LYMPHOMATOID GRANULOMATOSIS

By Gary M. White, MD

Lymphomatoid granulomatosis 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.

Clinical

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.

Treatment

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.

Additional Pictures

Lymphomatoid granulomatosis

References

JAAD Case Reports July 2015 Volume 1, Issue 4, Pages 234–237

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