Angioimmunoblastic lymphadenopathy. A generalized lymphoproliferative disorder with cutaneous manifestations

Abstract

Angioimmunoblastic lymphadenopathy (AIBL) is a recently described, systemic disorder characterized by an excessive proliferation of transformed lymphocytes, possibly the result of chronic antigenic stimulation, frequently by drugs. Cutaneous involvement, usually in the form of a pruritic, generalized, maculopapular rash, occurs in about 40% of cases. We saw a patient with AIBL who had an unusual, primarily plaque- and nodular-type of skin involvement and who had been receiving drugs not previously reported as being associated with this disorder. Because cutaneous involvement is a frequent, presenting complaint, dermatologists should be able to recognize this unusual, clinicopathologic entity.

Extranodal Marginal Zone Lymphoma–like Presentations of Angioimmunoblastic T-Cell Lymphoma

“…Interestingly, overexpression of c-maf has been shown in up to 75% of T-cell lymphomas 35,36 , and is linked to increased expression of VEGF, or vascular endothelial growth factor, and tumor replication 37 . Increased vascularity is a well-known feature of AITL in lymph nodes, and neoangiogenesis and increased vascularity has been documented in up to 40% of AITL with cutaneous dissemination 2,10,11,14,15,38 . Notably, FDC and endothelial cells in AITL show increased expression of FasL, while the tumor cells express Fas (CD95), highlighting the important role played by the interaction of Fas and FasL in the disease pathogenesis, and the close relationship between the environment of tumor cells and vessels 10 .…”

“…Skin rashes are associated with AITL in 50–80% of patients 2,10–17 . Typically, the rash is morbilliform, and less commonly purpuric, urticarial, nodular or petechial.…”

Skin lesions and neutrophilic leukemoid reaction in a patient with angioimmunoblastic T‐cell lymphoma: a case report and review of the literature

“…In the literature, uncommon skin lesions of AITL are mostly described in case reports or review articles (Table 2). It is not rare that the patient has a generalized pleomorphic rash composed of several types of rashes, such as macula, papules, maculopapules, [30] 77/M Macules; petechiae; purpura 1 year before Alive (4 month) Seehafer [10] 74/M Petechiae Concurrent Died (3 month) Seehafer [10] 61/M Erythroderma and purpura Concurrent Alive (48 month) Seehafer [10] 57/M Petechiae 10 month before Alive (48 month) Schmuth [31] 73/F Macules; petechiae; purpura 4 week before Alive (4 month) Martel [15] Necrotic purpura, maculopapules and urticaria Died (26 day) Martel [15] Pruritic papulovesicular (prurigo-like) lesion Alive (96 month) Hashefi [32] Maculopapules, petechiae 3 month before Died (23 month) Suarez-Vilela [33] 67/F Sarcoidosis 1 month before Huang [34] 62//M Erythroderma; plaques; nodules 3 year after Died (3 year) Jones [35] 67/M Erythroderma, toxic epidermal necrolysis Concurrent Died (5 month) Tsochatzis [36] 50/M Polyarthritis, subcutaneous nodules Concurrent Died (2 month) Jayaraman [11] 61/M Macules, papules, plaques, and nodules Concurrent Alive (5 year) Ortonne [37] 63/F Nodules, gingival ulceration Ortonne [37] 54/M Maculopapules, hemorragic/necrotic nodules Nassar [5] M nodules, erythroderma, urticaria, petechiae, purpura and so on. Necrotic purpura, polyarthritis, gingival ulceration, erythematous plaques (sometimes annular), toxic epidermal necrolysis, and hemorragic/necrotic nodules are also reported.…”

Multiple cutaneous monoclonal B‐cell proliferations as harbingers of systemic angioimmunoblastic T‐cell lymphoma

“…In their series, 7 of 11 skin biopsies yielded clonal results by PCR for TCR γ. Many other clinical and pathological presentations of cutaneous eruptions in AITL are also described in the literature, predominantly as single case reports 5,7–17 …”