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Abstract
Diffuse cutaneous mastocytosis (DCM) is a rare variant of mast cell disease with widespread erythema and is clinically apparent in early infancy. We report the case of a 1-day-old female neonate who presented with diffuse flush, pruritus, and extensive blistering. DCM was diagnosed by immunohistochemical staining with anti-CD117, which revealed mast cell infiltration. DCM is a severe and heterogeneous cutaneous disease, and is associated with mast cell mediator-related symptoms and risk of anaphylactic shock. We describe this case and provide the first literature review of neonatal onset DCM in Korea.
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Fig. 1.
Clinical presentation: Patient at birth with diffuse erythematous rash on back (A). Partly hemorrhagic vesicle and bulla on axilla (B). Hemorrhagic crusts and erosion found on the face (C). Diffuse cutaneous mastocytosis with erythema and leathery thickened skin on abdomen (D).
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