Fatal systemic cytophagic histiocytic panniculitis: A histopathologic and immunohistochemical study of multiple organ sites

doi:10.1016/S0190-9622(94)70256-X

Journal of the American Academy of Dermatology

Volume 31, Issue 5, Part 2, November 1994, Pages 901-905

https://doi.org/10.1016/S0190-9622(94)70256-X Get rights and content

The presence of hemocytophagic histiocytosis within subcutaneous tissue has been termed “cytophagic histiocytic panniculitis” (CHP). CHP may occur as a feature of hematopoietic malignancies or infections, particularly viral. In some patients with CHP, an underlying illness cannot be identified. We describe a young man with a rapidly fatal systemic illness characterized by CHP. Lymphoma was not present, and an infectious agent could not be identified. Immunohistochemical stains of tissue obtained at autopsy from multiple organ sites confirmed the presence of histiocytes and T lymphocytes within adipose tissue.

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Cited by (23)

Panniculitis
2009, Weedon's Skin Pathology: Third Edition
Histopathology and genetics of cutaneous T-cell lymphoma
2003, Hematology/Oncology Clinics of North America
Panniculitis. Part II. Mostly lobular panniculitis
2001, Journal of the American Academy of Dermatology
The second part of our review of panniculitis summarizes the clinicopathologic features of the mostly lobular panniculitides. Erythema induratum of Bazin (nodular vasculitis) represents the most common variant of lobular panniculitis with vasculitis, although controversy persists about the nature of the involved vessels. Mostly lobular panniculitides without vasculitis comprise a series of disparate disorders. These include sclerosing panniculitis that results from chronic venous insufficiency of the lower extremities; panniculitis with calcification of the vessel walls such as calciphylaxis and oxalosis; and inflammatory diseases with crystals within the adipocytes such as sclerema neonatorum, subcutaneous fat necrosis of the newborn, and poststeroid panniculitis. Connective tissue diseases, such as systemic lupus erythematosus and dermatomyositis, pancreatic diseases, and α₁-antitrypsin deficiency may also show a mostly lobular panniculitis with characteristic histopathologic features. Lobular panniculitis may also be an expression of infections, trauma, or factitial causes involving the subcutaneous fat. Lipoatrophy refers to a loss of subcutaneous fat due to a previous inflammatory process involving the subcutis, and it may be the late-stage lesion of several types of panniculitis. In contrast, lipodystrophy means an absence of subcutaneous fat with no evidence of inflammation and often the process is associated with endocrinologic, metabolic, or autoimmune diseases. Finally, cytophagic histiocytic panniculitis is the term that has been used to describe two different processes: one is inflammatory, a lobular panniculitis, and the other one is neoplastic, a subcutaneous T-cell lymphoma. The only common feature of these two different processes is the presence of cytophagocytosis in the lesions. (J Am Acad Dermatol 2001;45:325-61.) Learning objective: At the completion of this learning activity, participants should be familiar with the pathogenesis, clinical manifestations, histopathologic findings, and treatment options for the most frequent variants of the lobular panniculitides.
Cytophagic histiocytic panniculitis - A syndrome associated with benign and malignant panniculitis: Case comparison and review of the literature
1998, Journal of the American Academy of Dermatology
Many cases of hemophagocytic syndromes with cytophagic histiocytic panniculitis (CHP) are being classified and considered as a natural disease progression of subcutaneous panniculitic T-cell lymphoma (SPTL). Review of the literature on both CHP and SPTL discloses distinct patterns suggesting these disorders may not be equivalent, even though a terminal hemophagocytic syndrome may be associated with each. Some SPTL appear to have association with Epstein-Barr virus (EBV), show malignant histopathology in the skin, and may disseminate terminally, with a majority of cases showing a rapidly fatal progression. On the other hand, classic cases of CHP without proven lymphoma may not be associated with EBV, appear to be histologically benign, and have an indolent course unless terminal hemophagocytic syndrome develops. We compared and contrasted a case of CHP and a case of SPTL and reviewed the literature. Our observations suggest that the often fatal hemophagocytic syndrome may be associated with both benign and malignant subcutaneous panniculitis. (J Am Acad Dermatol 1998;39:721-36.)
Inflammatory diseases of the subcutaneous fat
2014, Lever's Histopathology of the Skin: Eleventh Edition
Cytophagic histiocytic panniculitis and hemophagocytic lymphohistiocytosis: An overview
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Fatal systemic cytophagic histiocytic panniculitis: A histopathologic and immunohistochemical study of multiple organ sites

J Am Acad Dermatol

J Am Acad Dermatol

J Am Acad Dermatol

J Am Acad Dermatol

Hemorrhagic diathesis associated with benign histiocytic, cytophagic panniculitis and systemic histiocytosis

Arch Intern Med

T-cell lymphoma involving subcutaneous tissue: a clinicopathologic entity commonly associated with hemophagocytic syndrome

Am J Surg Pathol

Subcutaneous T cell lymphoma: report of two additional cases and further observations

Arch Dermatol

Cytophagic histiocytic panniculitis is not always fatal

J Cutan Pathol