Idiopathic papuloerythroderma of Ofuji in two patients

Dear Sir,

Papuloerythroderma of Ofuji, first described by Shigeo Ofuji in 1984, is a variant of erythroderma characterized by intensely pruritic erythematous papules and peripheral eosinophilia. The initial lesions first appear as papules, which assemble and become confluent to form erythematous, scaly plaques. The predominant sites of involvement are the flexor aspects of the extremities and trunk, with sparing of the skin folds.^[1] Generalized papular eruption in these cases, with sparing of the skin folds, has been described as the “deck-chair sign.”^[2] Histopathological findings usually seen in such cases include mixed upper dermal infiltrate, mostly comprising lymphocytes, histiocytes, and eosinophils, along with other variable changes in the epidermis and upper dermal vessel walls.^[1,2] The disease mostly affects the elderly.^[3]

A 58-year-old male patient presented to us in the outpatient department with the chief complaints of red raised lesions and severe itching for the past 2½ months. The lesions first appeared on the left arm, followed by the right arm, upper back, chest, and gradually spread to involve the whole body within 1 month. The lesions increased both in size and number over this period and were associated with itching, severe enough to interfere with the patient’s routine activities and sleep. On examination, multiple bilaterally symmetrical, erythematous papules and plaques covering about ninety percent of the body surface area were present, being more prominent on the extensor aspects of limbs and trunk with relative sparing of the axillae, genitalia, and more strikingly, the abdominal folds- positive “Deck-chair” sign [Figure 1a]. Laboratory investigations were normal except for a higher eosinophil count, which comprised about 10% of the total granulocyte count. Serum immunoglobulin E (Ig E) levels were in the normal range. Skin biopsy from the anterior abdominal wall showed mild acanthosis, spongiosis, and a mild increase in basal cell pigmentation in the epidermis with few lymphocytes in the epidermis with no epidermotropism. The dermis showed a perivascular chronic inflammatory cell infiltrate with a few eosinophils. The patient was treated with narrow-band ultraviolet B (UVB) therapy, antihistamines, and emollients with complete remission of the lesions at the end of 2 months.

Another 50-year-old male patient presented in the outpatient department with similar complaints of itchy, red, raised lesions, which first appeared on the extensor aspect of the left arm and gradually spread to involve the whole body within 6 months. On examination, multiple bilaterally symmetrical, erythematous, scaly plaques with fine scaling and a few erythematous papules were present on the whole body, with sparing of the flexures, specifically the abdominal folds, and a positive “Deck chair sign” [Figure 1b]. Laboratory investigations were normal, including the serum Ig E levels. Skin biopsy from the abdominal wall showed epidermal hyperplasia, acanthosis, spongiosis, dermal edema, and a perivascular inflammatory infiltrate with a few eosinophils. This patient was also treated with narrow-band UVB therapy with good results and resolution of the lesions within 1 month. Figure 2 showing histopathological findings observed. Immunohistochemistry of the biopsy sample showed 3+ positivity for CD3, 4+ positivity for CD4, 3+ positivity for CD7, and was negative for CD8 [Figure 3].

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Figure 1: (a) A 58-year-old male patient with diffuse erythematous papules with sparing of the abdominal folds- positive “deck chair” sign, and (b) Another patient with multiple erythematous scaly papules and plaques with sparing of the abdominal folds.

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Figure 2: Histopathological findings with mild acanthosis, spongiosis (yellow arrow), and a mild increase in basal cell pigmentation in the epidermis with few lymphocytes in the epidermis (blue arrow). Perivascular chronic inflammatory cell infiltrate with a few eosinophils in dermis (green arrow) (Hematoxylin and eosin, 100x).

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Figure 3: Immunohistochemistry panel (IHC X 10x) showing positivity for (a) CD3, (b) CD4, and (c) CD7.

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The disease is classified into four types, based on etiology: (1) Primary/Idiopathic. (2) Secondary (atopy, neoplasm, infections, drugs). (3) Papuloerythroderma imitating cutaneous T cell lymphoma. (4) Other diseases with positive deck chair sign but the absence of papules- pseudopapuloerythroderma. Diagnostic criteria were also described, which consisted of five major and five minor criteria. The five major criteria included: erythrodermic eruption with coalescent flat-topped, red brown papules; skin fold sparing; pruritus; histopathology excluding lymphoma and other skin diseases, and absence of triggering factors (infections, atopy, neoplasms, drugs). Furthermore, five minor criteria have been described, which include: Male sex; age above 55 years; peripheral and/ or tissue eosinophilia; elevated IgE levels, and peripheral leucopenia. The diagnosis of the idiopathic form requires the fulfillment of all five major criteria.^[1-3] Both these patients were diagnosed with the idiopathic form of papuloerythroderma of Ofuji as they fulfilled all the major criteria and were successfully treated with narrow band UVB, and were lost to follow-up.