Pediatric Dermatology Consult

What's your diagnosis?

An 11-year-old female is seen in clinic with a 3-year history of alopecia. The patient recently immigrated to the United States from Afghanistan. Prior to immigrating, she was evaluated for "scarring alopecia" and had been treated with oral and topical steroids as well as oral and topical antifungals. When active, she had itching and tenderness. She is not actively losing any hair at this time, but she has not regrown any of her hair. The patient has no family members with alopecia. She reports some burning pain and itching of her scalp, and denies any muscle pain or weakness or sun sensitivity.


On physical exam, you see 50% loss of hair on the superior scalp with preservation of the anterior hair line. Patches of hair can be seen throughout, with segments of smooth-skinned alopecia, without pustules. There is a loss of the follicle pattern in scarred areas, and magnification or "dermoscopy" shows perifollicular erythema and scaling at the border of the affected scalp. Labs are all within normal limits. Bacterial and fungal cultures of the scalp do not grow organisms.

Your diagnosis is:

Telogen effluvium

Lichen planopilaris

Alopecia areata

Discoid lupus erythematosus

Trichotillosis

Given the longstanding scarring alopecia, with negative fungal cultures and with perifollicular erythema and scaling, this diagnosis is most consistent with lichen planopilaris.

Lichen planopilaris (LPP) is considered one of the primary scarring alopecias, a group of diseases characterized by inflammation and subsequent irreversible hair loss.1 LPP specifically is believed to be caused by dysfunction of cell-mediated immunity, resulting in T lymphocytes attacking follicular hair stem cells.2 It typically presents with hair loss, pruritus, scaling, burning pain, and tenderness of the scalp when active,1,3 with exam showing perifollicular scale and erythema on the borders of the patches of alopecia.4,5 Over time, scarring of the scalp develops with loss of follicular ostia.1 Definitive diagnosis typically requires punch biopsy of the affected scalp, as such can determine the presence or absence of inflammation in affected areas of the scalp.1

What’s the treatment plan?

Given that LPP is an autoimmune inflammatory disease process, the goal of treatment is to calm down the inflammation of the scalp to prevent further progression of a patient’s hair loss. This is typically achieved with superpotent topical corticosteroids, such as clobetasol applied directly to the scalp, and/or intralesional corticosteroids, such as triamcinolone acetonide suspension injected directly to the affected scalp.3,6,7 Other treatment options include systemic agents, such as hydroxychloroquine, methotrexate, mycophenolate mofetil, pioglitazone, and doxycycline.3,6 Hair loss is not reversible as loss of follicular ostia and hair stem cells results in permanent scarring.1 Management often requires a referral to dermatology for aggressive treatment to prevent further hair loss.

What’s the differential diagnosis?

The differential diagnosis of lichen planopilaris includes other scarring alopecias, including central centrifugal cicatricial alopecia, discoid lupus erythematosus, folliculitis decalvans. While nonscarring, alopecia areata, trichotillomania, and telogen effluvium are discussed below as well.

Dr. Michael Haft, University of California, San Diego

Dr. Michael Haft

Central centrifugal cicatricial alopecia is very rare in pediatrics, and is a type of asymptomatic scarring alopecia that begins at the vertex of the scalp, spreading centrifugally and resulting in shiny plaque development. Treatment involves reduction of hair grooming as well as topical and intralesional steroids.

Discoid lupus erythematosus presents as scaling erythematous plaques on the face and scalp that result in skin pigment changes and atrophy over time. Scalp involvement results in scarring alopecia. Treatment includes the use of high-potency topical corticosteroids, topical calcineurin inhibitors, and hydroxychloroquine.

Folliculitis decalvans is another form of scarring alopecia believed to be caused by an inflammatory response to Staphylococcus aureus in the scalp, resulting in the formation of scarring of the scalp and perifollicular pustules. Treatment is topical antibiotics and intralesional steroids.

Alopecia areata is a form of nonscarring alopecia resulting in small round patches of partially reversible hair loss characterized by the pathognomonic finding of so-called exclamation point hairs that are broader distally and taper toward the scalp on physical exam. Considered an autoimmune disorder, it varies greatly in extent and course. While focal hair loss is the hallmark of this disease, usually hair follicles are present.

Dr. Lawrence F. Eichenfield is the vice chair of the department of dermatology and a professor of dermatology and pediatrics at the University of California, San Diego

Dr. Lawrence F. Eichenfield

Trichotillosis, also known as trichotillomania (hair pulling), results in alopecia with irregular borders and broken hairs of different lengths secondary to the urge to remove or pull one’s own hair, resulting in nonscarring alopecia. It may be associated with stress or anxiety, obsessive-compulsive disorders, or other repetitive body-altering behaviors. Treatments include reassurance and education as it can be self-limited in some, behavior modification, or systemic therapy including tricyclic antidepressants or SSRIs.

Our patient underwent scalp punch biopsy to confirm the diagnosis and was started on potent topical corticosteroids with good disease control.

Dr. Haft is a pediatric dermatology research associate in the division of pediatric and adolescent dermatology, University of California, San Diego, and Rady Children’s Hospital, San Diego. Dr. Eichenfield is the vice chair of the department of dermatology and a professor of dermatology and pediatrics at the university, and he is chief of pediatric and adolescent dermatology at the hospital. Neither of the doctors had any relevant financial disclosures. Email them at [email protected].

References

1. J Am Acad Dermatol. 2005 Jul. doi: 10.1016/j.jaad.2004.06.015.

2. J Pathol. 2013 Oct. doi: 10.1002/path.4233.

3. Pediatr Dermatol. 2015 Sep-Oct. doi: 10.1111/pde.12624.

4. J Am Acad Dermatol. 2004 Jan. doi: 10.1016/j.jaad.2003.04.001.

5. J Am Acad Dermatol. 1992 Dec. doi: 10.1016/0190-9622(92)70290-v.

6. Clin Cosmet Investig Dermatol. 2018 Feb 27. doi: 10.2147/CCID.S137870.

7. Semin Cutan Med Surg. 2009 Mar. doi: 10.1016/j.sder.2008.12.006.

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