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A Clinical, Immunohistological, and Molecular Analysis

Philippe Martel, MD; Liliane Laroche, MD, PhD; Philippe Courville, MD; Claire Larroche, MD; Janine Wechsler, MD; Bernard Lenormand, MD; Marie-Hélne Delfau, MD, PhD; Christine Bodemer, MD; Martine Bagot, MD, PhD; Pascal Joly, MD, PhD; for the French Study Group on Cutaneous Lymphomas

Arch Dermatol. 2000;136:881-886.

Objective  To determine whether cutaneous involvement in patients with angioimmunoblastic lymphadenopathy with dysproteinemia (AILD) is related to a clonal T-cell proliferation.

Design  Retrospective study.

Setting  University hospitals.

Patients  Ten patients with AILD and cutaneous involvement.

Main Outcome Measure  The T-cell receptor- (TCRG) gene rearrangement was studied with the use of polymerase chain reaction and denaturing gradient gel electrophoresis in blood, nodal, and skin samples. Skin and nodal samples were investigated also for the presence of Epstein-Barr virus (EBV) RNA 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 urticarial lesions. A clonal TCRG gene rearrangement was detected in 7 skin samples, corresponding to a maculopapular eruption with a histological pattern of nonspecific mild lymphoid dermal infiltrate in 6 patients, and to erythematous 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. Five patients died by the end of the study, with a mean survival of 33.2 months. 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 of 8 lymph nodes tested.

Conclusions  Cutaneous involvement is often related to a clonal T-cell proliferation in AILD, even when clinical and histological features are nonspecific. Cutaneous infiltrate seems to be clonally related to the nodal T-cell proliferation. The role of EBV infection in skin lesions was not evidenced.

From the Clinique Dermatologique, Institut National de la Santé et de la Récherche Medical U519 (Drs Martel and Joly), Laboratoire d'anatomie et de cytologie pathologiques (Dr Courville), and Laboratoire d'Hématologie (Dr Lenormand), Hôpital Charles Nicolle, Rouen, France; Service de Dermatologie et Unité d'Hématologie, Hôpital Avicenne, Bobigny, France (Drs Laroche and Larroche); Département de Pathologie (Dr Wechsler), Service d'Immunologie Biologique (Dr Delfau), and Service de Dermatologie (Dr Bagot), Hôpital Henri Mondor, Creteil, France; and Service de Dermatologie, Groupe Hospitalier Necker, Paris, France (Dr Bodemer).

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