All issues > Volume 38(7); 1995

Case Report

J Korean Pediatr Soc. 1995;38(7):988-992. Published online July 15, 1995.

A Case of Disseminated Pagetoid Reticulodsis

Hyang Mi HM Kwon¹, Sun Seong SS Park¹, Hye Lim HL Jung¹, Dong Hyuck DH Keum¹, Kye Yong KY Song²

¹Department of Pediatrics, Koryo General Hospital, Seoul Korea
²Department of Anatomical Pathology, Chung Ang University College of Medicine, Seoul, Korea

Abstract

Pagetoid reficulosis is a rare skin disorder consisting of localized(WoringerKolopp type) or disseminated (Ketron-Goodman type), asymptomatic hyperkeratotic, cutaneous plaque. The disseminated form has clinical similarities with mycosis fungoides and histologically characteri-sed by prominent infiltration of cytologically atypical mononuclear cells within a hyperplastic epidermis. It is suggested that the infiltrating cells orginate from T-lymphocytes. A 6yr old male patient visited with slowly disseminating, multiple erythematous skin rashes on whole body for one year. Skin biopsy showed dense infiltration of atypical mononuclear cells and lymphocytes within lower portion of epidermis. Dermal mononuclear cells are small but epidermal mononuclear cells are large lymphoreticular cells. Large lymphoreticular cells in epi-dermis show convoluted nucleus with prominent nucleoli and abundant cytoplasm. Immunohist-ologic studies were taken with Pan-T, Lysozyme, LCA(Leukocyte Common Antigen), 5-100. The mature lymphoid cells infiltrated in dermis showed positive reaction with Pan-T and Lyso-zyme, but atypical mononuclear cells infiltrated in epidermis showed negative reaction with all immunohistochemical stains. Electronmicroscopic finding of large cells show only few electron dense granules and poorly developed microorganells. With above feature we concluded that dermal lymphoid cells are reactive non neoplastic cells, while epidermal cells are neoplastic and T-cell lineage. Systemic chemotherapy was done and then he improved clinically.

Keywords :Pagetoid reticulosis, T-lymphocyte, Chemotherapy