A 35-year-old male detected seropositive for HIV-1 diagnosed recently not on antiretroviral therapy (ART) presented with painful swelling over the genital, inguinal and periumbilical regions with distension of the abdomen since 1 month. There was a sudden onset of genital swelling followed by redness and severe throbbing pain. He had high-grade intermittent fever with weakness, loss of weight and appetite. On examination, he had pallor and bilateral inguinal lymphadenopathy. Cutaneous examination showed erythematous annular tender indurated plaque with well-defined irregular margins of size 7 cmx8 cm around the umbilicus. A diffuse erythematous, indurated, tender swelling of size 6 cmx10 cm was present over the penis and scrotum with sprouting erosive growth over the scrotum [Figure 1]. A differential diagnosis of cellulitis, cutaneous tuberculosis, lymphoma, Kaposi's sarcoma and histoplasmosis was considered.
On investigation, the patient had hemoglobin of 11.5 g%, total leukocyte count of 2,500/mm3, absolute lymphocyte count of 475 cell/mm3, adequate platelets count and erythocyte sedimentation rate (ESR) of 37 mm at the end of 1 h. Serum electrolytes, urine, stool, liver and renal function tests were normal. Venereal Disease Research Laboratory Research (VDRL), Hepatitis B surface antigen (HbsAg), Mantoux test, pus for acid fast bacillus (AFB) and sputum for AFB were negative. X-ray chest showed right-sided pleural effusion. Sonography of the abdomen and pelvis showed thickened anterior abdominal wall, retroperitoneal fibrosis, liver parenchyma disease and bilateral vaginal hydrocele. Ultrasonography chest showed right-sided pleural effusion with moderate pericardial effusion on echocardiography. Computerized tomography (CT) of the abdomen showed extensive abdominal subcutaneous fat with hypodensity in internal oblique to the left pararenal space. The CD4 count was 135/mm3. Skin biopsy showed dense infiltrate seen in the dermis with larger cells with formation of slits. Higher magnification showed characteristic splindeloid cells with hyperchromatic nuclei and scant cytoplasm. Tumor cells expressed epithelial membrance antigen (EMA), with possible kappa light-chain restriction. Thus, the final diagnosis of NHL, plasmablastic variety was made.
The patient was started on low-dose Cyclophosphamide, hydroxydoxorubicin (Oncovin), vincristine, prednisolone (CHOP) therapy. There was a significant reduction in eryrthema, induration and size of the lesion after two cycles. The patient took discharge against medical advice and succumbed after 1 month at home.