A Case of Primary Cutaneous CD4 Positive Small/medium T Cell Lymphoma

Primary cutaneous CD4‐positive small/medium‐sized pleomorphic T‐cell lymphoproliferative disorder: Report of a case and review of the literature

“…PCSM‐TCLPD accounts for approximately 2% to 3% of all cutaneous lymphomas. This designation applies to cases with a predominance of small/medium‐sized pleomorphic CD4+ T‐cell phenotype with a distinct clinical course from mycosis fungoides . The median age of onset is 60 years .…”

“…Due to multiple reports of progressive and systemic PCSM‐TCLPD, authors have nominated several clinical and histopathological features as markers of an aggressive phenotype including rapidly growing, bulky tumors, higher proliferative index, aberrant loss of CD2, decreased CD7 in tumor lymphocytes, decreased CD8+ tumor infiltrating lymphocytes, and paucity of eosinophils and plasma cells . Garcia‐Herrera et al reported many of these features in 5 cases with an “aggressive clinical outcome”; all patients died within 18–36 months of initial diagnosis.…”

“…Diagnosis further necessitates the absence of clinical features consistent with mycosis fungoides . Cases usually present with a solitary plaque or tumor, most commonly on the head or neck, although, cases involving other parts of the body and multifocal disease have been reported …”

“…PCSM‐TCLPD typically follows an indolent clinical course with a 5‐year survival rate of approximately 60% to 80% . Progression to systemic disease is exceedingly rare and patients often experience high cure rates from conservative therapies including excision or radiation therapy . Toberer et al reported a case that went into remission following treatment with a 21‐day course of oral doxycycline .…”

“…Cases usually present with a solitary plaque or tumor, most commonly on the head or neck, although, cases involving other parts of the body and multifocal disease have been reported. 1,[3][4][5][6][7] PCSM-TCLPD typically follows an indolent clinical course with a 5-year survival rate of approximately 60% to 80%. 1,8 Progression to systemic disease is exceedingly rare and patients often experience high cure rates from conservative therapies including excision or radiation therapy.…”