Cutaneous manifestations of lymphomatoid granulomatosis. Report of 44 cases and a review of the literature

Abstract

A review of records of 44 patients who had lymphomatoid granulomatosis (LYG) with skin lesions showed that cutaneous manifestations are protean and generally transient. Subcutaneous or dermal nodules were present in more than 60% of cases. A maculopapular rash or macular erythema was less common, and ulceration was rare. Skin lesions were widely distributed. Frequently, in patients who had early cutaneous involvement with LYG, associated involvement of the nervous system and joints occurred. Findings from laboratory studies were generally inconclusive. Lymphomatoid granulomatosis progressed to lymphoma in 18% of the cases, and death from respiratory failure was common. Theories on the possible cause and the pathogenesis of LYG are presented.

Differences in Epstein-Barr Virus Expression Between Primary and Secondary Cutaneous Angiocentric Lymphomas

“…[9][10][11][12][13] Skin can be the only site or one of the prominent sites of initial involvement. 7,8 In the present study, the presence of EBV was investigated in a series of CALs, including 12 primary and 2 secondary CALs. In both secondary CALs, EBV was strongly detected by in situ hybridization and immunohistochemistry.…”

Adhesion Molecules in Lymphomatoid Granulomatosis

“…20] including papules, nodules, ulcers, vescicles and ringshaped plaques with centrifugal healing. Ichthyosis-like lesions, alopecia or necrobiosis-lipoidica-like lesions may also be found [18,[21][22][23][24][25].…”

Granulomatous Lymphoproliferative Disorders

“…25 Clinically, patients with pulmonary involvement present with cough, shortness of breath, and chest pain. 25,26 Systemic symptoms such as fever, malaise, weight loss, and myalgias commonly occur. Other common symptoms include peripheral neuropathy and arthralgias.…”

“…Other common symptoms include peripheral neuropathy and arthralgias. 17,25,26 Although pulmonary involvement occurs in more than 90% of patients with LYG, it is not necessary for the diagnosis of LYG.…”

“…17,24 James and colleagues, 26 reporting on 44 patients with LYG, noted that skin manifestations can be painful and include subcutaneous nodules, dermal nodules, maculopapular eruptions, macular erythema, and ulcerations. These lesions are caused by arteritis of the small vessels of dermal and subcutaneous tissue.…”