Papuloerythroderma of ofuji-is it a homogeneous entity?

Introduction

Papuloerythroderma of Ofuji is a rare intensely pruritic generalized eruption of unknown etiology characterized by peripheral eosinophilia and typical deck chair sign.[1] Largely a clinical diagnosis, the disorder has also been shown to be variably associated with lymphopenia, high serum IgE level, Langerhans cells in the dermis, and even circulating Sezary cells.[2] Biopsy usually reveals a mixed upper dermal infiltrate, mostly comprising of lymphocytes, histiocytes and eosinophils. Variable pathological changes are also evident in the epidermis and vessel walls in upper dermis. Following is description of a similar case with some variation from the usual.

Case Report

A 70-year-old man presented in the Dermatology out patient department with a generalized erythematous eruption associated with intense itching. The eruption had started on the trunk ten years back, and within a span of two years had covered the entire body.

Cutaneous examination revealed erythroderma composed of coalescing papular lesions, but remarkably sparing the body folds, especially anterior transverse abdominal ones - the typical deck chair sign. Excoriations were a pointer towards itchy nature of the lesions. There was no lymphadenopathy or cutaneous infarcts, though few lesions of senile purpura were evident on the dorsa of forearms.

Laboratory investigations showed marked eosinophilia of 11%, while serum IgE level was in normal range. Skin biopsy from the anterior abdominal wall showed hyperkeratosis, acanthosis, focal spongiosis, moderate upper dermal infiltrate composed of lymphocytes and few eosinophils, and mild vessel wall dilatation. No exocytosis, vessel wall thickening or extravasation of red blood cells as seen. In the past, the patient had intermittently taken treatment, the nature of which was unknown. He was treated with oral prednisolone 40 mg daily (gradually tapered), antihistamines, topical betamethasone dipropionate and emollients. Improvement was evident in the form of partial remission of eruption at the end of one month.

Discussion

The presence of papuloerythroderma, deck chair sign and eosinophilia in the above case favour the diagnosis of Ofuji′s disease. But contrary to the cases reported before, this patient had no evidence of high serum IgE level, lymphopenia, exocytosis, vessel wall thickening or extravasation of red blood cells. Apart from this, associated variable clinical features like lymphadenopathy, nailfold and buttock infarcts and palmoplantar keratoderma, visceral malignancies, and laboratory features like circulating Sezary cells, as mentioned by many authors, were also absent.[3],[4],[5] This points to heterogeneity of the disease, which tends to manifest in more than one form, or may be the cases represent variants of a single disorder. This question will remain unanswered till the root cause of this rare enigmatic entity comes to light.