Cutaneous manifestations of angioimmunoblastic lymphadenopathy

Abstract

Six of 22 patients with angioimmunoblastic lymphadenopathy seen at the Mayo Clinic from 1962 to 1978 had associated cutaneous lesions exhibiting characteristic histologic features at biopsy. Clinically, three patients had generalized maculopapular eruptions, two had generalized petechiae, and one had generalized erythroderma and petechiae located on the extremities. In each patient, the eruption occurred concurrent with or prior to the onset of generalized lymphadenopathy. Pathologically, all skin biopsy specimens showed lymphohistiocytic vasculitis, vascular proliferation, extravasated RBCs, and occasional scattered mature plasma cells. These histologic findings were similar to but less intensive than those noted on biopsy specimens of involved lymph nodes. Although suggestive of angioimmunoblastic lymphadenopathy, the histologic findings of skin lesions do not appear to be diagnostic, thus stressing the difficulty of defining the syndrome in the absence of lymph node biopsy.

Angioimmunoblastic T‐cell lymphoma

“…The most frequently involved extranodal sites include the bone marrow, spleen, skin and lungs. The histological appearances in these sites are usually non‐specific but mimic some of the features described in the lymph node, including increased vascularity and a polymorphic inflammatory infiltrate with or without clear cells (Seehafer et al , 1980; Ghani & Krause, 1985; Brown et al, 2001). Cytological features of malignancy can rarely be identified, and tumour involvement can only be shown by immunohistochemistry and molecular clonality analysis (Martel et al, 2000; Murakami et al, 2001).…”

“…A number of autoimmune phenomena have been reported in association with AITL. These include autoimmune haemolytic anaemia (Brearley et al , 1979), vasculitis (Seehafer et al , 1980; Hamidou et al, 2001; Sugaya et al, 2001), polyarthritis, rheumatoid arthritis (Pieters et al , 1982; Pautier et al , 1999) and autoimmune thyroid disease (Ambepitiya, 1989; Pautier et al , 1999).…”

Angioimmunoblastic T Cell Lymphomas: Frequent Cutaneous Skin Lesions and Absence of Human Herpes Viruses

“…Dermatologists should be aware of AITL and include skin lesions of AITL among the differential diagnoses of fever of unknown origin with generalized skin rash [13][14][15] . As the skin lesions closely mimic a benign condition and the skin biopsies of skin lesions of AITL are not malignant, clonality testing with clinical suspicion is crucial for early and accurate diagnosis and can prevent mistreatment or confusion of treatment policy.…”

Cutaneous Involvement in Patients With Angioimmunoblastic Lymphadenopathy With Dysproteinemia<subtitle>A Clinical, Immunohistological, and Molecular Analysis</subtitle>

“…[21][22][23] Indeed, only 2 of 10 patients presented with infiltrated plaques that were clinically suggestive of a cutaneous T-cell lymphoma. On the contrary, 8 patients presented with nonspecific cutaneous features such as maculopapular morbilliform eruptions and urticarial or purpuric lesions.…”