Display Settings:

Format

Send to:

Choose Destination
See comment in PubMed Commons below
Arch Dermatol. 2000 Jul;136(7):889-96.

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

Author information

  • 1Istituto di Scienze Dermatologiche, University of Milan and IRCCS Ospedale Maggiore, Via Pace, 9, 20122 Milan, Italy.

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.

Comment in

PMID:
10890991
[PubMed - indexed for MEDLINE]
PubMed Commons home

PubMed Commons

0 comments
How to join PubMed Commons

    Supplemental Content

    Full text links

    Icon for Silverchair Information Systems
    Loading ...
    Write to the Help Desk