The Aestheticians Journal May'23 issue

Tofacitinib for Plaque

Psoriasis: A Case Report and Clinical Review

Lupus Vulgaris

Mimicking Basal Cell

Carcinoma- A Case Report

Pyoderma Gangrenosum

Dermatologic Disorders – A Case Report

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Published for the period of May -2023

Present Scenario Aesthetics in Dermatology

Aesthetic dermatology is a new and unique dermatology practice that focuses on treatments to improve personal appearance rather than addressing underlying diseases. The field is growing due to the rising influence of the fashion industry and the desire to achieve a flawless body shape. Aesthetic dermatology includes a group of treatments such as botox application, wrinkle fillers, microdermabrasion, liposuction and enhancements. The advancement in aesthetic procedures and energy-based devices has helped achieve promising results and, therefore, has increased the number of patients seeking these treatments. Aesthetic dermatology is relevant to dermatology because the skin is not only a functional organ but also a medium of visual and tactile communication. The procedures that are currently in high demand are those that minimize lines and wrinkles and smooth the skin of the face, such as neuromodulators, dermal fillers, intense pulsed light, RF microneedling and ablative non-ablative and ablative fractional lasers.

In this issue, we have articles on Plaque Psoriasis, Lupus Vulgaris Mimicking Basal Cell Carcinoma and Pyoderma Gangrenosum.

HOPE YOU HAVE A GREAT READ

Thanks & Cheers

- Dom Daniel Executive Editor & Publisher

Tofacitinib for Plaque Psoriasis: A Case Report and Clinical Review

Dr. Komal Jerath, MD

Lupus Vulgaris Mimicking Basal Cell Carcinoma- A Case Report

Dr. Pradip Sarkar, MD

Pyoderma Gangrenosum Dermatologic Disorders – A Case Report

Dr. Faisal Aman, MBBS, DDVL

Advisory Committee

Dr.Arun Kumar S.

Dr.Chaitra Shenoy

Dr.Satish K. M.

DR.Arangi Kathyayani

Dr.Rambabu Paladugu

Dr.Sirisha Polagani

Dr.Srushti Jinagouda

Dr.Sugunavathi C.

Dr.Thejovathi G.S.

Editorial Board

Dr. Komal Jerath MD (Dermatology)

Dermatologist, Cosmetologist and Trichologist

Komal Skin and Cosmetology Clinic, Amritsar, Punjab

Dr. Pradip Sarkar MD (Dermatology)

Assistant Professor

Department of Dermatology Medical College

Kolkata, West Bengal

Dr. Faisal Aman MBBS, DDVL

Consultant Dermatologist, Venereologists, Leprologists and Cosmetologist Bhopal, Madhya Pradesh

Tofacitinib for Plaque Psoriasis: A Case Report and Clinical Review

Dr. Komal Jerath

MD (Dermatology)

Dermatologist, Cosmetologist and Trichologist

Komal Skin and Cosmetology Clinic

Amritsar, Punjab

Psoriasis is a chronic autoimmune skin condition that causes the rapid build up of skin cells. It is characterized by the development of thick, scaly patches on the skin's surface. These patches called as plaques which is typically red, inflamed and it can be itchy or painful. The most common form of psoriasis is plaque psoriasis, which affects about 80-90% of people with psoriasis.1

The exact cause of psoriasis is not known, but the condition results from the immune system signalling, in which immune cells called T cells mistakenly attacks healthy skin cells causing them to grow and multiply too quickly, leading to inflammation and the rapid turnover of skin cells forming the build up of the characteristic plaques.2 This can happen as a result of a genetic predisposition, environmental triggers, or a combination of both. Certain triggers, such as stress, injuries to the skin, certain medications and infections, can cause psoriasis to worsen.

Symptoms of psoriasis can range from mild to adverse condition which include:2

• Raised, red, scaly patches of skin (plaques)

• Itching and burning sensation

• Dry, cracked bleeding skin

• Thickened, pitted nails with ridges

• Swollen and painful joints (in the case of psoriatic arthritis)

While there is no cure for psoriasis, but it is treatable and most people with psoriasis are able to reduce the severity and frequency of flare-ups and improve their quality of life with proper treatment and management. It can be managed with a variety of treatments including topical medications like creams, light treatment, and oral or parenterals. Light therapy popular as phototherapy, uses natural or artificial light to slow down skin cell growth. Systemic medications can include oral or injectable medications which target the immune system to slow down the skin cell production and reduce inflammation.2

The severity of psoriasis can vary greatly from person to

person. Some people may have only a few small patches, while others may have extensive coverage of their skin.

Since psoriasis is a chronic condition, ongoing monitoring and regular follow-up are necessary to adjust treatment as needed and to monitor for any potential side effects. Furthermore, lifestyle changes such as diet, exercise, relaxation techniques to reduce stress, avoiding smoking and alcohol can also help in the management of plaque psoriasis.3

Psoriasis can have a significant impact on a person's quality of life, both physically and emotionally. People with psoriasis may feel self-conscious about their appearance and may experience anxiety and depression.

The main types of psoriasis are: 2, 4

1. Plaque psoriasis

Plaque psoriasis is the most common form of psoriasis and is characterized by raised, red patches of skin (plaques) that are covered by a silvery white buildup of dead skin cells. These plaques can appear anywhere on the body, but they are most commonly found on the elbows, knees, scalp and lower back.

2. Guttate psoriasis

Guttate psoriasis is the type of psoriasis, it is characterized by small, red, drop-shaped patches of skin. It is most commonly found on the trunk, limbs and scalp. Guttate psoriasis is more common in children and young adults, often appears after a streptococcal infection.

