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Dermatology Clinic
CASE #1
Patches of Hair Loss
TIFFANEY TRAN, BS; TARA BRAUN, MD; CHRISTOPHER RIZK, MD
A 15-year-old girl presents with patches of hair loss on her scalp that have been present for the last 6 months. She reports that her scalp is not itchy or painful and denies any recent illnesses, diet changes, or stressful life events. She does not have any other medical conditions and takes no medications. Her uncle had a similar condition that started when he was in his 20s, but no one else in the family has experienced hair loss. The patient says she has not tried any treatments for the condition. On examination, she has several well-demarcated patches of hair loss on her frontal and parietal scalp, with no perifollicular erythema or scale.
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CASE #2
Pink Pedunculated Nodule on Palm
DOMINIQUE JACOBS, BS; KATRINA HANSEN, DO
A 65-year-old man of Middle Eastern descent presents with a tender, weeping mass on his right palm. Since appearing 8 months ago, the lesion has enlarged into a 0.9-cm, pink pedunculated nodule with abundant serous crust. The patient has no significant history of trauma. His medical history is significant for atrial fibrillation, hypertension, coronary artery disease, congestive heart failure, and lung cancer, which was treated with right lobectomy. It is unclear what medications were used to treat his lung cancer. His current medications include metoprolol, lisinopril, allopurinol, aspirin, and digoxin. The patient also presents with additional pink, scaly plaques on his right ankle, consistent with nummular eczema.
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Dermatology Clinic
CASE #1 Alopecia Areata
Alopecia areata (AA) is a sudden-onset autoimmune condition characterized by discrete areas of nonscarring hair loss.1 The extent of alopecia can range from a few patches of hair loss to complete loss of all scalp hair (alopecia totalis) or all body hair (alopecia universalis).1 The earliest known records of AA are found in ancient Egyptian papyruses dating back to 1500 BC. In these texts, a phrase that translates to “bite alopecia” is thought to describe patchy AA.2 The term alopecia was first mentioned by Hippocrates in approximately 400 BC; later Celsus (25 BC-50 AD) first described “areas of alopecia” in his medical writings.2 In these documents, Celsus used the term alopekia to describe bald spots in the scalp and beard. AA also has been referred to as porrigo decalvans, tinea decalvans, and phytoalopecia.2
In the United States, the lifetime prevalence of AA is estimated to be 2.5%.3,4 Disease onset may occur at any age, including early childhood, and both sexes are affected equally.5 The majority of cases develop before 40 years of age.4
Alopecia areata is thought to be caused by the breakdown of immune privilege (IP) in the hair follicle (HF) and the subsequent development of autoimmunity against the HF.6 Immune privilege refers to the ability of certain organs to evade potential harm from an inflammatory immune response.6 Normally, HFs produce a milieu of local factors that maintain their IP, but in patients with AA, this process breaks down.1 The underlying cause of AA remains unknown; it is hypothesized that susceptible patients may have defects in the HF or dysregulation of their immune system.6
Risk factors for AA include genetic susceptibility, concurrent autoimmune disease, and environmental factors.7 Individuals with a family history of AA are more likely to develop AA, but onset and severity are difficult to predict.6,7 Genome-wide association studies have identified numerous genetic loci associated with increased AA risk, especially in the human leukocyte antigen region.1,7 Alopecia areata is associated with other autoimmune diseases, such as vitiligo, inflammatory bowel disease, psoriasis, thyroid disease, rheumatoid arthritis, and systemic lupus erythematosus.1 In patients with a genetic predisposition, possible environmental triggers include emotional stress, physical injury, viral infection, drugs, and vaccinations; however, in the majority of cases, the precipitating factor cannot be identified.1
In addition to its genetic variability, AA also demonstrates phenotypic variability. Alopecia areata classically manifests as well-circumscribed round, hairless patches without perifollicular scaling or erythema, but other distinctive patterns may be observed.1 For instance, ophiasis denotes a band-like pattern of hair loss along the occipital and temporal margins, and sisaipho denotes the opposite, that is, extensive hair loss sparing the margins.1 As AA may affect any hair-bearing site, hair loss may occur in the beard area and may involve the eyebrows and eyelashes.1 It may target pigmented hairs and spare white hairs, or it may induce diffuse hair loss without discrete patches.1 In some patients, the disease may progress to alopecia totalis or alopecia universalis. Nail changes, such as pitting or trachyonychia, are associated with severe manifestations of AA.1
In AA, exclamation point hairs, a specific finding defined by proximal narrowing of the hair shaft, may be appreciated on dermoscopy in addition to nonspecific yellow dots, black dots, and dystrophic hairs.1 Although AA usually is clinically diagnosed, a skin biopsy may be performed if the diagnosis is uncertain.
