The clinicopathologic spectrum of lymphomatoid papulosis: study of 31 cases

Herein we review the Mayo Clinic experience with thirty-one cases of lymphomatoid papulosis seen since 1965. All patients had chronic, recurrent, and self-healing erythematous papulonodular lesions, which often became pustular, ulcerated, and resolved with scarring. The clinical features often corresponded to those seen in Mucha-Habermann disease; however, the predominant histopathologic feature was an infiltrate composed primarily of atypical lymphoid cells suggestive of malignant lymphoma. In six patients, a lymphoproliferative disorder was eventually diagnosed. There were two cases of mycosis fungoides (stage I), one case of nodular sclerosing Hodgkin's disease, and three cases of malignant lymphoma--one diffuse mixed large and small cell type with features of T-immunoblastic type, one diffuse large cell type, and one follicular small cleaved cell type. The clinical course of the lymphomatoid papulosis was unaffected by chemotherapy for the lymphoproliferative disorder. Our data indicate that, with sufficient duration of follow-up, malignant lymphoma may develop in some patients with lymphomatoid papulosis.