A Rare Case of Cutaneous Lymphomatoid Granulomatosis: A Case Report and Literature Review

Lymphomatoid granulomatosis is a rare lymphoproliferative disease caused by dysfunction of host surveillance of Epstein-Barr virus (EBV)-infected B cells, which predominantly affects male adults, with a median onset at 46 years. Diagnosis can be challenging due to its nonspecific symptoms and the need for careful histopathological evaluation, particularly in atypical presentations. Here, we present a case of a 42-year-old Black man from Senegal with no past medical history who presented with a chronic, non-healing ulcer on his right upper extremity that had worsened over six months. Family history revealed a sister with kidney disease who required early dialysis. Initial vital signs showed a blood pressure of 178/123 mmHg and a heart rate of 127 beats/min, with normal temperature and oxygen saturation. A 4 × 5 cm crusted, non-tender stage 3 ulcer with hyperpigmented edges was found on his right arm. Laboratory tests showed pancytopenia, stage 5 chronic kidney disease with nephrotic-range proteinuria, and normocytic anemia. Blood cultures, HIV, and leishmaniasis tests were negative, and chest radiograph findings were normal. Initial evaluation also revealed chronic hepatitis B infection. CT raised concern for osteomyelitis, but MRI was negative. Skin biopsy showed nonspecific inflammatory changes, and cultures revealed methicillin-sensitive Staphylococcus aureus. He received antibiotics with minimal improvement. Five weeks later, he was readmitted with worsening constitutional symptoms, uremia requiring dialysis, and progression of the skin lesion. A repeat skin biopsy revealed an EBV-positive lymphoproliferative disorder consistent with grade 3 lymphomatoid granulomatosis, with predominance of CD4+ T cells. Molecular studies revealed an ATM gene mutation (p.Asn2282Ser) and acquired trisomy X with a normal Y chromosome. The ulcer improved without any further intervention. This case underscores the need for heightened clinical suspicion, repeat tissue sampling, and early dermatologic evaluation, which are critical for diagnosis.