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Indian Pediatr 2013;50: 1073-1074

Childhood Bullous Mastocytosis


Dipti Das, Anupam Das and Swapan Sardar

Department of Dermatology, Medical College and Hospital, Kolkata, West Bengal, India.
Email: [email protected]
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A 1-year-old girl presented with numerous pruritic bullae all over the body. There were complaints of recurrent episodes of diarrhea and vomiting. Development was normal. She started developing episodes of intense itching at 8 months of age, followed by appearance of erythematous macules, plaques, and tense bullae at the sites of itching as well as other sites (scalp, trunk and upper extremities) (Fig. 1 and 2). Bullae were present on a non-urticated base containing clear fluid. Few of the urticarial plaques showed peau-d-orange appearance. Darier’s sign was positive. The palms, soles and mucosae were free. Routine investigations and urine analysis were normal. Skin biopsy showed sub-epidermal bulla and an upper dermal inflammatory infiltrate comprising lymphocytes and many mast cells. Toluidine blue staining showed metachromatic granules and a diagnosis of bullous mastocytosis was made. The patient was treated with antihistamines for itching and topical as well as systemic antibiotics for preventing secondary infection. Parents were counseled regarding the prognosis and course of disease and the importance of avoiding certain medications that may provoke mast cell degranulation.

Fig. 1 Multiple tense bullae and erosions over the trunk.

Fig.2 Bullae, plaques and erosions on face and trunk.

Bullous mastocytosis is a severe variant of mastocytosis, 30% cases manifesting within 6 months of age. The typical childhood disease is linked to Glu-839-Lyc c-kit mutation. Commonest clinical variants are telangiectasia macularis eruptiva perstans, mastocytoma, diffuse cutaneous mastocytosis and urticaria pigmentosa. Many agents stimulate the degranulation of mast cells, such as bacterial toxins, physical stimuli, poisons, biological peptides, polymers and drugs like aspirin, codeine, morphine, quinine etc. The close clinical differentials include chronic bullous disease of childhood, epidermolysis bullosa and staphylococcal scalded skin syndrome (SSSS). Combinations of H1 and H2 blocking agents have been the mainstay of treatment for most of the uncomplicated cases.

 

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