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.

OBJECTIVES: 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.

CONCLUSION: 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.