermis of the skin, without associated deposits in internal organs. The presence of amyloid can be confirmed through various diagnostic methods. The diagnosis of cutaneous amyloidosis is confirmed through histopathological examination

Figure 2 (A&B) : Dermatoscopy

of a skin biopsy hematoxylin and eosin (H&E) staining, which reveals the presence of eosinophilic hyaline material in the papillary dermis. This material appears as pink or amorphous deposits under the microscope. Congo red staining of the biopsy specimen is commonly used to detect the amyloid deposits, which exhibit characteristic apple-green birefringence under polarized light. However, it is worth noting that congo red staining may not always detect amyloid in all cases, especially in macular amyloidosis where amyloid deposition is sparse. Direct Immunofluorescence (DIF) testing is another technique were fluorescentlabeled antibodies specific to amyloid proteins are applied to a skin biopsy sample and the presence of amyloid is detected by fluorescence microscopy. Direct immunofluorescence testing for tissue-bound autoantibodies can serve as a useful adjunct in diagnosing primary cutaneous amyloidosis and differentiating it from similar dermatological conditions. Immunohistochemistry is another approach that can be utilized which involves using specific antibodies against amyloid proteins to label and visualize the deposits under a microscope. In direct immunofluorescence, amyloid deposits often show positive fluorescence for immunoglobulins, particularly immunoglobulin M (IgM), or Complement 3 (C3). Additionally, immunohistochemical

findings can confirm the presence of keratin epitopes in the amyloid of lichen amyloidosis and macular amyloidosis. Since identifying and differentiating is quite a challenge, a confirmed diagnosis usually requires a combination of clinical evaluation, histopathological examination of a skin biopsy and ancillary testing such as congo red staining, immunohistochemistry or direct immunofluorescence to identify the presence of amyloid deposits or other specific markers.1,2,3,4

These diagnostic techniques, including congo red staining, direct immunofluorescence testing and immunohistochemistry, are valuable in confirming the presence of amyloid in primary localized cutaneous amyloidosis. They help differentiate this condition from other skin disorders with similar clinical features and provide a more definitive diagnosis.1

Treatment

Treatment options for cutaneous amyloidosis are limited and the focus is mainly on managing symptoms such as itching. Topical corticosteroids, antihistamines and other supportive measures may be employed to alleviate discomfort and improve the patient's quality of life.

Topical corticosteroids, such as hydrocortisone or more potent formulations may be prescribed to reduce inflammation, relieve itching and potentially decrease the

appearance of skin lesions. Oral antihistamines, such as cetirizine or loratadine, blocks the effects of histamine, help to alleviate itching and provide symptomatic relief. Regular use of emollients and moisturizers can help soothe and hydrate the skin, reducing dryness and itching. In some cases, topical capsaicin cream, derived from chili peppers, deplets substance P, a neurotransmitter involved in itch perception thus alleviate itching. Capsaicin works by phototherapy i.e. certain forms of cutaneous amyloidosis, such as macular amyloidosis, may respond to phototherapy. Narrowband ultraviolet B (NB-UVB) phototherapy or psoralen plus ultraviolet A (PUVA) therapy may be considered to reduce itching and improve the appearance of skin lesions. Phototherapy should be administered under medical supervision. Laser treatments, such as pulsed dye laser or carbon dioxide laser, have shown some success in managing itching and improving the appearance of skin lesions in certain types of cutaneous amyloidosis. Dermabrasion, were the top layer of the skin is gently abraded, has been found to be beneficial in lichen amyloidosis, which helps remove thickened and lichenified skin, improving the appearance and symptoms. Systemic medications such as etretinate or cyclophosphamide can be used particularly for more severe or refractory cases. Intralesional injection of corticosteroids may be

Dermatoscopy of Cutaneous Amyloidosis: A Case Report

employed for localized and resistant lesions to help reduce inflammation and promote regression of the amyloid deposits. Nodular amyloidosis has shown a good response to shave removal or curettage, which involves removing the nodules or tumors from the skin surface. This can help improve the appearance and symptoms associated with nodular amyloidosis. The effectiveness of these treatments may vary depending on the subtype and severity of cutaneous amyloidosis. The choice of treatment depends on factors such as the subtype and extent of cutaneous amyloidosis, the individual patient's characteristics and their response to previous treatments.5

Dermatoscopy, also known as dermoscopy or dermatoscopy, is a noninvasive imaging technique that allows for the examination of skin lesions at a magnified level. While dermatoscopy is primarily used for the diagnosis of skin conditions, it can also provide useful information for the aid in the assessment, management, treatment and monitoring of therapeutic interventions and allows the visualization of specific features and changes within the skin lesions that may occur during treatment. These features may include the reduction in pigmentation, improvement in vascularity or changes in the surface texture of the lesions. It provides a non-invasive method for evaluating treatment efficacy and helps guide decisions

regarding the continuation or modification of therapeutic interventions. However, it is important to note that dermatoscopy is not a specific treatment for cutaneous amyloidosis itself.

