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A Case of Disseminated Pagetoid Reticulodsis

Journal of the Korean Pediatric Society 1995;38(7):988-992.
Published online July 15, 1995.
A Case of Disseminated Pagetoid Reticulodsis
Hyang Mi Kwon1, Sun Seong Park1, Hye Lim Jung1, Dong Hyuck Keum1, Kye Yong Song2
1Department of Pediatrics, Koryo General Hospital, Seoul Korea
2Department of Anatomical Pathology, Chung Ang University College of Medicine, Seoul, Korea
Disseminated Pagetoid Reticulosis 1례
권향미1, 박순성1, 정혜림1, 금동혁1, 송계용2
1서울 고려병원 소아과
2중앙대학교 의과대학 병리학교실
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.
Key Words: Pagetoid reticulosis, T-lymphocyte, Chemotherapy


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