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Bullous Mastocytosis Presenting as Chronic Bullous Disease of Childhood
Author(s) -
Goldenberg G.,
Pichardo R.,
Jorizzo J.,
Sangueza O.
Publication year - 2005
Publication title -
journal of cutaneous pathology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.597
H-Index - 75
eISSN - 1600-0560
pISSN - 0303-6987
DOI - 10.1111/j.0303-6987.2005.320cf.x
Subject(s) - medicine , dermatology , pathology , dermis , dapsone , langerhans cell histiocytosis , bullous pemphigoid , direct fluorescent antibody , disease , antibody , immunology
We report a 6‐month old male infant who presented to our pediatric dermatology clinic with a generalized bullous eruption since three months of age. A sepsis work up performed prior to presentation did not reveal any evidence of infection. Clinical presentation revealed multiple tense bullae in a “string of pearls” arrangement and multiple erosions on the bilateral upper extremities and trunk. This clinical presentation was strongly suggestive of chronic bullous disease of childhood. Laboratory evaluation revealed a normal complete blood cell count and glucose‐6‐phosphate dehydrogenase level. Polymerase chain reaction for herpes simplex virus types I and II was negative. Histological sections demonstrated a large space of separation between the epidermis and dermis which was filled by a monomorphous infiltrate composed of round to oval cells with centrally placed nuclei, consistent with mast cells. Leder and c‐kit stains were strongly positive confirming the diagnosis of bullous mastocytosis. Direct immunofluorescence did not reveal IgA deposits or any other findings. Treatment included fluocinonide 0.05% cream QAM and tacrolimus 0.1% ointment QHS to active lesions, and silver sulfadiazine 1% cream to erosions. Improvement was noted during follow up examination.