Familial lymphocytic infiltration of the skin: histochemical and molecular analysis in three brothers.
BACKGROUND
Lymphocytic infiltration of the skin (Jessner and Kanof) is a T cell pseudolymphoma characterized by the occurrence of recurrent asymptomatic papules and plaques and by a coat-sleeve-like perivascular lymphoid infiltrate. Rarely, familial cases have been reported.


OBJECTIVE
Our study was performed to address the question of a genetic predisposition in a case of familial lymphocytic infiltration by histochemical and molecular analysis.


RESULTS
We report on 3 brothers with typical clinical and histological features of Jessner's lymphocytic infiltration of the skin. Immunohistochemical analysis revealed a mixed lymphocytic infiltrate with a predominance of CD8+ T cells in all 3 patients. Molecular determination of T cell clonality by PCR-based GeneScan analysis of the T cell receptor (TCR)-gamma-chain showed oligoclonal, pseudomonoclonal or polyclonal TCR-gamma rearrangement patterns in lesional skin and in peripheral blood of all 3 brothers, while no common TCR idiotype was detected.


CONCLUSIONS
Inherited deviations in TCR usage seem unlikely as a special cause of familial Jessner's lymphocytic infiltration of the skin; lack of clonality furthermore supports the notion that this variant of the disease is as true a pseudo-T-cell lymphoma as are the spontaneous cases.
Dippel
E
E
Department of Dermatology, University Medical Center Benjamin Franklin, Free University of Berlin, Germany. edgar.dippel@haut.ma.uni-heidelberg.de
Poenitz
N
N
Klemke
C D
CD
Orfanos
C E
CE
Goerdt
S
S
D000328:Adult; D001707:Biopsy, Needle; D005148:Facial Dermatoses / Q000235:genetics / Q000473:pathology; D020022:Genetic Predisposition to Disease; D006801:Humans; D007150:Immunohistochemistry; D008214:Lymphocytes / Q000473:pathology*; D016410:Lymphoma, T-Cell, Cutaneous / Q000235:genetics* / Q000473:pathology*; D008297:Male; D010375:Pedigree; D016133:Polymerase Chain Reaction; D011379:Prognosis