By Gary M. White, MD
Bullous mastocytosis is a variant of mastocytosis in which diffuse congenital deposition of mastocytes results in bulla formation. The term diffuse cutaneous mastocytosis with bulla and bullous mastocytosis are synonyms.
- If onset is at birth or in the first few weeks of life, there is often systemic involvement and death may occur.
- Potential complications include electrolyte and fluid loss, shock, GI bleeding, infection and mast cell leukemia. In contrast, delayed onset has a much better prognosis.
- See also solitary mastocytoma, urticaria pigmentosa, adult, diffuse mastocytosis and TMEP.
Bulla distributed diffusely in an infant is characteristic. The bulla may be hemorrhagic. Darier sign is positive. The skin may be bright red or hyperpigmented. There may be generalized thickening. Sessile papules or nodules may also be found. Typically absent are other classic lesions of urticaria pigmentosa. Some newborns with this disease have been thought to have staphylococcal scalded skin syndrome.
- Oral antihistamines.
- Avoidance of triggers of mast cell degranulation.
Excessive friction or changes in temperature should be avoided. Antihistamines and topical antibacterial ointments may be helpful. Drugs which may cause degranulation should be avoided. This list includes dextromethorphan which is related to codeine and can be found in over-the-counter cough syrups for children [PD 1996;13;410]. PUVA and cromylyn have been used.
A 1-year-old girl. Indian Pediatrics
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