3. Inverse psoriasis

Inverse psoriasis appears as

smooth, red patches of skin in the armpits, groin and under the breasts. It is more common in people who are overweight or have a lot of skin folds.

4. Pustular psoriasis

Pustular psoriasis is characterized by red, tender skin covered by white or yellow pustules (blisters filled with pus). It can be localized to a specific area of the body or can cover a large area.

5. Erythrodermic psoriasis

Erythrodermic psoriasis is a severe and very rare form of psoriasis that causes widespread, fiery redness and scaling of the skin. It can also cause severe itching and pain.

6. Scalp psoriasis

Scalp psoriasis is characterized by red, scaly patches on the scalp. This type of psoriasis can be associated with severe itching, burning and hair loss.

although the symptoms can be similar, psoriasis is a distinct condition from eczema, seborrheic dermatitis and other skin conditions and should be diagnosed by a dermatologist.

Case Presentation

A 29 years old male patient presented to our Dermatology

7. Nail psoriasis

Nail psoriasis affects the nails and can cause discoloration, thickening and separation from the nail bed.

8. Psoriatic Arthritis

Psoriatic arthritis affects the joints; it can be a debilitating condition and cause a lot of pain in affected areas.

A B

It's also possible to have more than one type of psoriasis at the same time that can affect different parts of the body and each type has its own unique set of symptoms and characteristics. A dermatologist can diagnose the type of psoriasis based on a person's symptoms, medical history and a physical examination of the affected skin. It's important to note that

OPD with the chief complaint of numerous plaques on his body since 5 years. Physical examination revealed welldemarcated erythematous, scaly plaques with overlying thick white scales distributed throughout his body with the major involvement of elbows, forearms, back, abdomen, legs, palms and dorsum of feet and nails. There was no history of similar complaints on his face and scalp but rest of the body parts were affected. The patient experienced itching often and described the intensity as unbearable affecting his quality of life. He had no medical history of any other disease in the past. He had no history of alcohol intake. On close observation of his history found winter exacerbation of disease because it was aggravating in winter and sometimes he had summer flares also. Based on his history and physical examination; the diagnosis of plaque psoriasis was made. Fewer tests were performed like montoux test which is typically performed on skin to confirm tuberculosis. The test turned out to be normal. Chest x-ray was also conducted which was also normal. These 2 tests were conducted as mandatory tests for starting the therapy with the drug Tofacitinib. The basic blood work including CBC, LFT, RFT, Cholesterol were all normal.

Tofacitinib for Plaque Psoriasis: A Case Report and Clinical Review

Tofacitinib for Plaque Psoriasis: A Case Report and Clinical Review

The patient was then started on oral Tofacitinib 5 mg twice daily for 3 months. Oral Loratadine 10 mg for 4 weeks was given to relieve symptoms like itching. An occlusive and humectants combination with glycerine and butters was prescribed to the patient for topical application on the whole body twice a day during the entire treatment. Clobetasol propionate 0.05% with salicylic acid 6% once daily was given for all scaly lesions once daily for 2 weeks only. Patient was called for a follow up after 1 month of therapy. The patient started to show some improvement of symptoms. The patient did not turn up for the 2nd follow up which was after 2 months of treatment. The patient kept on taking Tab Tofacitnib 5 mg bd for nearly 3 months on his own and visited after 3 months. There was marked improvement seen in the 3rd month follow up. Patient had fewer numbers of lesions after the oral Toficitinib treatment. Patient stopped using steroid, salicylic acid applications after 2 weeks. Before and after pictures of patient were clicked and compared.

Before treatment

After 3 months of treatment

Before treatment

After 3 months of treatment

Figure 1: Well-demarcated erythematous, scaling plaques on the back

Figure 2: Plaques with red patches on abdomen

Before treatment

After 3 months of treatment

Before treatment

After 3 months of treatment

Figure 4 (a, b & c,): Red and white scaly plaques on the legs, dorsum of feet and nails

Figure 3: White scaly patches on elbows and forearms

Treatment

Treatment for psoriasis varies depending on the type and severity of the condition, location of the condition, as well as the person's overall health and preferences.

Topical treatments are often the first line of treatment for mild to moderate psoriasis. These include creams, ointments and shampoos that are applied directly to the affected skin. They may contain ingredients such as coal tar, salicylic acid, corticosteroids or vitamin D.

Topical treatments can be very effective in reducing

inflammation and slowing the growth of skin cells. Use of topical medications that target specific cells in the immune system, such as calcipotriene and calcineurin inhibitors like tacrolimus.5

Light therapy, also known as phototherapy, is another option for treating psoriasis. This involves exposing the affected skin to controlled doses of ultraviolet (UV) light. A variety of light/lasers having varied action can be used. Some of the examples are ultraviolet B, psoralen ultraviolet A, lightemitting diodes, intense pulsed

light (IPL) and so on. Light therapy can be effective, but it can also increase the risk of skin cancer, photo aging, sun damage, erythema, skin burning so it's important to be closely monitored by a dermatologist.6

Oral and injectable medications are often used to treat moderate to severe psoriasis or when other treatments have been ineffective. These include medications such as methotrexate, acitretin, cyclosporine and biologic medications that target specific proteins in the immune system such as adalimumab, infliximab,

Figure 6 : Plaques on the dorsal side of the hands

Figure 5 : White scaly plaques on the palms

ustekinumab, secukinumab and ixekizumab. These medications are usually administered via injection or infusion and can be effective in treating psoriasis but may have serious side effects. All biological drugs affect the immune system; therefore there is a higher risk of causing infection like bronchitis, upper respiratory tract infection, urinary infection.2

