Bullous mastocytosis mimicking congenital epidermolysis bullosa

Abstract

A 2-month-old female infant was referred to DebRA Mexico from the Regional Children's Hospital because of a generalized dermatosis from birth characterized by multiple blisters and erosions on the trunk, face and limbs, associated with minor trauma. A skin biopsy showing subepidermal blisters associated with a dermal infiltrate of Giemsa-positive cells and CD117-positive antibody was consistent with the diagnosis of bullous mastocytosis. Treatment with oral antihistamines, topical steroids, and antibiotics was initiated, leading to a remission of the lesions.

Keywords: Bullous epidermolysis; Bullous mastocytosis; Darier's sign.

Fig. 1

a Erosions on the chest and left arm. b Hemorrhagic blisters with crusts suggestive of EB. Note the presence of erythema around the maculas after the friction characteristic of Darier's sign. c Ulcer of the left leg, 127 × 101 mm (300 × 300 DPI).

Fig. 2

a Monocellular infiltrates are observed in the papillary and reticular dermis, associated with subepidermal multicenter blisters. HE. ×10. b Subepidermal blister without inflammatory infiltrate. HE. ×40. c CD117-positive infiltrate, dermis cells (×20). d Subepidermal blister associated with CD117-positive mast cells (×40). 106 × 127 mm (300 × 300 DPI).