Fig. 13.1
Upper panel reveals centrally confluent hairless smooth patches, characteristic of lichen planopilaris. Lower panel reveals closely set hairless patches with scaling and excoriations in a patient with discoid lupus erythematosus
Case
A patient presents with multiple patches of complete alopecia over the scalp.
Clinical differential diagnosis is well known to dermatologists and is divided between so-called nonscarring and scarring alopecia. As the term “scarring” refers to a specific type of dermal fibrosis, which lacks in most disorders referred to as scarring alopecia, a better term may be irreversible or permanent patchy alopecia. Reversible or nonpermanent patchy alopecia in almost all cases is alopecia areata. Permanent patchy alopecia is most often
lichen planopilaris, LPP and less frequently
DLE
pseudopelade, and
folliculitis decalvans.
Clinical Clues
Alopecia areata is easily recognizable by its history of rather acute onset, early age of onset, randomly scattered patches, no loss of the follicular ostia, presence of exclamation hairs, frequent spontaneous regrowth of hair, and the occasional presence of patches of hair loss over the face.
LPP tends to favor middle-aged women and has an insidious onset; hence, patients present to a dermatologist several months after the onset of hair loss. It also strongly favors the vertex and crown of the scalp, may be rarely associated with other manifestations of lichen planus including genital lesions and facial papules, and upon examination, reveals the characteristic “footsteps in the snow” appearance of generally 1–1.5 cm round to oval patches of invariably complete alopecia, surrounded by a narrow rim of activity consisting of pinpoint-to-1 mm, pinkish to violaceous papules made up of keratin in the follicular ostium. The combination of these findings is so characteristic that a biopsy may not be required.
DLE may cause smooth patches of cicatricial alopecia only rarely. I have seen around 200 patients with DLE, among whom only one had smooth patches of hair loss limited to the scalp and with a smooth surface. In general, the surface of lesions of DLE is scaly, often with follicular keratotic plugging, and diffuse erythema in addition to induration, especially at the borders. To my surprise, in the patient referred to above, the histological findings of more than one biopsy specimen did reveal basal vacuolization and a superficial and deep lymphocytic infiltrate with mucin characteristic of acute DLE . The other epidermal findings of DLE were all absent; hence, contributing to the smooth surface of lesions.
Pseudopelade is a historical term , which was initially coined to describe a disorder that looked grossly like alopecia areata but, in which, hair loss was permanent and irreversible. As per the initial description, especially in the last three decades, the nosology of pseudopelade has been debated extensively.
Some adhere to the view that pseudopelade is a clinical descriptive term rather than a true disorder, and that the term should apply to end-stage patchy permanent alopecia whether it results from LPP , DLE, folliculitis decalvans, or other disorders. In this view, the term may be deleted from the dermatology lexicon or used in cases for which the exact etiology of patchy cicatricial alopecia is not known at the time.