Tofacitinib is a medication that is approved for the treatment of moderate to severe rheumatoid arthritis and has been used in some clinical trials for the treatment of plaque psoriasis. Tofacitinib is an oral Janus kinase (JAK) inhibitor, which works by blocking certain proteins in the immune system that contribute to inflammation. By targeting these proteins, tofacitinib can help to reduce inflammation and slow the growth of skin cells.7

It is important to note that while tofacitinib has shown to be effective, it may have serious side effects. Hence it is important to have regular monitoring by the doctor while on tofacitinib treatment as it can have some serious side-effects. Common side effects of tofacitinib include upper respiratory infections, headache, diarrhea and high cholesterol; however these side effects are usually mild. Rare but serious side effects include an increased risk of serious infections, malignancies and blood clots, thrombocytopenia (low platelets) which can lead to bleeding. Tofacitinib is used as an alternative for people who do not respond well to traditional systemic psoriasis therapy. Hence, it is important to work closely with a dermatologist to determine if tofacitinib is a

good treatment option for you. And as with any treatment, it is important to weigh the risks and benefits of tofacitinib before starting treatment.8

Tofacitinib is not for everyone, and it should be used in conjunction with other psoriasis treatments, such as topical medications, light therapy or phototherapy.

For those who do not respond or have adverse effects to the above treatment, other systemic drugs can be tried such as apremilast, and secukinumab.2

Another option for treating psoriasis is through lifestyle changes and self-care such as:3

• Maintaining a healthy weight

• Avoiding smoking and excessive alcohol consumption

• Reducing stress

• Keeping skin moisturized

Discussion

Psoriasis is a common chronic, recurrent, immune mediated disease of the skin and joints.9 Psoriasis has a major genetic component. Plaque psoriasis is the most common form of the condition, characterized by raised, red patches of skin covered with a silvery white buildup of dead skin cells, called plaques. Psoriasis vulgaris is also called plaque-type psoriasis and is the most prevalent type.2 The extra skin cells form scales and red patches that are sometimes itchy and painful.

The diagnosis of psoriasis is primarily clinical, sometimes there may need skin biopsy.

Clinical features of psoriasis observed on diagnosis are well bordered erythramatous plaque with silvery scales. It

may be localised to any part with upper and lower limb being the most common site. On skin histopathology studies shows psoriasiform pattern, described as epidermal hyperplasia with bulbous enlargement at the ridges.10

There is currently no cure for psoriasis. Treatment for plaque psoriasis can include topical medications, such as creams and ointments that are applied directly to the affected skin. These medications can help reduce inflammation, itching and scaling, but they can also cause side effects like skin irritation.4

Symptoms of plaque psoriasis can vary from person to person and can range from mild to severe. Common symptoms include red, scaly patches on the skin, itching and burning sensations, dry and cracked skin. Some people may also experience joint pain and stiffness, a condition known as psoriatic arthritis.2

Psoriasis is a chronic skin condition as well as systemic or lifestyle disease; it can be manageable but not curable. It is also worth noting that diet and lifestyle changes also have a role in managing plaque psoriasis symptoms. It is different for every person and can change over time. Some people find that maintaining a healthy diet and weight, avoiding smoking, reducing stress and avoiding alcohol can be helpful for managing symptoms.3, 4

Proper diagnosis and treatment, including management of comorbidities, is crucial in order to improve outcomes for people with psoriasis. It is important for primary care physicians to be

Tofacitinib for Plaque Psoriasis: A Case Report and Clinical Review

aware of the signs and symptoms of psoriasis and to refer patients to a dermatologist for further evaluation and treatment when necessary. Treatment options for psoriasis include topical treatments, light therapy and systemic medications. Systemic medications can include oral or injectable medications which target the immune system to slow down the skin cell production and reduce inflammation.

A topical therapy is the main player in managing psoriasis. Topical Corticosteroids and Vitamin D analogue is the drug of choice among other topical agents. Since light spectra has good penetrating power, hence can be used in various target tissues. It has an advantage of higher dose radiation delivery in short period of time.5

Conclusion

In conclusion, psoriasis is a chronic, complex inflammatory skin condition, characterized by the rapid growth of skin cells, which form red, scaly patches on the skin. It can affect different parts of the body and can be accompanied by other health problems. The condition can also have a significant impact on a person's quality of life, affecting their physical, emotional and social well-being.

Considering the oral and systemic medication, various factors play vital role for choosing the choice of medicine. Depending on severity, location and suitability choice is made. Tofacitinib is usually prescribed after other options have failed and it should only be used under the care of a doctor and close monitoring, because of potential safety concerns. As Tofacitinib

targets the immune system, it may increase the risk of infections and lab test should be done to check the blood counts and cholesterol regularly. Tofacitinib is a promising treatment option for moderate to severe plaque psoriasis; it has been found to be effective in reducing symptoms and improving the quality of life of patients with the condition. While treatment can help manage the symptoms of psoriasis and improve the appearance of affected skin, there is currently no cure for the condition.

It's important to work closely with a dermatologist to develop an individualized treatment plan. Since psoriasis is a chronic condition, ongoing monitoring and regular follow-up is necessary to adjust treatment as needed and to monitor for any potential side effects.

