Lymphomatoid Papulosis from Childhood with Anaplastic Large-Cell Lymphoma of the Small Bowel

Lymphomatoid papulosis (LyP) is a lymphoproliferative disorder that exists on a spectrum of diseases with cutaneous CD30+ anaplastic large-cell lymphoma (ALCL). Multiple treatment options are available, although none are curative. The typical age of onset for LyP is in the third and fourth decades, but it has been seen occasionally in children. Lymphomatoid papulosis is associated with primary cutaneous ALCL and other lymphoproliferative malignancies, but is rarely associated with extranodal systemic ALCL. A 43-year-old man developed lymphomatoid papulosis lesions at 3 years of age, which persisted into adulthood, and he later developed ALCL of the duodenum. Treatment with standard CHOP (cyclophosphamide/doxorubicin/vincristine/prednisolone) chemotherapy resulted in complete remission of his gastrointestinal lymphoma and temporary improvement of his skin lesions. However, the LyP relapsed and proved refractory to psoralen plus ultraviolet-A phototherapy, and was only temporarily and partially responsive to bexarotene and denileukin diftitox.