Diffuse cutaneous bullous mastocytosis with IgM deposits at dermo–epidermal junction

Abstract

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.

Granular IgM deposition at basement membrane zone in an infant with diffuse cutaneous mastocytosis

“…in a 6-month-old infant with bullous DCM. [ 10 ] Further studies in a large number of patients with DCM are required to confirm this finding.…”

“…Mast cell granules and protease–heparin proteoglycan complexes that are released from mast cells may also induce cleavage of the basal membrane components directly or by activation of collagenolytic matrix metalloproteinases leading to the formation of subepidermal bulla. [ 10 ]…”

Diffuse Cutaneous Bullous Mastocytosis and Disseminated Intravascular Coagulation Postvaccination

“…Extracutaneous complications such as lymphadenopathy, hepatomegaly, splenomegaly, and diarrhea have been reported in children with diffuse cutaneous mastocytosis. 17,18 Vomiting, hypotension, and vasodilatory shock have also been previously reported as potential complications. 18 Recently, an adverse complication of pulmonary infiltration and respiratory distress was reported in an infant with diffuse cutaneous mastocytosis.…”

“…17,18 Vomiting, hypotension, and vasodilatory shock have also been previously reported as potential complications. 18 Recently, an adverse complication of pulmonary infiltration and respiratory distress was reported in an infant with diffuse cutaneous mastocytosis. 19 More commonly, these extracutaneous symptoms occur in the systemic subtype of mastocytosis.…”