Pemphigus vulgaris with loss of hair on the scalp

Abstract

Pemphigus vulgaris is an autoimmune disease, which leads to flaccid bullae and erosions. Although the scalp is commonly involved, hair loss is rarely seen. We herein report a case of 32-year-old female patient with a 2-year history of pemphigus vulgaris, involving the skin and mucosa. Although clinical improvement was achieved with oral corticosteroids and mycophenolate mofetil, the patient developed tender plaques on her scalp, accompanied by hair loss. Histopathology and direct immunofluorescence were consistent with pemphigus vulgaris. Skin swab cultured Staphylococcus aureus. Treatment with oral and topical corticosteroids combined with mycophenolate mofetil resulted in clinical remission, with regrowth of scalp hair. Alopecia is an unusual clinical manifestation of pemphigus vulgaris. Our case supports the hypothesis that hair loss is induced by antibody-mediated outer root sheath keratinocyte acantholysis, in combination with skin infection, that together lead to the observed hair loss.

The Significance of Scalp Involvement in Pemphigus: A Literature Review

“…Scalp lesions in pemphigus may lead to alopecia. Nonscarring alopecia was reported to be an unusual manifestation of scalp pemphigus [ 24 ]. Veraitch et al described 5 patients with pemphigus vulgaris and progressive hair loss with alopecic patches [ 5 ].…”

“…The authors suggested that the combination of anti-desmoglein autoantibody-mediated acantholysis with a concomitant infection may cause the weakening of the hair follicle anchorage and result in alopecia [ 5 ]. Hadayer et al reported a case of a patient with pemphigus vulgaris, in whom alopecia was also accompanied by Staphylococcus aureus infection [ 24 ]. Our literature search revealed only one case report of nonscarring alopecia occurring in pemphigus foliaceus [ 25 ].…”

“…In pemphigus foliaceus, acantholysis is located under the corneal layer [ 25 ]. Acantholysis may also be observed in the structures of hair follicles ( Figure 4 ) [ 24 , 27 , 40 , 41 ]. Veraitch et al observed acantholysis between keratinocytes of the outer root sheath extending from the infundibulum to the suprabulbar level in anagen hair follicles of pemphigus vulgaris lesions [ 5 ].…”

The value of trichoscopy in the differential diagnosis of scalp lesions in pemphigus vulgaris and pemphigus foliaceus

“…4 , 5 Frequently, the scalp is the first site of clinical manifestation of the disease. 4 , 6 Isolated pemphigus lesions of the scalp have been reported in the literature. 7 - 9 Occasionally, scalp lesions may be extremely resistant to treatment.…”

“… 10 , 11 Scalp lesions in the course of pemphigus can lead to several types of non-cicatricial and cicatricial alopecia. 6 , 12 - 14 Thus, differential diagnosis of isolated scalp lesions and hair loss in the course of pemphigus…”