Cameo

LYMPHOMATOID GRANULOMATOSIS

THtODORE ROStN, M.D. AND MARVIN E. CHERNOSKY. M.D.

Lymphomatoid granulomatosis is a multisystem disease of obscure etiology. The disorder has a predilection for middle-aged men, the male to female ratio being 3:2. A necrotizing, granulomatous angiitis affects the lungs (most cases), kidneys (45% of cases) and central and peripheral nervous systems (20% of cases).^ Cutaneous lesions occur in 45% of patients and may be the initial manifestation.^ Skin lesions may include papules, nodules, tumors and plaques with or without ulceration. Biopsy specimen reveals an angiocentric lymphohistiocytic infiltrate containing variable numbers of atypical mononuclear cells. Although improvement may follow treatment with steroids and/or immunosuppressive agents, the prognosis is generally poor and progression to overt lymphoma occurred in 13% of the patients in the original series.' The position of lymphomatoid granulomatosis in relation to Wegener's granulomatosis, allergic granulomatosisand lymphoreticular malignancies such as histocytic lymphoma and mycosis fungoides has yet to be well defined. Address for reprints: Theodore Rosen, M.D., Room 1194, Medical School Main Building, 6431 Fannin. Houston, TX 77030.

From thf Department at Dermatology, University of Texas Medical School at Houston, Texas

Fig. 1. Top, c onfluent ulcerated induration of' chest. Fig. 2. Bottom, firm facial papules and nodules.

0011-9059/79/0700/0497/$00.60 © Interndtiunal Society of Tropical Dermatology, Inc.

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INTERNATIONAL lOURNAL OF DERMATOLOGY

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r i g . i . Angiocentric lymphohistioc ytit (H.&E., original magnification x 400).

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luly-August

1979

Vol. 18

ihematous plaques and nodules on the face, neck and upper arms (Fig. 2), and variable sized purpuric macules on the forearms and forelegs. Neurologic evaluation disclosed uniformly decreased deep tendon reflexes and substantial symmetric distal motor weakness. While hospilalized, the palient developed extensive bilaleral pulmonary infiltrates coincident with the onset of dyspnea. Multiple sputum cultures failed to reveal pathogens. Laboratory studies showed mild anemia (hemoglobin 12 g/dl, hematocrit ^b%}, decreased sera total prolein and albumin, and 2+ proteinurla. Skin biopsy specimens demonstrated a distinctly pefivas

Lymphomatoid granulomatosis.

Cameo LYMPHOMATOID GRANULOMATOSIS THtODORE ROStN, M.D. AND MARVIN E. CHERNOSKY. M.D. Lymphomatoid granulomatosis is a multisystem disease of obscur...
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