Neonatal bullous mastocytosis

I am reporting a 17-day old female neonate attended in an emergency with a history of generalized multiple bullous lesions all over the body with spontaneous ruptures of these bullous lesions resulting in erosions and healing with dark pigmentations. Provisional clinical diagnoses were bullous mastocytosis or congenital syphilis. Hematological investigations were against the diagnosis of congenital syphilis. The skin lesion’s biopsy with an array of histopathological staining and immunohistochemical typing confirmed the diagnosis of diffuse cutaneous erythrodermic mastocytosis. The attending clinician should know the process of evoking bullous mastocytosis in front of pruritic blisters associated with dermographism. Enlarged bullous form of the disease should be considered an emergency and dealt with urgency.