The choice of treatment depends on the subtype and clinical characteristics of the condition, as well as the individual patient's response. When using dermatoscopy for the diagnosis of cutaneous amyloidosis, several findings may be observed, depending on the specific subtype and characteristics of the lesions. These dermatoscopic features alone may not be sufficient for a definitive diagnosis and should be correlated with clinical presentation and histopathological examination which can aid in distinguishing cutaneous amyloidosis from other skin conditions. Some common features are wickham striae which are fine white lines or linear structures, seen on the surface of the lesions. They can be observed in macular amyloidosis and lichen amyloidosis, diffuse pigmentation (Lesions may display diffuse brownish pigmentation which can be seen in macular amyloidosis and lichen amyloidosis), vascular patterns; dermatoscopy may reveal abnormal vascular patterns, such as linear vessels or dotted vessels, within the lesions. These vascular changes can be seen in macular amyloidosis and lichen amyloidosis. Furthermore in some cases, cutaneous amyloidosis

lesions may exhibit a homogeneous blue-gray pigmentation when observed under dermatoscopy. Dermatoscopy can serve as a helpful adjunctive tool in the diagnosis of cutaneous amyloidosis, but it should be used in conjunction with clinical examination and histopathological evaluation for a definitive diagnosis.5,6,7,8,9

Further research and studies are needed to explore and develop more effective treatment options for cutaneous amyloidosis. A multidisciplinary approach involving dermatologists, rheumatologists and other specialists may be necessary for the comprehensive management of patients with cutaneous amyloidosis.1

Discussion

Localized cutaneous amyloidosis can indeed manifest as macular amyloidosis, lichen amyloidosis and nodular amyloidosis. In some cases, both lichen amyloidosis and macular amyloidosis can coexist in the same patient, leading to biphasic amyloidosis (BA). The exact etiology and pathogenesis of primary cutaneous amyloidosis are still not fully understood. The proposed pathogenesis of primary cutaneous amyloidosis involves the "keratinocyte theory" "keratinocyte apoptosis theory or "filamentous degeneration theory" put forward by Kumakiri and Hashimoto explains that epidermal keratinocytes undergo several stages of filamentous

degeneration, leading to the formation of amyloid. These stages include fibrillar changes in keratinocytes, degeneration of cell organelles with the formation of bundles by tonofilaments, filamentous cells, mass formation and finally the deposition of amyloid. The exact mechanisms underlying the aberrant keratinocyte apoptosis and subsequent amyloid formation are not fully understood. However, it is believed that genetic, environmental and immune factors play a role in the pathogenesis of primary cutaneous amyloidosis. Females are more commonly affected than males, particularly in subtypes like macular amyloidosis where as it has been observed that individuals of Southeast Asian, East Asian or Hispanic descent have more prevalent primary localized cutaneous amyloidosis. Environmental factors like exposure to chronic friction, rubbing or scrubbing of the skin may contribute to the development of primary cutaneous amyloidosis, commonly observed in lichen amyloidosis, where repeated scratching or rubbing of the skin can trigger amyloid deposition. Sun exposure, especially chronic or excessive exposure to ultraviolet (UV) radiation, acts as a potential risk factor for some subtypes, including macular amyloidosis. A possible association between primary cutaneous amyloidosis and atopic conditions, such as atopic dermatitis (eczema) are at a risk of developing

primary cutaneous amyloidosis. Autoimmune diseases, such as systemic lupus erythematosus (SLE) or Sjögren's syndrome, suggests a possible role of immune dysregulation in its pathogenesis. Some wide range clinical manifestations include asymptomatic pigmented macules (in macular amyloidosis), waxy translucent nodules (in nodular amyloidosis), purpura (small bleeding points or purple discoloration due to blood vessel fragility), plaques and bullous lesions (is a rare variant in which blisters or bullae form on the skin which can be fragile and prone to rupture). Understanding the etiopathogenesis of primary cutaneous amyloidosis can provide insights into potential therapeutic targets for the management of this condition. As, primary cutaneous amyloidosis is a common dermatological and cosmetic problem, particularly in the middle-aged population, the most commonly affected sites are the pretibial region for lichen amyloidosis and the interscapular region for macular amyloidosis. The treatment remains challenging and satisfactory results are often difficult due to limited understanding of the etiological factors. It has been observed that many patients with primary cutaneous amyloidosis have a history of scrubbing and while friction may be a contributing factor to it. Microscopic examination of primary cutaneous amyloidosis reveals epidermal changes such as acanthosis, hyperkeratosis,

pigmentation of basal cells and papillomatosis. In both lichen amyloidosis and macular amyloidosis, dermal changes include amyloid deposition in the papillary dermis, lymphohistiocytic inflammatory infiltrate and pigment incontinence. Histopathological analysis, in conjunction with clinical features and patient history, is necessary for an accurate diagnosis, which can help guide appropriate therapeutic interventions. However, further studies are needed to investigate the genetic, personal and cultural factors that contribute to the development of the disease. Advancing our understanding of the etiology and pathogenesis of primary cutaneous amyloidosis, as well as identifying specific genetic and environmental factors, can provide valuable insights into the development of targeted and effective treatment strategies. Continued research efforts are necessary to improve our understanding of this condition and develop more satisfactory therapeutic options for patients with primary cutaneous amyloidosis.4

