Acrodermatitis enteropathica-like eruption in metabolic disorders: acrodermatitis dysmetabolica is proposed as a better term.

BACKGROUND: Acrodermatitis acidemica is a recently proposed term for the rash that is similar to acrodermatitis enteropathica, which is encountered in organic acidemias. However, acrodermatitis enteropathica-like eruption may be seen in metabolic disorders other than organic acidemias. OBJECTIVE: The aim of this study was to evaluate the clinical features of acrodermatitis enteropathica-like eruption secondary to metabolic disorders. METHODS: Clinical and demographic features of 12 patients with acrodermatitis enteropathica-like eruption were prospectively evaluated between 2004 and 2006 in this single-center study. RESULTS: Among the 12 patients, underlying metabolic disorders included maple syrup urine disease (n = 5), methylmalonic acidemia (n = 3), phenylketonuria (n = 2), ornithine transcarbamylase deficiency (n = 1), and propionic acidemia (n = 1). Mean age at first presentation was 29.9 months. Mean duration of acrodermatitis enteropathica-like eruption at the time of presentation was 25.2 days. The diaper area was involved in all presentations. Plasma zinc level was measured in 62.5% (n = 10) of the presentations and all had normal levels. All phenylketonuria cases had a low plasma phenylalanine level, and a low plasma isoleucine level was observed in the propionic acidemia case and all maple syrup urine disease cases. The rash responded dramatically to appropriate diet management in all cases. CONCLUSION: In this study, acrodermatitis enteropathica-like eruption was noted in various metabolic disorders, including organic acidemias. We suggest that acrodermatitis dysmetabolica might be a better term for acrodermatitis enteropathica-like eruption occurring secondary to metabolic disorders other than acquired zinc deficiency.

Extranodal type T/NK-cell lymphoma with an atypical clinical presentation.

Peripheral-type natural killer (NK)- or T-cell lymphomas are rare disorders characterized with clonal proliferation of mature lymphocytes. They have been linked to chronic and active Epstein-Barr virus infection (CAEBV), which itself is not defined as a malignant hematological disorder. The authors present a patient with T/NK-cell lymphoma involving skin, kidneys, spleen, pancreas, and meninges. She was remarkable for having the mosaic feature of more than one type of extranodal T/NK-cell lymphoma. She also had mixed findings of CAEBV that might have been attributed both to hypersensitivity to mosquito bites and to hemophagocytic lymphohistiocytosis.