Histopathology will show characteristic peribulbar and intrabulbar lymphocytic infiltrate. The infiltrate also may contain eosinophils, CD1+ cells, and CD8+ cells. Other histologic findings may include pigment incontinence and an increased number of miniaturized hair follicles.1
Depending on examination findings, the clinician may consider other causes of nonscarring alopecia in the differential diagnosis, such as tinea capitis, androgenetic alopecia, telogen effluvium, and trichotillomania. In contrast to AA, tinea capitis presents with pruritus, erythema, and scaling of the scalp.1 In patients with androgenetic alopecia, diffuse hair loss occurs in a sex-specific pattern, and in patients with trichotillomania, patches of hair loss are irregular in shape and contain broken hairs.1 Telogen effluvium presents as diffuse hair shedding as opposed to the discrete patches of hair loss seen in AA.1
There is no cure for AA and treatment options have variable efficacy.8 Patients may recover spontaneously but most treated patients experience relapse.1 For patients with patchy disease, intralesional corticosteroids administered
For patients with patchy disease, intralesional corticosteroids administered over multiple sessions are preferred.
over multiple sessions are preferred; for those with limited disease, topical corticosteroids alone may be beneficial. However, for patients with extensive or rapidly progressive disease, systemic corticosteroids may need to be considered, bearing in mind potential side effects. Topical options for AA include minoxidil, anthralin, and immunotherapy (eg, diphenylcyclopropenone).1,8 Promising results have been reported with novel targeted approaches, such as Janus kinase inhibitors that block the downstream signaling pathway of interferon-γ.
8
With any of these treatment approaches, patient education and counseling are necessary to manage patient expectations. Given its unpredictability and cosmetic consequences, AA can be a devastating diagnosis that negatively affects quality of life. Psychological comorbidities include depression and anxiety, and patients may require professional support as they cope with the disease.1
The patient in this case was diagnosed with AA clinically and she was immediately started on corticosteroid injections, after which she experienced some hair regrowth. However, several months later she started developing new patches of hair loss for which she is undergoing additional corticosteroid injections.
Tiffaney Tran, BS, is a medical student at Baylor College of Medicine, in Houston, Texas; Tara L. Braun, MD, is a resident in the Department of Dermatology at Baylor College of Medicine; and Christopher Rizk, MD, is a dermatologist affiliated with Baylor College of Medicine.
References
1. Pratt CH, King LE, Messenger AG, Christiano AM, Sundberg JP. Alopecia areata. Nat Rev Dis Primers. 2017;3:17011. 2. Broadley D, McElwee KJ. A “hair-raising” history of alopecia areata. Exp Dermatol. 2019;29(3):208-222. 3. Benigno M, Anastassopoulos KP, Mostaghimi A, et al. A large cross-sectional survey study of the prevalence of alopecia areata in the United States. Clin Cosmet Investig Dermatol. 2020;13:259-266. 4. Mirzoyev SA, Shrum AG, Davis MDP, Torgerson RR. Lifetime incidence risk of alopecia areata estimated at 2.1% by Rochester Epidemiology Project, 1990-2009. J Invest Dermatol. 2014;134(4):1141-1142. 5. Fricke ACV, Miteva M. Epidemiology and burden of alopecia areata: a systematic review. Clin Cosmet Investig Dermatol. 2015;8:397-403. 6. Rajabi F, Drake LA, Senna MM, Rezaei N. Alopecia areata: a review of disease pathogenesis. Br J Dermatol. 2018;179(5):1033-1048. 7. Simakou T, Butcher JP, Reid S, Henriquez FL. Alopecia areata: a multifactorial autoimmune condition. J Autoimmun. 2019:98:74-85. 8. Renert-Yuval Y, Guttman-Yassky E. The changing landscape of alopecia areata: the therapeutic paradigm. Adv Ther. 2017;34(7):1594-1609.
CASE #2 Lobular Capillary Hemangioma
Lobular capillary hemangioma (LCH), also known as pyogenic granuloma, is a benign vascular tumor that most commonly arises in tissues such as the skin and mucous membranes and occasionally is found subcutaneously, intravascularly, or in the gastrointestinal tract.1,2 It can develop spontaneously in sites of injury or within capillary malformations.1
Lobular capillary hemangioma typically begins as a small, red papule that may grow over weeks to months before stabilizing in size. The pedunculated papule is friable and often undergoes ulceration.3 A collarette of scales often is present at the base of the lesion, but this sign is not unique to this entity.3 The lesions can bleed profusely after unavoidable trauma.3 The diameter varies from a few millimeters to several centimeters, and satellite lesions may develop around a primary lesion or disseminate.3
Mucosal LCH is seen most commonly within the oral cavity.3 Cutaneous LCH typically appears on the trunk and extremities in adults and on the head and neck region in children.3
Histologic examination may be warranted to diagnose LCH. If the lesion is excised, it should be sent for histopathologic confirmation to rule out malignancy. Histologically, LCH consists of aggregates of capillary-sized vessels that develop within highly vascular granulation tissue with lobules of thin-walled capillaries embedded within a loose fibrous stroma.3 Scattered fibroblasts and inflammatory infiltrate often are found.3 The overlying epidermis is often thinned and develops erosion and ulceration in more severe cases.3 These lesions are neither pyogenic nor granulomatous; thus, the term pyogenic granuloma is not accurate. To correct the misleading terminology, Mills et al suggested using the more descriptive term lobular capillary hemangioma.4
Atypical lesions may necessitate the use of immunohistochemistry staining to rule out other causes.3 These lesions stain positive for vascular markers such as CD31, CD34, and factor VIII antigen but negative for glucose transporter-1, unlike infantile hemangioma.3,5
Pyogenic granuloma may occur in all age groups and there is no clear gender predominance.2 Reports conflict regarding the epidemiologic pattern of disease.2,6,7
The cause of LCH is unknown and no single pathway has been defined for pathogenesis of the lesions. Proposed mechanisms include an imbalance of proangiogenic and
Dermatology Clinic
antiangiogenic factors that leads to rapid proliferation of neovascular, friable, and lobular capillaries.3 Reactive granulation tissue from minor trauma and other predisposing factors, such as infection and preexisting vascular malformations, may contribute to pathogenesis.3 In pregnant patients, hormonal factors appear to play a role.2,8
Certain variants of LCH are associated with medication use. The most common medication-associated variant is multiple periungual pyogenic granuloma, which also is found in patients with other chronic dermatoses, such as atopic dermatitis and psoriasis.3 Drugs implicated most often include systemic and topical retinoids and antiretroviral, antineoplastic, and immunosuppressive agents.3 It is unclear whether the presenting patient had received chemotherapy or any other potentially causative agents previously because he did not follow up after biopsy and diagnosis.
