Childhood pemphigus foliaceus. Report of a case

Abstract

A case is reported of a 4-year-old black boy with pemphigus foliaceus. The patient is unusual because of age, sex, race, and distribution of lesions, Confirmation of diagnosis was made by both routine histopathology and direct immunofluorescence microscopy. The patient responded rapidly to prednisone therapy.

Eye and lid involvement as an uncommon feature of pemphigus foliaceus in a pediatric patient

“…Less frequently, the groin, pubis, and lower extremities were involved in a few patients. 3,[14][15][16][17] Palpebral involvement has been reported in just two cases, without mention of any ocular symptoms or apparent association with greater disease severity. 13,18 Systemic or topical steroids were generally first-line treatment.…”

“…The most frequent locations were the scalp and face, followed by trunk and upper extremities. Less frequently, the groin, pubis, and lower extremities were involved in a few patients 3,14–17 . Palpebral involvement has been reported in just two cases, without mention of any ocular symptoms or apparent association with greater disease severity 13,18 .…”

Juvenile pemphigus foliaceus

“…PF, a less severe variant of pemphigus, is a rare disease, especially in the first 14 years; it affects both sexes equally 5 , 7 –10 Despite its rarity, however, it is more frequent in Brazil 11 and India 12 . Adult PF has been reported to be associated with other autoimmune disorders 13 , 14 and drugs, 15 but Graves' disease is the only condition reported to coexist with childhood PF 9 …”

“…The onset is often slow, with crusting, yellowish, scaling lesions starting on the face and scalp, as in our case, 8 which can mimic impetigo and seborrhoeic dermatitis; unlike PV, the oral mucosa is rarely involved 5 ,. 7 Histologically, PF is characterized by acantholysis high in the epidermis, either subcorneally or in the stratum granulosum 16 . These superficial changes are similar in PF and pemphigus erythematosus (PE) but, clinically, PE may simulate lupus erythematosus 17 ,.…”

“…PF, the less severe form of pemphigus, may be treated simply with topical steroids or low‐dose corticosteroids; 7 the more generalized form of PF has been treated with high‐dose corticosteroids 9 ,. 25 However, Basset et al 26 .…”