Conclusion

Dermatoscopy or dermoscopy can provide valuable insights into the diagnosis and management of cutaneous amyloidosis. It allows for the examination of skin lesions at a magnified level, revealing specific dermatoscopic features that can aid in the differentiation of cutaneous amyloidosis from

Dermatoscopy of Cutaneous Amyloidosis: A Case Report

other skin conditions. Some common dermatoscopic findings in cutaneous amyloidosis include wickham striae, diffuse pigmentation, abnormal vascular patterns and homogeneous blue-gray pigmentation. These features, when observed in conjunction with clinical presentation and histopathological examination, can contribute to a more accurate diagnosis. The most common type of cutaneous amyloidosis is primary localized cutaneous amyloidosis which can be present in different sub-types called macular amyloidosis, lichen amyloidosis and nodular amyloidosis. Differentiation amongst these types is important for effective treatment and results. The basic treatment includes use of topical corticosteroids, such as hydrocortisone, oral antihistamines, phototherapy, laser treatments, systemic medications such as etretinate or cyclophosphamide etc can be given. Dermatoscopy is particularly useful in assessing treatment response and monitoring disease progression over time. It allows for the visualization of changes in lesion characteristics, such as pigmentation reduction, improvement in vascularity or changes in surface texture, which can guide treatment decisions and adjustments. However, it is important to note that dermatoscopy should not be used as a standalone diagnostic tool for cutaneous amyloidosis as it is a complementary technique that should be integrated into a comprehensive diagnostic approach. If there

is a suspicion of cutaneous amyloidosis, it is advisable to consult with a dermatologist or healthcare professional experienced in dermatoscopy and the management of this condition. They will be able to utilize dermatoscopy in conjunction with other diagnostic tools to provide an accurate diagnosis and develop an appropriate treatment plan.

References

1. Mehrotra, Krati et al. “Primary Cutaneous Amyloidosis: A Clinical, Histopathological and Immunofluorescence Study.” Journal of clinical and diagnostic research : JCDR vol. 11,8 (2017): WC01-WC05. doi:10.7860/JCDR/2017/24273.10334

2. Carole Guillet, Simona Steinmann, Julia-Tatjana Maul, Isabel Kolm; Primary Localized Cutaneous Amyloidosis: A Retrospective Study of an Uncommon Skin Disease in the Largest Tertiary Care Center in Switzerland. Dermatology 2 May 2022; 238 (3): 579–586. https:// doi.org/10.1159/000518948

3. Wang Lei, Xu Ai-E ,Diagnosing of primary cutaneous amyloidosis using dermoscopy and reflectance confocal microscopy, published on 21 February 2022,https://doi.org/10.1111/ srt.13143, https://onlinelibrary.wiley. com/doi/full/10.1111/srt.13143

4. Thejaswi, Sanjay Ramachandra; Rao Shendre, Mohan Eshwar1,; Kudligi, Chandramohan1; Tophakhane, Raghavendra2. A Clinicopathological Study of Primary Cutaneous Amyloidosis in Tertiary Care Center, Hubballi. Clinical Dermatology Review 6(2):p 133-139, Jul–Dec 2022. | DOI: 10.4103/cdr. cdr_91_21

5. Weidner T, Illing T, Elsner P. Primary Localized Cutaneous Amyloidosis: A Systematic Treatment Review. Am J Clin Dermatol. 2017 Oct;18(5):629-642. doi: 10.1007/s40257-017-

6. Dincy Peter, C V et al. “Dermoscopy in Cutaneous Amyloidosis. - A

Prospective Study from India.” Indian journal of dermatology vol. 67,1 (2022): 94. doi:10.4103/ijd.ijd_850_20

7. Zychowska, M.; PiIta, K.; Rudy, I.; Skubisz, A.; Reich, A. Dermoscopic Features of Lichen Amyloidosis in Caucasians—A Case Series and Literature Review. Medicina 2021, 57, 1027. https://doi.org/10.3390/ medicina57101027

8. Franco Rongioletti, et al , unique dermoscopy pattern of primary cutaneous nodular amyloidosis mimicking a granulomatous disease, DERMOSCOPY CASE OF THE MONTH|,VOLUME 74, ISSUE 1, E9E10, JANUARY 2016A, DOI:https:// doi.org/10.1016/j.jaad.2015.09.026, https://www.jaad.org/article/S01909622(15)02199-4/pdf

9. Sonthalia, Sidharth; Agrawal, Mahima1; Sehgal, V. N.2. Dermoscopy of Macular Amyloidosis. Indian Dermatology Online Journal 12(1):p 203205, Jan–Feb 2021. | DOI: 10.4103/idoj. IDOJ_507_19 https://journals.lww.com/ idoj/Fulltext/2021/12010/Dermoscopy_ of_Macular_Amyloidosis.45.aspx

Dermatoscopy of Cutaneous Amyloidosis: A Case Report

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