Cutaneous involvement in patients with angioimmunoblastic lymphadenopathy with dysproteinemia: a clinical, immunohistological, and molecular analysis.
OBJECTIVE
To determine whether cutaneous involvement in patients with angioimmunoblastic lymphadenopathy with dysproteinemia (AILD) is related to a clonal T-cell proliferation.


METHODS
Retrospective study.


METHODS
University hospitals.


METHODS
Ten patients with AILD and cutaneous involvement.


METHODS
The T-cell receptor-gamma (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.
Martel
P
P
Clinique Dermatologique, Hôpital Charles Nicolle, 1, rue de Germont, 76031 Rouen Cedex, France.
Laroche
L
L
Courville
P
P
Larroche
C
C
Wechsler
J
J
Lenormand
B
B
Delfau
M H
MH
Bodemer
C
C
Bagot
M
M
Joly
P
P
D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D001796:Blood Protein Disorders / Q000150:complications; D005260:Female; D015329:Gene Rearrangement, T-Lymphocyte; D004854:Herpesvirus 4, Human / Q000302:isolation & purification; D006801:Humans; D007119:Immunoblastic Lymphadenopathy / Q000150:complications* / Q000235:genetics / Q000276:immunology / Q000821:virology; D016130:Immunophenotyping; D017403:In Situ Hybridization; D008198:Lymph Nodes / Q000821:virology; D008297:Male; D008875:Middle Aged; D016133:Polymerase Chain Reaction; D012367:RNA, Viral / Q000032:analysis; D012189:Retrospective Studies; D012867:Skin / Q000276:immunology / Q000821:virology; D012871:Skin Diseases / Q000150:complications* / Q000473:pathology / Q000821:virology
D012367:RNA, Viral