The differential diagnosis of LCH should include certain red flag lesions such as melanoma, squamous cell carcinoma, basal cell carcinoma, angiosarcoma, and malignant lymphoma. In an immunosuppressed individual, bacillary angiomatosis and Kaposi sarcoma should be included in the differential diagnosis. Benign lesions that present similarly to LCH include other capillary hemangiomas, irritated nevi, Spitz nevi, warts, granulation tissue from minor trauma, glomus tumor, and angiolymphoid hyperplasia with eosinophilia.1,3 Histopathology will help differentiate these conditions.
Despite its benign nature, LCH may necessitate treatment to alleviate discomfort, bleeding, and/or obstruction of other structures. The selection of treatment should be made individually, depending on the size and severity of the lesions as well as patient characteristics and preferences. Treatment typically consists of excision, which results in the lowest rate of recurrence.1 Findings by Akamatsu et al suggest that shave excision followed by carbon dioxide laser ablation is linked to lower recurrence rates and is significantly less painful than excision and suture.9 Other treatments include curettage, electrocautery, radiosurgery, cryosurgery, sclerotherapy, and laser treatment. Successful photodynamic therapy with 5-aminolevulinic acid has been reported for a single LCH lesion on a finger; however, the advantage of this treatment compared with laser ablation has not been proven.1 ■
Lobular capillary hemangioma may necessitate treatment to alleviate discomfort, bleeding, or tissue obstruction.
Dominique Jacobs, BS, is a fourth-year medical student and aspiring dermatologist attending the Philadelphia College of Osteopathic Medicine; Katrina Hansen, DO, is the chief dermatology resident at Beaumont Farmington Hills in Michigan. Her interests include medical and surgical dermatology.
References
1. Wollina U, Langner D, França K, Gianfaldoni S, Lotti T, Tchernev G. Pyogenic granuloma - a common benign vascular tumor with variable clinical presentation: new findings and treatment options. Open Access Maced J Med Sci. 2017;5(4):423-426. 2. Plachouri KM, Georgiou S. Therapeutic approaches to pyogenic granuloma: an updated review. Int J Dermatol. 2019;58(6):642-648. 3. Sarwal P, Lapumnuaypol K. Pyogenic granuloma. In: StatPearls. StatPearls Publishing; 2021. Updated December 5, 2020. Accessed June 28, 2021. https:// www.ncbi.nlm.nih.gov/books/NBK556077 4. Mills SE, Cooper PH, Fechner RE. Lobular capillary hemangioma: the underlying lesion of pyogenic granuloma. A study of 73 cases from the oral and nasal mucous membranes. Am J Surg Pathol. 1980;4(5):470-479. 5. Seyedmajidi M, Shafaee S, Hashemipour G, Bijani A, Ehsani H. Immunohistochemical evaluation of angiogenesis related markers in pyogenic granuloma of gingiva. Asian Pac J Cancer Prev. 2015;16(17):7513-7516. 6. Harris MN, Desai R, Chuang TY, Hood AF, Mirowski GW. Lobular capillary hemangiomas: an epidemiologic report, with emphasis on cutaneous lesions. J Am Acad Dermatol. 2000;42(6):1012-1026. 7. Koo MG, Lee SH, Han SE. Pyogenic granuloma: a retrospective analysis of cases treated over a 10-year. Arch Craniofac Surg. 2017;18(1):16-20. 8. Jafarzadeh H, Sanatkhani M, Mohtasham N. Oral pyogenic granuloma: a review. J Oral Sci. 2006;48(4):167-175. 9. Akamatsu T, Hanai U, Kobayashi M, Miyasaka M. Pyogenic granuloma: a retrospective 10-year analysis of 82 cases. Tokai J Exp Clin Med. 2015;40(3):110-114.
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