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Diffuse cutaneous bullous mastocytosis with IgM deposits at dermo–epidermal junction
Author(s) -
Slavescu Kinga Cristina,
Chiorean Roxana,
Danescu Sorina,
Bota Madalina,
Rogojan Liliana,
Baican Adrian
Publication year - 2016
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/cup.12635
Subject(s) - cutaneous mastocytosis , urticaria pigmentosa , medicine , pathology , dermatology , systemic mastocytosis , biopsy , skin biopsy , disease
Cutaneous mastocytosis is a disease characterized by the infiltration and proliferation of mast cells in the skin. In children, the most common form of presentation is urticaria pigmentosa, while the diffuse cutaneous bullous mastocytosis is one of the rarest subtypes seen. The aim of this paper is to present a case of diffuse bullous mastocytosis with detection of IgM deposits at dermo–epidermal junction using direct immunofluorescence (DIF) microscopy. The diagnosis of diffuse bullous mastocytosis is a challenge, and DIF microscopy is necessary in order to exclude an autoimmune bullous disorder. However, IgM deposits at dermo–epidermal junction can be nonspecific, being found in a variety of skin disorders. A 6‐month‐old girl presented with bullous lesions and erosions on the scalp and the trunk. During hospitalization, further bullous lesions appeared, along with generalized erythrodermia. Skin biopsy revealed aspects of urticaria pigmentosa. Taking into account the clinical findings, the case was enclosed as bullous mastocytosis. Treatment included the avoidance of trigger factors, and administration of antihistamines along with a short‐term course of systemic steroids. The evolution was favorable, with remission of the existing lesions and without occurrence of new ones.