Lymphomatoid Papulosis Associated with Recurrent Cutaneous T-Cell Lymphoma

INTRODUCTION: Lymphomatoid papulosis is a chronic benign disease which may be associated with malignant lymphomas. This case illustrates the relapsing and remitting nature of both lymphomatoid papulosis and its potential of developing cutaneous T-cell lymphoma and narrow-band ultraviolet B (NB-UVB) phototherapy as a new modality of treatment of early-stage mycosis fungoides in these patients. CLINICAL PICTURE: A 44-year-old woman has had recurrent crops of papules and nodules of lymphomatoid papulosis on the limbs for 15 years. Histological features are consistent with the type B lesions of lymphomatoid papulosis. Eight years after the initial onset of these lesions she developed cutaneous T-cell lymphoma (mycosis fungoides). Since then, she has had recurrence of mycosis fungoides following the cessation of phototherapy, but had no evidence of systemic involvement. TREATMENT: The lesions of lymphomatoid papulosis responded to intermittent courses of oral methotrexate. Mycosis fungoides was treated with oral psoralen and ultraviolet A phototherapy with good response. Unfortunately, the lesions relapsed, whenever phototherapy was discontinued. The most recent recurrence of mycosis fungoides was treated with NB-UVB therapy. OUTCOME: The papules of lymphomatoid papulosis continue to appear but she remains free of lesions of mycosis fungoides, 10 months after cessation of NB-UVB therapy. CONCLUSION: Long-term surveillance is essential in all cases of lymphomatoid papulosis as accurate predictors for the development of malignant lymphoma in these individuals are still lacking.