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Papuloerythroderma of Ofuji presenting as a paraneoplastic phenomenon in myelodysplastic syndrome

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Papuloerythroderma of Ofuji (PEO) is characterised by flat-topped, red-brown papules that coalesce into an erythroderma with striking sparing of skin folds (the deckchair sign). It tends to affect elderly Japanese… Click to show full abstract

Papuloerythroderma of Ofuji (PEO) is characterised by flat-topped, red-brown papules that coalesce into an erythroderma with striking sparing of skin folds (the deckchair sign). It tends to affect elderly Japanese men, with various underlying aetiologies being implicated, including drugs (such as isoniazid, frusemide and aspirin), infections (e.g. HIV and enterobacteriaceae) and malignancy. The latter has been observed in up to 55% of PEO patients, underscoring the importance of recognising this rare dermatosis. We report a case of PEO associated with myelodysplastic syndrome bearing a cytogenetic abnormality with poor prognosis. A 77-year-old Chinese man with a history of hypertension that had been treated with amlodipine for the preceding 3 years presented with an 11-month history of pruritic rash. He had been treated for asteatotic eczema with topical steroids and emollients with partial response, with his rash becoming more extensive in the previous 7 months. A 1-week trial of prednisolone 20 mg daily provided only transient improvement in his condition. Physical examination revealed red-brown papules over his arms and thighs, confluent scaly plaques on the trunk, and prominent sparing of abdominal folds (Fig. 1a). A skin biopsy showed focal parakeratosis, moderate spongiosis and mild superficial perivascular infiltrates of mature lymphocytes (Fig. 1b), without evidence of epidermotropism or interface changes. Direct immunofluorescence was negative. A blood test revealed new-onset leukopaenia, atypical mononuclear cells and immature blasts. A bone marrow evaluation confirmed myelodysplastic syndrome-refractory anaemia with excess blasts, with a monosomy-7 cytogenetic abnormality. He was thus diagnosed with PEO associated with myelodysplastic syndrome. His serum immunoglobulin E and peripheral eosinophil and lymphocyte counts were normal. Although azacitidine was initiated for myelodysplastic syndrome, he remained dependent on transfusions and died 5 months later from pneumonia. First described in 1984, PEO remains under-recognised. The median duration from onset to diagnosis is

Keywords: paraneoplastic phenomenon; presenting paraneoplastic; myelodysplastic syndrome; papuloerythroderma ofuji; syndrome; ofuji presenting

Journal Title: Australasian Journal of Dermatology
Year Published: 2018

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