Case Presentation: A 40-year-old white male who had noticed a nodule over the extensor surface of his right forearm, which then grew to a size of 3 cm tumor and ulcerated over six months. At one point, he was assumed to have an infection and treated with antibiotics with no improvement. Around the same time, he started noticing a rapidly growing nodule on the left ala of the nose. The review of systems was positive for generalized pruritus. He denied weight loss, night sweats, or fevers. The physical exam is positive for ulcerated lesion about 3 cm in diameter over the right forearm, 4 mm papule over the left nasal ala and 1cm palpable left inguinal node and axillary lymph node were also present. A biopsy of the elbow lesion showed sheets of large CD30-positive with abundant eosinophilic cytoplasm and round-shaped nuclei with prominent nucleoli which confirmed the diagnosis of ALCL. Positron emission tomography (PET/CT) showed shotty left inguinal and axillary lymphadenopathy with mildly fluorodeoxyglucose (FDG) avidity. He was found positive for Human Immunodeficiency Virus (HIV)-1. His CD4 count was 160/mm3, and CD4/CD8 ratio was 0.14. His Hepatitis B and C serologies were negative, and human T-lymphotropic virus (HTLV) 1/2 was also negative. The patient has started on HAART, his CD4 count increased to 460/mm3, viral load nearly was undetectable shortly after treatment, and interestingly his elbow lesion has also decreased, and his nasal lesion wholly resolved.

Discussion: Anaplastic large T cell lymphoma (ALCL) has two morphologically identical but clinically different subtypes referred to as primary systemic ALCL and primary cutaneous ALCL (pc-ALCL); although morphologically similar, clinical features and treatments differ. Clinical presentation of pc-ALCL can be variable. HIV related pc-ALCL is extremely rare and occurs earlier than the classic pc-ALCL (mean age: within the fourth decade). A trend, likely due to HIV infection demographics (1,2). A high index of suspicion is necessary for patients presenting with chronic plaques and nodules unresponsive to topical or oral medications. Clinical or radiographic suspicion of the disease should be confirmed by biopsy. Though, the involvement of regional lymph nodes does not necessarily indicate the presence of systemic disease. Despite the fact that a wide range of therapeutic options for the management of pc-ALCL described in the literature, the efficacy of those available therapies is not well established in HIV/AIDS patients. Pc-ALCL have a tendency for spontaneous regression. So, single, small lesions can be observed if they are not bothersome to the patient. Other options would be treating with surgery or radiation. In contrast, multiple lesions should be treated with more aggressive measures, including repeated surgical excisions and/or radiation or systemic chemotherapy. Interestingly, HIV patients with pc- ALCL usually have a good response after anti-retroviral therapy as observed in our patient.

Conclusions: Clinical presentation of pc-ALCL can be variable. Although pc-ALCL is extremely rare in HIV patients, a high index of suspicion is necessary for patients presenting with chronic skin lesions as early diagnosis is crucial for HIV patients, given the fact that pc-ALCL usually have a good response after anti-retroviral therapy.