Primary cutaneous peripheral T‐cell lymphoma, not otherwise specified, associated with lymphomatoid papulosis after a 9‐year follow up: A case report

• Published in: Journal of dermatology Vol. 47; no. 6; pp. 641 - 645
• Main Authors: Muraoka, Kyoko, Fujimoto, Masakazu, Iwahashi, Yoshifumi, Matsuzaki, Ibu, Yonei, Nozomi, Murata, Shin‐ichi, Jinnin, Masatoshi
• Format: Journal Article
• Language: English
• Published: England Wiley Subscription Services, Inc 01-06-2020
• Subjects: Administration, Cutaneous; Aged; Aged, 80 and over; Biopsy; Case reports; CD56; Cerebral infarction; Chemoradiotherapy - methods; cytotoxic phenotype; Disease Progression; extranodal natural killer/T‐cell lymphoma; Fatal Outcome; Follow-Up Studies; Glucocorticoids - administration & dosage; Humans; Lymphocytes; Lymphoma; Lymphoma, T-Cell, Cutaneous - diagnosis; Lymphoma, T-Cell, Cutaneous - etiology; Lymphoma, T-Cell, Cutaneous - pathology; Lymphoma, T-Cell, Cutaneous - therapy; Lymphoma, T-Cell, Peripheral - diagnosis; Lymphoma, T-Cell, Peripheral - etiology; Lymphoma, T-Cell, Peripheral - pathology; Lymphoma, T-Cell, Peripheral - therapy; Lymphomatoid papulosis; Lymphomatoid Papulosis - complications; Lymphomatoid Papulosis - diagnosis; Lymphomatoid Papulosis - drug therapy; Lymphomatoid Papulosis - pathology; Male; Mycosis; Mycosis fungoides; peripheral T‐cell lymphoma, not otherwise specified; Skin - pathology; Skin Neoplasms - diagnosis; Skin Neoplasms - etiology; Skin Neoplasms - pathology; Skin Neoplasms - therapy; T-cell lymphoma; Japan; lymphomatoid papulosis; peripheral T-cell lymphoma, not otherwise specified; cytotoxic phenotype; CD56; extranodal natural killer/T-cell lymphoma
• ISSN: 0385-2407; 1346-8138
• DOI: 10.1111/1346-8138.15351

Lymphomatoid papulosis (LyP) is a self‐limiting cutaneous T‐cell lymphoproliferative disorder that may progress into malignant lymphoma. Most of the previously reported associated lymphomas are primary cutaneous anaplastic large‐cell lymphoma and mycosis fungoides with a low mortality rate. We report a case of primary cutaneous peripheral T‐cell lymphoma, not otherwise specified (pcPTCL‐NOS), associated with LyP after long‐term follow up. The patient was a 79‐year old Japanese man followed up for 9 years. He suddenly developed a 3‐cm ulcerated lesion on his forehead, which was diagnosed as an exacerbation of LyP. The lesion regressed after conservative treatment, but the patient soon developed multifocal pcPTCL‐NOS. Thereafter, the patient developed pneumonia and cerebral infarction and died within a few months of the onset of malignant lymphoma. Aggressive cutaneous lymphoma may develop in LyP patients. The present case re‐emphasizes the need for careful follow up of patients with persistent LyP.