Panniculitis histiocytic, cytophagicM35.6
HistoryThis section has been translated automatically.
Winkelmann and Bowie, 1980
DefinitionThis section has been translated automatically.
Very rare, recurrent, lobular panniculitis with often fulminant clinical course, with fever, severe feeling of illness, hepatosplenomegaly, mucous membrane ulcers, histiocytic proliferation or hemophagocytosis.
Discussed are variants of panniculitis nodularis nonsuppurativa febrilis et recidivans, Langerhans cell histiocytosis or T-cell lymphoma.
EtiopathogenesisThis section has been translated automatically.
Not clear. Discussed are α-1-antitrypsin deficiency, acquired angioedema or paraproteinemia. In recent years, increasing numbers of observations on the association with T-cell lymphomas, especially panniculitis-like T-cell lymphoma.
ManifestationThis section has been translated automatically.
Occurs in early to middle adulthood, less frequently in adolescents or children. No gender preference.
LocalizationThis section has been translated automatically.
Runmp; Extremities
Clinical featuresThis section has been translated automatically.
Panniculitis in the trunk and extremities, anaemia, fever, hepatosplenomegaly, thrombocytopenia; serositis, aphthous lesions, peripheral swelling of lymph nodes, mucous membrane ulcerations, ecchymoses, coagulation defects.
LaboratoryThis section has been translated automatically.
Pancytopenia as a severe complication.
HistologyThis section has been translated automatically.
Picture of mixed septal/lobular panniculitis. The picture is characterized by lymphocytes, macrophages, neutrophilic granulocytes and plasma cells. Typical are macrophages that have phagocytised lymphocytes, erythrocytes and thrombocytes (bean bag cells). Atypical cells can be detected in cases associated with T-cell lymphomas.
TherapyThis section has been translated automatically.
Cooperation with internists. Polychemotherapy is required, but no standard therapy is known. Different cytostatic drugs have been tried so far, e.g. CHOP-scheme, Ciclosporin A.
Progression/forecastThis section has been translated automatically.
Unfavorable, fatal generalized hemorrhage, liver failure.
LiteratureThis section has been translated automatically.
- Bassez G et al (2003) Inflammatory myopathy with abundant macrophages (IMAM): a condition sharing similarities with cytophagic histiocytic panniculitis and distinct from macrophagic myofasciitis. J Neuropath Exp Neurol 62: 464-474
- Craig AJ et al (1998) Cytophagic histiocytic panniculitis--a syndrome associated with benign and malignant panniculitis: case comparison and review of the literature. J Am Acad Dermatol 39: 721-736
- Csato M et al (1981) Cytophagic panniculitis. Dermatologist 32: 370-371
- Kuno Y et al (1999) Livedo vasculitis associated with regional cytophagic histiocytoses. J Dermatol 26: 817-820
- Marzano AV et al (2000) Cytophagic histiocytic panniculitis and subcutaneous panniculitis-like T-cell lymphoma: report of 7 cases. Arch Dermatol 136: 889-896
- Phelps RG et al (2001) Update on panniculitis. Mt Sinai J Med 68: 262-267
- Winkelmann RK, Bowie EJ (1980) Hemorrhagic diathesis associated with benign histiocytic, cytophagic panniculitis and systemic histiocytosis. Arch Internal Med 140: 1460
- Winkelmann RK (1981) Panniculitis with cell phagocytosis. dermatologist 31: 588-594
- Zollner TM et al (2001) Monitoring of phagocytic activity in histiocytic cytophagic panniculitis. J Am Acad Dermatol 44: 120-123