References

1. Badri T, Kumar P, Oakley AM. Plaque Psoriasis. [Updated 2022 Aug 8]. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2022 Jan-. Available from: https://www.ncbi.nlm. nih.gov/books/NBK430879/

2. Rendon A, Schäkel K. Psoriasis Pathogenesis and Treatment. Int J Mol Sci. 2019 Mar 23;20(6):1475. doi: 10.3390/ijms20061475. PMID: 30909615; PMCID: PMC6471628.

3. Ko SH, Chi CC, Yeh ML, Wang SH, Tsai YS, Hsu MY. Lifestyle changes for treating psoriasis. Cochrane Database Syst Rev. 2019 Jul 16;7(7):CD011972. doi: 10.1002/14651858.CD011972. pub2. PMID: 31309536; PMCID: PMC6629583.

4. Kim WB, Jerome D, Yeung J. Diagnosis and management of psoriasis. Can Fam Physician. 2017 Apr;63(4):278-285. PMID: 28404701;

PMCID: PMC5389757.

5. Torsekar R, Gautam MM. Topical Therapies in Psoriasis. Indian Dermatol Online J. 2017 Jul-Aug;8(4):235-245. doi: 10.4103/2229-5178.209622. PMID: 28761838; PMCID: PMC5518573.

6. InformedHealth.org [Internet]. Cologne, Germany: Institute for Quality and Efficiency in Health Care (IQWiG); 2006-. Psoriasis: Oral medications and injections. 2017 May 18. Available from: https://www.ncbi.nlm.nih.gov/books/ NBK435704/

7. Bronson, Joanne (2013). [Annual Reports in Medicinal Chemistry] Volume 48 || To Market, To Market—2012. , (), 471–546. doi:10.1016/B978-0-12417150-3.00028-4

8. Ly K, Beck KM, Smith MP, Orbai AM, Liao W. Tofacitinib in the management of active psoriatic arthritis: patient selection and perspectives. Psoriasis (Auckl). 2019 Aug 28;9:97-107. doi: 10.2147/PTT.S161453. PMID: 31696051; PMCID: PMC6717840.

9. Langley, R G B (2005). Psoriasis: epidemiology, clinical features, and quality of life. Annals of the Rheumatic Diseases, 64(suppl_2), ii18–ii23. doi:10.1136/ard.2004.033217

10. Gisondi P, Bellinato F, Girolomoni G. Topographic Differential Diagnosis of Chronic Plaque Psoriasis: Challenges and Tricks. J Clin Med. 2020 Nov 8;9(11):3594. doi: 10.3390/ jcm9113594. PMID: 33171581; PMCID: PMC7695211.

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Lupus Vulgaris Mimicking Basal Cell Carcinoma- A Case Report

Dr. Pradip Sarkar

MD (Dermatology)

Assistant professor

Department of Dermatology

Medical College Kolkata, West Bengal

Introduction

Lupus vulgaris is progressive, paucibacillary form cutaneous tuberculosis seen in patients sensitized to M. Tuberculosis 1 Mainly such conditions are visible in patients with moderate to low degree of immunity. This is susceptible to all age group equally with three to four times more chances of females as compared to males. The most salient histopathology trait spotted was tubercle formation with or without caseation, encircle by some epitheliod histiocytes.2 The head and the neck regions are most likely being affected.3 Spontaneous involution takes place with addition of new lesions within old scar. No improvement is seen without appropriate therapy. Some other features like atrophy with extravagant hyperkeratosis were also observed. There are five variations of lupus vulgaris: hypertrophic or vegetation, plaque, tumour-type, papular or nodular and ulcerative types.2

Basal cell carcinoma is mainly observed in skin areas that are more prone to sun exposures and it is mainly a non melanoma skin cancer which was also

known as basal cell epithelioma, with increasing prevalence rates. It is a slow growing tumour with fewer chances for metastasis and lesser fatality rate and can turn highly destructive when treatment is inadequate or delayed. It mainly emerges as flesh- or pink-colored, pearly papules surrounded by ulceration or telangiectatic vessels.2

26 subtypes of BCC have been reported up till now, amongst which most common are nodular, micro nodular, superficial, morpheaform, infiltrative and fibroepithelial. Majority cases are amelanotic but fluctuating amounts of melanin is found on these tumours. Method of treatment involved is excision, electrodesiccation and curettage (EDC), cryosurgery and Mohs micrographic surgery. These methods are restricted for localised BCC and offers 95% cure rates.4

Here we report an interesting case of lupus vulgaris mimicking basal cell carcinoma.

Case Report

A 65 years old male farmer by

occupation presented to our outpatient department with one year history of small skin bump on his nose which on given period of time slowly progressed further. The patient initially came with complaints of pain, ulceration pigmented raised lesion over his nose. There was no history of any predisposing factors such as radiation burns, immunocompromised status and arsenic exposure. No significant past, family and medical history. On cutaneous examination revealed ulcerative plaque on nose with crust and well defined border (Figure 1). Ulcer border was found to be undermining with swallow base. Clinically it was indicating lupus vulgaris with peculiar atrophy at one end and progression at other end. On further evaluation of the lesion for indicated basal cell carcinoma (BCC), squamous cell carcinoma (SCC), cutaneous tuberculosis. Histopathological examination showing caseating granuloma. There was no lymph node involvement which indicated no lymphadenopathy. There was no bone involvement as well. Final diagnosis was made lupus vulgaris.

