Neonatal cutaneous mastocytosis

Neonatal cutaneous mastocytosis is rare infiltrative disorder of the skin. Though often asymptomatic, systemic features may be associated with any clinical pattern of the disorder at any age group. We present our experience with a 3 1/2 months old female child. She presented with recurrent eruption of tense bullae preceded by flushing, irritability since day 3 of life. Darier sign and dermatographism were present. Skin biopsy confirmed the case as urticaria pigmentosa which is the most common form of cutaneous mastocytosis. Investigation revealed deranged liver function. Child was given H1 antihistaminics and topical glucocorticoid. Family counselled about chance of anaphylaxis to various toxins and drugs and risk of development of systemic mastocytosis.