Cutaneous involvement in patients with angioimmunoblastic lymphadenopathy with dysproteinemia - A clinical, immunohistological, and molecular analysis

Objective: To determine whether cutaneous involvement in patients with angi oimmunoblastic lymphadenopathy with dysproteinemia (AILD) is related to a c lonal T-cell proliferation. Design: Retrospective study. Setting: University hospitals. Patients: Ten patients with AILD and cutaneous involvement. Main Outcome Measure: The T-cell receptor-gamma (TCRG) gene rearrangement w as studied with the use of polymerase chain reaction and denaturing gradien t gel electrophoresis in blood, nodal, and skin samples. Skin and nodal sam ples were investigated also for the presence of Epstein-Barr virus (EBV) RN A by in situ hybridization. Results: A transient morbilliform eruption of the trunk was seen most often . Other cutaneous features were infiltrated plaques and purpuric or urticar ial lesions. A clonal TCRG gene rearrangement was detected in 7 skin sample s, corresponding to a maculopapular eruption with a histological pattern of nonspecific mild lymphoid dermal infiltrate in 6 patients, and to erythema tous plaques with histological findings of typical cutaneous lymphoma in 1 patient. In the 5 patients in whom a TCRG gene rearrangement was evidenced in skin and lymph node samples, identical clones were detected in both. Fiv e patients died by the end of the study, with a mean survival of 33.2 month s. Four of these 5 patients had a clonal infiltrate in skin and lymph nodes . The EBV RNA was detected in only 1 of 10 skin biopsy specimens and in 5 o f 8 lymph nodes tested. Conclusions: Cutaneous involvement is often related to a clonal T-cell prol iferation in AILD, ec en when clinical and histological features are nonspe cific. Cutaneous infiltrate seems to be clonally related to the nodal T-cel l proliferation. The role of EBV infection in skin lesions was not evidence d.