Lymphomatoid Papulosis : A Clinical , Histopathologic and Follow - up Study / 대한피부과학회지

ABSTRACT

BACKGROUND: Lymphomatoic papulosis, a disease with recurrent and hemorrhagic papu-lonodular lesions, shows a benign course clinically but lymhomatous features histopathologically. Although sporadic cases have been reported, we could not find any comprehensive report of clinical and histopathologic features of lymphomatoid papulosis in Korean literature. OBJECTIVE: The purpose of this study was to examine the clinical and histopathologic features and follow up observation of 16 patients with lymphomatoid papulosis. METHODS: We reveiwed the clinical and histopathologic findings, associated diseases, and response to various treatments in 16 patients with lymphomatoid papulosis during the 10 year period from January 1984 to May 1994. RESULTS: The results were summarized as fallows. 1. There were 14 males and 2 females. Sex distribution showed a male predominence with male female ratio of 7 1. 2. Age distribution ranged from 20 to 65, with the mean age of 43 years. 3. Duration of symptoms ranged from 4 days to 10 years, with a mean duration of 2.4 years. 4. The lesions were located on the trunk and extremities(7 cases), extremities(7 cases), and trunk(2 cases). 5. The morphology of lesions were papules or plaques(11 cases), and nodules(5 cases). 6. Associated diseases were tuberculosis(2 cases), Behcet's disease(2 cases), Klatskin tumor(1 case), osteomyelitis(1 case), hypertesion and peptic ulcer(1 case), and chronic active hepatitis(1 case). 7. Histopathologic features revealed wedge-shaped superficial and deep perivascular polymorphic infiltrate with pleomorphic cells in all specimens except one. Histopathologic types classified by Willemze's Classification were type A(9 cases), type B(6 cases), and type A and type B(1 case). 8. Treatment including PUVA, methotrexate and systemic steroid had beneficial effect only temporarlly. 9. In one patient, large anaplastic CD30 lymphoma(Ki-1 lymphoma) of the skin and inguinal lymph node developed 3 years after the onset of Lymphomatoid papulosis. CONCLUSION: All pat.ients with lymphomatoid papulosis should be followed up for a long-term period to rule out the development of lymphoma.