Diagnosis

Basal cell carcinoma (BCC) is a type of skin cancer, usually slowgrowing and found mainly in sun damaged areas. To diagnose BCC, a dermatologist will usually start by examining the skin for any suspicious growths or changes. Clinically it was indicating lupus vulgaris with peculiar atrophy at one end and progression at other end.2 Biopsy was also performed where a small sample of tissue is taken from the suspicious area and examined under a microscope to determine if it is cancerous. On further evaluation of the lesion for indicated basal cell carcinoma, squamous cell carcinoma, cutaneous tuberculosis.5 The lesions were sharp bordered, irregular, brownishred plaques on physical appearance with noticeably swollen nose. Ulcer border was found to be undermining with swallow base. Histopathology of the lesion on the nose showed a granulomatous tubercle. Biopsy also revealed ulcerated area encircled superficially by crusts, some giant cells within. The patchy area had atrophic plaque with some ulceration on the extremes. There was no lymph node involvement which indicated no lymphadenopathy. There was no bone involvement as well. Final diagnosis suggested was lupus vulgaris. Other tests, such as an imaging scan or a blood test may also be ordered if the dermatologist suspects that the cancer has spread beyond the skin.

Discussion

This case report seeks to highlight unusual cases of lupus vulgaris involving the nose and mimicking basal cell carcinoma. Lupus vulgaris is earliest recognized chronic, destructive, slowly progressive type of cutaneous tuberculosis. Mainly observed on sun-damaged skin by atrophy, scarring and ulcerative lesions. Sometimes associates with, lymph node bone and lung. Primary areas of target are the head and neck regions. On persistent cases, patients have developed squamous cell carcinoma, basal cell carcinoma and sometimes associated with deformity.6

It is a reinfection tuberculosis originating externally by direct inoculation of the bacilli or internally by haematogenous or lymphatic spread from affected area in host. In this case the lesion imitated

Lupus Vulgaris Mimicking Basal Cell Carcinoma- A Case

Figure 1: Ulcerative plaque on nose with crust and well defined border

Figure 2: Histopathology of the lesion showed a granulomatous tubercle with some giant cells

basal cell carcinoma by keeping in mind the occupation of the patient involving maximum sun time exposure. The physical appearance depicting a nodulo-ulcerative plaque with undermine border and no lymphadenopathy. There was appearance of new lesions in the areas of old scar. 90% of skin cancers consist of basal cell carcinoma and squamous cell carcinomas originating from basal keratinocytes of the epidermis and sometimes from hair follicle and sebaceous glands.7

Conclusion

Cutaneous tuberculosis is one of the causes of basal cell carcinoma. Basal cell carcinoma is a malignant tumour arriving from keratinocytes of the epidermis leading to skin cancer. In above mentioned case patient was experiencing lupus vulgaris which later turned out to be secondary malignancy which on histopathological examination confirmed to be basal cell carcinoma. From the above case presentation we can conclude that, there are rare chances of long lasting lupus vulgaris which eventually developed into malignancy case. We can hence conclude that cutaneous tuberculosis abides to be a great mimic of skin cancers including squamous and basal cell carcinoma. Diagnosis of such a case is difficult thus it is essential to carry out wide excision of the affected area especially the affected and scared region. Although the cases of cutaneous tuberculosis are rare but still its diagnosis and treatment are very fundamental for both patient and public health. Early diagnosis and treatment may be helpful to

stop prevailing of skin cancer.

References

1. Hassan I, Ahmad M, Masood Q. Lupus vulgaris: An atypical presentation. Indian J Dermatol Venereol Leprol 2010;76:180-181

2. Pai VV, Naveen KN, Athanikar SB, Dinesh US, Divyashree A, Gupta G. A clinico-histopathological study of lupus vulgaris: A 3 year experience at a tertiary care centre. Indian Dermatol Online J. 2014 Oct;5(4):461-5. Doi: 10.4103/2229-5178.142497. PMID: 25396129; PMCID: PMC4228641.

3. Theodosiou, G., Papageorgiou, M., & Mandekou-Lefaki, I. (2018). An Unusual Presentation of Lupus Vulgaris and the Practical Usefulness of Dermatoscopy. Case Reports in Dermatological Medicine, 2018, 1–3. Doi:10.1155/2018/1036162

4. Mcdaniel B, Badri T, Steele RB. Basal Cell Carcinoma. [Updated 2022 Sep 19]. In: statpearls [Internet]. Treasure Island (FL): statpearls Publishing; 2022 Jan-. Available from: https://www.ncbi. nlm.nih.gov/books/NBK482439/

5. Abhinesh N, Danny C G, Srinivasan S, Pravin A. Basal cell carcinoma masquerading as lupus vulgaris - A case report. IP Indian J Clin Exp Dermatol 2021;7(2):172-174.

6. Kate MS, Dhar R, Borkar D B, Ganbavale DR. Longstanding lupus vulgaris with basal cell carcinoma. Indian J Pathol Microbiol 2009;52:58890

7. Khandpur, S., & Reddy, B. (2003). Lupus vulgaris: unusual presentations over the face. Journal of the European Academy of Dermatology and Venereology, 17(6), 706–710. Doi:10.1046/j.14683083.2003.00838.x

Pyoderma Gangrenosum Dermatologic Disorders – A Case Report

Dr. Faisal Aman

MBBS, DDVL

Consultant Dermatologist, Venereologists, Leprologists and Cosmetologist

Bhopal, Madhya Pradesh

Introduction

Pyoderma gangrenosum (PG) is a rare and chronic inflammatory skin disorder that causes the formation of painful ulcers on the skin with hemorrhagic exudates. The ulcers typically begin as small, red, tender bumps that quickly progress to large, deep and necrotic (dead tissue) ulcers. The ulcers are typically accompanied by erythema (redness) and purulence (pus) around the edges. The ulcers can occur anywhere on the body, but are most commonly found on the legs, the lower abdomen and the genitals. Pyoderma gangrenosum is a type of neutrophilic dermatosis, which means that it is characterized by an accumulation of neutrophils (a type of white blood cell) in the affected area of the skin.1

