Abstract

Lymphomatoid granulomatosis (LG) is an angiocentric and angioinvasive Epstein-Barr virus associated mature B-cell lymphoproliferative disorder. Lung is the most common and initial site of presentation followed by skin. We report a case of LG in a young female with cutaneous lesions preceding the onset of pulmonary symptoms. A 21-year-old female presented with skin ulcerations on bilateral legs, abdomen, and back. She was seen by an outside dermatologist and workup including skin biopsy did not yield a specific etiology. She subsequently developed sepsis from the leg ulcer getting secondarily infected and was treated with antibiotics. Two months later, she developed a productive cough with bloody sputum and was found to have pulmonary nodules at an outside hospital. Given worsening symptoms and concern for infection or autoimmune disease, repeat punch biopsies obtained from several skin lesions showed a superficial to deep perivascular, interstitial and periadnexal infiltrate of atypical large cells with vesicular nuclei, variably prominent nucleoli and identifiable mitoses with areas of necrosis. Immunohistochemical stains showed that the lesional cells are positive for CD20, BCL-2, MUM-1 and diffusely positive for EBER (by ISH). No microorganisms were identified on special stains. Immunostains also highlighted angioinvasion and angiodestruction by the atypical B cells. Given the clinical picture of multiple ulcerative nodules in the skin and multiple lung nodules along with similar histomorphological findings in a concurrent tracheal biopsy, a diagnosis of Grade 3 LG was made and chemotherapy was initiated. LG commonly presents in the 6th decade with a male predominance (2:1). Skin lesions often develop after the onset of pulmonary disease. Our case report highlights the importance of keeping a high index of suspicion for LG in adults presenting with lung and cutaneous lesions even in a younger age group and irrespective of initial site of lesion.

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