A new case of diffuse cutaneous mastocytosis: diagnosis and management.

Abstract

Diffuse cutaneous mastocytosis (DCM) is a rare form of mastocytosis, usually arising within the first 3 years of life. In DCM mast cells infiltrate the entire skin. We report here a case of DCM in order to help clinicians in recognizing and properly managing the condition. The initial diagnosis of DCM may be difficult because it can present as reddish skin with widespread large bullae, yellow-orange infiltration and minimal small blisters and, less frequently, pachydermia and extremely folded skin. An isolated dermographism may be one of the first signs of neonatal DCM. Cutaneous mastocytosis is usually characterized by favorable prognosis and self-limited course but in DCM a higher risk of systemic involvement or life-threatening events has been reported. Until now there are no prognostic markers that predict systemic mastocytosis (SM) in children. Tryptase level is considered a marker of SM, but in DCM it may be increased even though systemic involvement is absent; therefore, a careful periodic clinical follow-up is needed.