Many variants of pyoderma gangrenosum are observed: Peristomal pyoderma gangrenosum, pustular pyoderma gangrenosum, bullous pyoderma gangrenosum and vegetative pyoderma gangrenosum.2

Some common symptoms associated with pyoderma gangrenosum include:1

• Painful skin ulcers that can be deep and large

• Redness and swelling around the ulcer

• Tenderness or warmth in the affected area

• Pus or drainage from the ulcer

• Fatigue

• Fever

The exact cause of pyoderma gangrenosum is unknown, but it is thought to be a type of immune-mediated disorder. Some cases have been associated with underlying inflammatory conditions such as inflammatory bowel disease, ulcerative colitis rheumatoid arthritis, hematologic disorders and myelodysplastic syndrome.2 It is also reported that pyoderma gangrenosum is an outturn from some drug regimen like propylthiouracil, pegfilgastrim and gefinib. Also in immunocompromised host due to exposure to some bacterial infection.1

The diagnosis of pyoderma gangrenosum is based on clinical presentation and the

exclusion of other conditions. It is mainly diagnosed based on the patient's history, physical examination and the appearance of the ulcers. A skin biopsy may be performed to confirm the diagnosis.1 The treatment of pyoderma gangrenosum typically involves a combination of therapies, including topical and systemic immunosuppressive medications, antibiotics, biologics wound care and surgery. The goal of treatment is to control the inflammation and promote healing of the ulcers.3

It is a rare, chronic and a debilitating condition with a potential of affecting the quality of life of the patient. Early diagnosis and appropriate management can help in preventing the formation of deep and extensive ulcers, which can lead to significant scarring and disfigurement.

Clinical Presentation

Pyoderma gangrenosum typically presents as painful ulcers on the skin. The clinical presentation can vary depending on the stage of the condition and the location of the ulcers. The most common presentation of pyoderma gangrenosum is a painful, violaceous and necrotic ulcer that may be preceded by a minor skin injury or infection.4 Mainly it presents ulcers which are deep and defined borders; having undermined edge accompanied by erythromatous or lesions skin encircling the wound.2 Primarily observed on the lower legs especially near the pretibial area.1

Some common features of

pyoderma gangrenosum include:

1. Ulceration: The ulcers typically begin as small, red and tender bumps that quickly progress to large, deep and necrotic (dead tissue) ulcers. These ulcers have usually cat paw like appearance.2

2. Erythema: The ulcers are typically accompanied by erythema (redness) and purulence (pus) around the edges with edema.1

3. Tenderness: The ulcers are typically painful and tender to the touch.1

4. Malodorous: Owing to secondary infection, a purulent cover is formed over the ulcers.1

5. Rapid progression: The ulcers can grow rapidly, often within a matter of hours or days.3

6. Pruritus: Itching is a common complaint in patients with pyoderma gangrenosum.

7. Systemic symptoms: Patients may also experience systemic symptoms such as fever, malaise and weight loss.2

8. Hepatitis C, ulcerative colitis, rheumatoid arthritis, lymphoproliferative disorders and myelodysplastic syndrome are believed to be the underlying diseases which can cause pyoderma gangrenosum.1

The clinical presentation of pyoderma gangrenosum can be variable and it may be mistaken for other skin disorders such as cellulitis, necrotizing fasciitis or vasculitis. A biopsy is often performed to confirm the

diagnosis.2

Case Report

A 35 year old male patient was presented to the outpatient department with complaint of painful ulcerations on both lower limbs since past three months. He initially found few boils which on given period of time grew bigger to erythematous ulcers. On further investigation found the person was is non diabetic and non hypertensive and farmer by occupation. He is non smoker and didn't show any presence of varicosity on both limbs. On clinical examinations showed violet or blue coloured ulcers with well defined border, vasculitis on the ridges of ulcers and some areas of thrombosis were observed. Differential diagnosis initially was infective ulceration which was then sent for skin biopsy which revealed deep folliculitis and a network of neutrophilic infiltrate. Which further lead to the conclusion of pyoderma gangrenosum.

Some treatments were then started which included methyl prednisolone (16 mg) tablet to be taken in the morning and night for 5 days, tablet methotrexate 7.5 mg once a week for 2 weeks, folic acid supplement one tab in night for 15 days, Cefprozil 500 mg twice daily for 5 days and hydroxyzine hydrochloride in night for 7 days.

Clinical Examination

Pyoderma gangrenosum is a rare and serious skin condition that typically requires an accurate diagnosis and prompt treatment to prevent serious complications. Early diagnosis is must to avoid any future implications of secondary infections leading to formation of disfiguring scars which many cause difficulties in diagnosis.5 Pyoderma gangrenosum is often diagnosed based on the patient's medical history, physical examination and the characteristic appearance of the affected skin.4 There are no specific laboratory findings for this condition.

