Clinical Features

Anaplastic large cell lymphoma represents the second most common form of CL, with a prevalence of 1-2 per million, occurring mainly in people in their sixth decade, with a male preponderance (male:female ratio = 2-3:1) (3,4). Children can also have ALCL. The vast majority of primary cutaneous ALCL in HIV-infected individuals are also CD30+ (5). The clinicopathologic features are otherwise identical to those of ALCL occurring in immunocompetent patients.

ALCL usually manifests as an asymptomatic, solitary, firm, and often ulcerated nodule (Fig. 1) (3,6). The extremities and head are sites of predilection (3,7,8). Approximately 20% of the patients have multifocal disease, i.e., two or more lesions at multiple anatomic sites (4). Most patients experience a history of rapidly growing tumors over a time span of 4-6 weeks without preceding longstanding patches or plaques. If there is no therapeutic intervention, spontaneous regression has been reported to occur in 10-42% of the tumor lesions (3,4), which led to the original description of regressing atypical histiocytosis (RAH) (9).

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