Cytophagic histiocytic panniculitis and subcutaneous panniculitis-like T-cell lymphoma: report of 7 cases.

Abstract

BACKGROUND:

Cytophagic histiocytic panniculitis (CHP) is a rare subtype of panniculitis that usually follows a fatal course, with a terminal hemophagocytic syndrome. Recent reports on a subset of peripheral T-cell lymphoma named subcutaneous panniculitis-like T-cell lymphoma (SPTL) raised the question about the relationship between these entities.

OBSERVATIONS:

We describe 7 patients in the study: 1 with fatal CHP, 4 with SPTL, and 2 with long-term CHP. The 5 patients with fatal CHP and SPTL died of complications of hemophagocytic syndrome, with a disease duration ranging from 8 to 74 months. The other 2 patients were still alive 6 and 41 years after disease onset. Immunohistochemical results proved that 2 of the SPTL cases were type alpha/beta and expressed the cytotoxic/suppressor antigen CD8, while the other 2 were type gamma/delta and were positive for the natural killer-associated antigen CD56. In these 4 cases, molecular biology studies by polymerase chain reaction detected T-cell receptor gamma gene rearrangement, indicating a clonal process. In contrast, in the 2 patients who had long-term CHP, the polymerase chain reaction results failed to disclose clonality. In the subject with fatal CHP, genotypic analysis was not performed.

CONCLUSION:

Our observations suggest that CHP and SPTL may span a clinicopathologic spectrum in which there is a natural disease progression from CHP to SPTL.

• Authors: A V Marzano, E Berti, M Paulli, R Caputo
• Journal: Archives of dermatology (Arch Dermatol) Vol. 136 Issue 7 Pg. 889-96 (Jul 2000) ISSN: 0003-987X [Print] United States
• PMID: 10890991 (Publication Type: Journal Article)

Chemical References

• RNA, Viral

Topics

• Adolescent
• Adult
• Child
• Female
• Gene Rearrangement, T-Lymphocyte
• Herpesvirus 4, Human (isolation & purification)
• Histiocytosis (complications, pathology, virology)
• Humans
• In Situ Hybridization
• Lymphoma, T-Cell, Cutaneous (genetics, pathology, virology)
• Male
• Middle Aged
• Panniculitis (complications, genetics, pathology, virology)
• Polymerase Chain Reaction
• RNA, Viral (analysis)
• Skin (pathology)
• Skin Neoplasms (genetics, pathology, virology)