By Gary M. White, MD
Courtesy of Michael O. Murphy, MD
Lymphomatoid granulomatosis (LG) is a rare an angiocentric and angioinvasive B cell, Epstein-Barr virus (EBV)-driven, lymphoproliferative disorder.
- LG is a lymphoproliferative disorder of EBV positive B cells associated with a large population of reactive, infiltrating T cells.
- If untreated, LG can lead to progressive pulmonary failure, central nervous system disease, or progression to overt EBV-positive lymphoma.
40% of patients have cutaneous lesions--the most common being a cutaneous nodule. Other skin changes include eroded and crusted lesions, facial edema, digital ulcers and necrosis, papules and a folliculitis-like eruption. The skin is the second most commonly involved organ with the lung being the first. Cough, sputum production, shortness of breath and bilateral basilar nodules on CXR are characteristic. Fever, peripheral neuropathy, CNS lesion and arthralgias also may occur.
Some cases remit, but others are aggressive with fatal pulmonary involvement. Chemotherapy, radiation therapy or both are used. Systemic steroids, intereron-alfa-2b and rituximab have been employed.
JAAD Case Reports July 2015 Volume 1, Issue 4, Pages 234–237
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