Diffuse cutaneous mastocytosis

Published on 11/06/2015 by admin

Filed under Pediatrics

Last modified 22/04/2025

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Diffuse cutaneous mastocytosis. Linear blisters from stroking the infant’s skin.

Diffuse cutaneous mastocytosis with thickened, leathery yellowish skin.

CLINICAL FEATURES

Diffuse cutaneous mastocytosis presents as blistering in the neonatal period. Involvement is generalized and discrete hyperpigmented macules and papules as in urticaria pigmentosa are not seen. The blisters are often large and leave large denuded erosions. After a severe blistering episode, 5−10 days may be required to regranulate mast cells. Marked dermographism is present. With time, the skin becomes thickened, pink to yellow, with a doughy feel and a texture resembling an orange peel. Recurrent bullae, intractable pruritus and flushing attacks are common. Severe syncopal episodes and death from prolonged hypotension have been reported.

Gastrointestinal symptoms are seen as the child becomes older, including chronic diarrhea and recurrent episodes of vomiting associated with abdominal pain. Gastrointestinal stromal tumors may appear in adults.

TREATMENT

Oral H1 antihistamines are the first line of therapy. Cetirizine 2.5 to 10 mg given every morning with hydroxyzine 1 mg/kg at bedtime is an excellent initial therapy. Alternatively, hydroxyzine 1 mg/kg may be given every 6 hours. H2 antihistamines and oral cromolyn sodium may be valuable in infants with gastrointestinal symptoms. Phototherapy with narrow band UVB or psoralens plus UVA (PUVA) twice a week will minimize symptoms.