However, some confirmatory test undertaken is as follows:

1. Clinical examination: Any diseases if present, progression of ulcers and effectiveness of therapy prescribed.4

2. Physical examination: Ulcers, erythema and involvement of any other internal organs.4

3. Biopsy: A skin biopsy may be performed to confirm the diagnosis. It is sent for detection of histopathology of the existing

state along with test for acid fast bacilli.6 The biopsy typically shows a neutrophilic infiltrate in the dermis and subcutaneous tissue.1

4. Pathergy test: A positive pathergy test, which is a skin reaction to a needle prick, is seen in about 40% of cases.6

5. Laboratory tests: Blood tests may be done to rule out underlying conditions that can cause pyoderma gangrenosum such as inflammatory bowel disease, rheumatoid arthritis and myelodysplastic syndrome.2

7. Imaging: Imaging studies like X-rays, CT scans or MRI, abdominal ultrasound, endoscopy and colonoscopy if digestive symptoms are presented these may be done to rule out underlying conditions.4

A definitive diagnosis of pyoderma gangrenosum can be challenging, as it can mimic other skin conditions, such as cellulitis, necrotizing fasciitis or vasculitis. A biopsy is often performed to confirm the diagnosis and exclude other conditions. As biopsy aids in eliminating any other ailments like malignancy, vaculitis or infection.2 Once the diagnosis is made, the treatment plan can be formulated accordingly.

Figure 1: Rapid growing pyoderma gangraenosum affecting the left leg

Figure 2: Pyoderma gangrenosum with undermined borders affecting the right leg

Treatment

Pyoderma gangrenosum is a rare skin condition that typically requires treatment by a dermatologist or other specialist. Main aim of therapy is to reduce tissue destruction and enhance healing of the wound. Through medication relieve from existing ulcers and inhibition from formation of new ulcers should be the primary motive of any medication.2

Treatment options may include: Topical medications to control infection, corticosteroids (topical), immune-suppressing medications, systemic therapy, biologic medications and surgery.3

Topical treatments

Corticosteroids: On initial stages doctors often prescribe oral corticosteroids for remission. Prednisolone being the preferred drug and usually given on high doses. On chronic exposure to these high doses have lead to some steroid related side effects.1 This is an effective treatment in case of unilesional pyoderma. Size of the lesion is an effective parameter in determining time of healing.3

Tacrolimus: It is topical calcineurin inhibitors. The main mechanism involved is reduction in the inflammatory cytokines (IL-2, IL-3 and interferon-y) generations and promotion of lymphocyte proliferation. Usually advised to apply twice a day.3 Sometimes it has said to show systemic absorption.3

Pimecrolimus: It is also a calcineurin inhibitor having

Figure 3: Approach to the patient with pyoderma gangrenosum

Figure 4: Skin biopsy from ulcerated pyoderma showing gangrenosum dermal lymphocytic infiltrate

Figure 5: Higher magnification suggested the infiltrate to be composed of neutrophils

primary actions such as reduction in augmentation of T-cell and its further activation process, prevents degranulation and release of further proinflammatory cytokines. It is mainly preferred in conditions like mild localized pyoderma gangrenosum. Sometimes as an adjunct therapy to some systemic drugs in case where conditions are severe or unresponsive.3

Sometimes even topical cyclosporine is used in refractory cases. It has appeared to exempt from tolerability issues which is main problem linked to systemic treatment.3

Systemic therapy

In cases of rapidly proliferative condition use of systemic drug is a must.

Systemic Corticosteroids

It is the first line therapy which is adopted by many doctors. It mainly shows antiinflammatory effects along with down regulation of proinflammatory cytokines and chemokines involved in its pathogenesis.3 They also heckle ulcerative progression and thereby new lesions are prohibited.4 Prednisolone are mostly administered and methylprednisolone for action of pulse therapy.1

Immunosuppressive: Since the main pathophysiology associated is an alteration in immune system, which implies that immune suppressive stands out as one of the treatment. Administered topically or systemically depending on severity of

wound.5 Mainly cyclosporine, azathioprine, methotrexate and mycophenolate mofetil to control the underlying autoimmune disorder.3

Cyclosporine being the main line of therapy which disrupts the synthesis of ILs, particularly IL-2 which is associated T-lymphocyte deactivation process.3

Methotrexate which is another immunomodulating drug mainly involved in release of adenosine which further brings down the inflammation and linked immune response. Also brings about nitric oxide uncoupling which decreases susceptibility of T-cells for appotosis.3

Mycophenolate Mofetil: It mainly acts against inosine monophosphate dehydrogenase (in purine salvage pathway) which further decreases guanosine triphosphate which indirectly decreases the lymphocyte proliferation as it lacks purine salvage pathway. It is mainly used as steroid-sparing agent.3

Azathioprine: It brings down the level of circulating monocytes and also inactivates T- cell. Same pathway as mycophenolate mofetil.3

Biologics

Anti-tumour necrosis factor ɑ agents: Infliximab, adalimumab and golimumab which is an monoclonal antibody against tumour necrosis factor a which binds to soluble form and said to induce apoptosis, etanercept which is a recombinant protein that is associated to neutralise the soluble factors are mainly

used agents.2

Interleukin-1ß Inhibitors: In pyoderma gangrenosum cases it has been observed that there is raising levels of Interleukin-1ß. Commonly used agents are anakinra, canakinumab and gevokizumab.3

Surgery

Autologous split-skin grafts are most adopted methods. It is advised to go for surgery in partly remission cases. Sometimes bioengineered skin is also used which are one of the newer developments.1

Wound care

Moist wound management is very crucial since there is presence of heavy exudates in much ulcerative condition. In such cases dressings (silicone, foam) having many different layers are usually recommended. Also use of adsorbents like alginate (haemostatic properties) for bleeding condition, hydrofiber can be applied.3

The treatment plan will depend on the severity of the condition and the patient's response to therapy. Close follow-up with a healthcare provider is crucial to monitor the condition as some cases, the condition may recur even after successful treatment.

