Papuloerythroderma of Ofuji

Excerpt

Papuloerythroderma of Ofuji is a rare skin disorder most commonly found in Japan. It is characterized by polygonal, flat-topped erythematous-brown papules coalescing into sheets that often cover the entire skin surface but spare the skin folds. Papuloerythroderma of Ofuji was first discovered in Japan in 1984 by Ofuji, Furukawa, Miyachi, and Ohno. It is commonly accompanied by pruritus, peripheral eosinophilia, and elevated immunoglobulin E (IgE).

The basis of the disease and initial diagnosis stems from an initial meta-analysis comprised of 170 patients via Torchia et al, who proposed a preliminary set of 5 major and 5 minor criteria that define Papuloerythroderma of Ofuji. These criteria help determine etiology and allow for categorization into 4 subtypes, which include primary, secondary, and a nonpapular form of Papuloerythroderma of Ofuji. There is also a consideration for patients that appear clinically, like Papuloerythroderma of Ofuji, but have a diagnosis more consistent with lymphoma.