Abstract
A 45-year-old man was diagnosed with atopic dermatitis when he was 15 years old. He has received treatments intermittently but showed an insufficient response. He developed a tumor on his back six months prior to presentation in our hospital, and more tumors developed gradually. At his visit to our hospital, a physical examination revealed xerosis of his whole body, scaly erythema, pigmentation, many scratch marks, and four reddish nodules or tumors on his back and waist. Skin biopsy showed infiltration of large abnormal lymphocytes from the dermis to subcutaneous tissue. Immunohistochemically, the tumor cells were positive for CD3, CD4, CD30, and CD45RO, but were negative for CD8, CD20, and ALK. Southern blotting revealed gene rearrangement of TCR-Cb1. PET-CT and lymph node biopsy showed no evidence of extracutaneous disease. Based on these results, we diagnosed primary cutaneous ALCL associated with atopic dermatitis, and the patient was treated with local radiotherapy. The cutaneous lesions responded to this treatment. However, he developed swelling of neck lymph nodes and was subsequently treated with systemic chemotherapy (CHOP and ESHAP). The prognosis of PCALCL is generally excellent, with an average 10-year survival rate of 90%. Nevertheless, our patient died 21 months after we diagnosed him. We suggest that PCALCL in patients with severe atopic dermatitis has an aggressive clinical course.