Discussion

Pyoderma gangrenosum is a rare, chronic inflammatory skin condition that causes the development of painful, ulcerating sores. Pyoderma gangrenosum is a debilitating condition that can affect the quality of life of patients. Mainly affects 40–60 years of age.

Pyoderma Gangrenosum Dermatologic Disorders –

The exact cause of pyoderma gangrenosum is not known, but it is thought to be related to an abnormal immune response. The condition can occur in individuals of any age and can affect both men and women.1 The diagnosis of pyoderma gangrenosum is based on the patient's history, physical examination and the appearance of the ulcers. A skin biopsy may be performed to confirm the diagnosis.4 Due to the rarity of the condition, some healthcare providers may not be familiar with the diagnosis and management of pyoderma gangrenosum, so referral to a specialist in dermatology or wound care may be necessary. The diagnosis is often made by a dermatologist or a wound care specialist, but it can be challenging as it can mimic other skin conditions.

Symptoms of pyoderma gangrenosum typically include the development of one or more painful, red, swollen sores that may be accompanied by itching or burning. These sores can become deep and large; can result in scarring and disfigurement if left untreated. Pyoderma gangrenosum can also cause fever and joint pain in some individuals.1 There is no one definitive treatment for pyoderma gangrenosum and treatment plans are often tailored to the individual.

Treatment options may include the use of immunosuppressive medications, antibiotics, biologics wound care and surgery.1 The mainstay of treatment for pyoderma gangrenosum is the use of

systemic corticosteroids, which are effective in controlling the inflammation and promoting healing. In some cases, immunosuppressive agents such as cyclosporine, azathioprine, methotrexate and mycophenolate mofetil may be needed to control the underlying autoimmune disorder.2 Biologic medications such as TNF inhibitors and IL-1 inhibitors can also be effective in treating pyoderma gangrenosum, especially in cases that are refractory to other treatments. In severe cases, surgery to remove the affected skin may be necessary to prevent sepsis and tissue loss.3

Close follow-up with a healthcare provider is crucial to monitor the condition and adjust treatment as necessary. It is important for patients to seek treatment from a board-certified dermatologist or wound care specialist for proper diagnosis, treatment and management. With proper treatment, the prognosis of pyoderma gangrenosum can be managed; however, it is a chronic condition that requires long-term management.

Conclusion

In conclusion, pyoderma gangrenosum is a rare and chronic skin condition characterized by painful, ulcerating sores and can cause significant morbidity and even mortality if not treated appropriately. The condition is characterized by the sudden onset of painful, violaceous and necrotic ulcers that typically develop on the legs. It may be preceded by a minor skin injury

or infection. The condition can be difficult to treat and may require a combination of therapies, including corticosteroids, immunosuppressant’s and wound care. The prognosis for individuals with pyoderma gangrenosum can vary and may depend on the severity of the condition and the effectiveness of the treatment. The mainstay of treatment for pyoderma gangrenosum is the use of systemic corticosteroids, which are effective in controlling the inflammation and promoting healing. In some cases, immunosuppressive agents, biologic medications and surgery may be needed to control the underlying autoimmune disorder. Close follow-up with a healthcare provider is crucial to monitor the condition and adjust treatment as necessary. Due to its chronic nature and the difficulty in treating it, pyoderma gangrenosum can have a significant impact on a person's quality of life. Therefore it is advised to consult with a board-certified dermatologist or a specialist in inflammatory skin conditions as early as possible, as pyoderma gangrenosum can progress quickly and lead to severe complications if left untreated.

The management of pyoderma gangrenosum requires a multidisciplinary approach and close collaboration between a dermatologist and other specialists such as rheumatologist or gastroenterologist. Early diagnosis and treatment can help prevent complications and

Pyoderma Gangrenosum Dermatologic Disorders

improve outcomes. Research is ongoing to better understand the causes of pyoderma gangrenosum and to develop new treatment options.

References

1. Wollina, U. Pyoderma gangrenosum – a review. Orphanet J Rare Dis 2, 19 (2007). Https://doi. org/10.1186/1750-1172-2-19

2. Brooklyn T, Dunnill G, Probert C. Diagnosis and treatment of pyoderma gangrenosum. BMJ. 2006 Jul 22;333(7560):181-4. Doi: 10.1136/bmj.333.7560.181. PMID: 16858047; PMCID: PMC1513476.

3. Maronese, C.A., Pimentel, M.A., Li, M.M. et al. Pyoderma Gangrenosum: An Updated Literature Review on Established and Emerging Pharmacological Treatments. Am J Clin Dermatol 23, 615–634 (2022). https://doi. org/10.1007/s40257-022-00699-8

4. Konopka, Clóvis Luíz; Padulla, Geórgia Andrade; Ortiz, Michele Purper; Beck, Anderson Kahl; Bitencourt, Mariana Rechia; Dalcin, Diogo Chagas (2013). Pioderma Gangrenoso: um Artigo de Revisão. Jornal Vascular Brasileiro, 12(1), 25–33. doi:10.1590/S167754492013000100006

5. Skopis, M.; Bag-Ozbek, A. Pyoderma Gangrenosum: A Review of Updates in Diagnosis, Pathophysiology and Management. J 2021, 4, 367-375. https://doi. org/10.3390/j4030028

6. Riyaz N, Mary V, Sasidharanpillai

S, Roshin RA, Snigdha O, Latheef EN, Rahima S, Bindu V, Anupama RN, Sureshan DN, Sherjeena PV. Pyoderma gangrenosum: A clinico-epidemiological study. Indian J Dermatol Venereol Leprol 2017;83:33-39