Subject(s)
Anti-Allergic Agents/therapeutic use , Antibodies, Monoclonal/therapeutic use , Dermatitis, Atopic/drug therapy , Antibodies, Monoclonal, Murine-Derived , Antigens, CD20/drug effects , Dendritic Cells/drug effects , Dermatitis, Atopic/blood , Dermatitis, Atopic/immunology , Eosinophils/drug effects , Humans , Immunoglobulin E/blood , Immunoglobulin E/drug effects , Plasma Cells/drug effects , Rituximab , T-Lymphocytes/drug effectsABSTRACT
Diffuse cutaneous mastocytosis (DCM) is a rare, severe, variant of cutaneous mastocytosis. The authors report the case of a male infant who developed maculae and maculopapulae on his legs and abdomen when aged 3.5 months, which spread to all body surfaces within weeks. Diagnosis of DCM was made at the age of 6 months when he had developed extensive bullous eruptions, generalised pruritus, flushing and abdominal pain. Treatment was started with oral dimethindine maleate. At the age of 18 months, oral sodium cromoglicate (SCG) was introduced. At the age of 23 months, additional treatment was started with a cutaneous emulsion containing 4% SCG. Continued treatment with oral dimethindine maleate, oral SCG with the dose maintained at 25 mg/kg/day, and SCG 4% cutaneous emulsion applied two to four times daily has resulted in a steady improvement of symptoms and skin appearance.
Subject(s)
Cromolyn Sodium/administration & dosage , Mastocytosis, Cutaneous/drug therapy , Administration, Oral , Administration, Topical , Humans , Infant , MaleABSTRACT
A segmental distribution of pigmented purpura (PP) is rare. Our patient presented at 5 years of age with red-brown petechial macules and telangiectases on her chest, the inner and dorsal aspects of the right upper extremity, and the dorsal aspect of the hand, which had developed slowly over the previous 7 months. The lesions were intermittently pruritic. A biopsy from the right arm revealed discrete hydropic degeneration of the basal layer of the epidermis, dilation of the blood vessels, numerous extravasated erythrocytes and lymphocytes, and lymphocytes in the lower part of the epidermis. The skin disease resolved almost completely within 18 months. The general physical examination revealed an open foramen ovale, slight hypogammaglobulinemia (G and A), and enhanced fragility of the blood vessels.