홍피증과 어린선양 피부 병변을 보인 균상식육종

Abstract

Clinical manifestations of mycosis fungoides can vary from the classical type, which is associated with patch, plaque and tumor, to the rare atypical type which is associated with erythroderma, hypopigmentation or hyperpigmentation, and an ichthyosiform patch. A 45-year-old man presented with a 10-year history of asymptomatic erythroderma and an ichthyosiform patch on his trunk and lower extremities. On physical examination, there were asymptomatic erythematous nodules on the Rt. thigh and a palpable lymph node on the Lt. popliteal area. A histopathologic study showed infiltration of atypical cells into the upper dermis with mononuclear cell infiltration of the epidermis. On immunohistochemistry, atypical cells showed positivity for CD3, CD4, and CD8 staining, and negativity for CD20 staining. TCR-gamma rearrangement showed monoclonality. Excisional biopsy of Lt. popliteal lymph nodes showed pathology findings compatible with that of the trunk. A diagnosis of stage IVa mycosis fungoides, which is associated with erythroderma and ichthyosiform manifestations, was made. The patient was treated by a combination of PUVA and MTX chemotherapy and is still on regular follow up.