전암성 병변으로 알려진 Type D 림프종양구진 환아 1예
A case of Lymphomatoid Papulosis Type D in a Child
Abstract
Lymphomatoid papulosis is a papulonodular skin lesion with histologic features of a CD30-positive lymphoid proliferation of atypical T-cells. Lymphomatoid papulosis usually occurs in adults, and rarely in children. Lymphomatoid papulosis is divided into type A, B, C, D, and E depending on histologic features. Lymphomatoid papulosis type D is known as a premalignant lesion which can progress to T-cell type skin or systemic lymphoma. We report a case lymphomatoid papulosis type D developed in a young boy. A 13-year-old boy who had a history of atopic dermatitis presented with generalized erythematous to brownish papulonodular skin lesions on trunk, axilla and face for two weeks. The patient was treated under diagnosis of viral infection or allergic reaction at a local Dermatology clinic, but many brown-colored papules and nodules newly appeared on penile shaft and scrotum. The patient was then transferred to the department of Dermatology, Kangbuk Samsung hospital. At the time of visit to our hospital, he complained fatigue but had no other systemic symptoms such as fever, sweats or weight loss. The results of blood tests including CBC, RBC index, chemistry, LDH were normal except low serum iron, TIBC and ferritin. Chest PA and abdominal ultrasonography did not show thymic enlargement or hepatosplenomegaly, respectively. Punch biopsy of skin lesion was done from the groin and penile shaft, and histopathologic examination revealed an spongiotic dermatitis and acanthotic epidermis. Gomori methenamine silver stain revealed no specific microorganism. Various kinds of immunostains were done to exclude histiocytic proliferative disease and T-cell lymphoma, and the patient was finally diagnosed as “CD8-positive lymphomatoid papulosis (type D)”, a less aggressive form of CD30-positive lymphoproliferative lesion, but a premalignant disease. The patient was treated with oral 2nd generation cephalosporin, minocycline, ibuprofen, and antibiotic ointment for the skin lesion at the department of Dermatology, and oral ferrous sulfate treatment for iron deficiency at the department of Pediatrics. The skin lesion on whole body improved without surgical removal, and iron profile normalized after 3 months of iron treatment. The patient is recommended to be examined regularly by Pediatric hemato-oncologist for long period.