Cytophagic histiocytic panniculitis with fever, cytopenia, liver failure, and terminal hemorrhagic diathesis

doi:10.1016/S0190-9622(81)70022-7

Journal of the American Academy of Dermatology

Volume 4, Issue 2, February 1981, Pages 181-194

https://doi.org/10.1016/S0190-9622(81)70022-7 Get rights and content

We have seen five adult patients with a clinical picture of recurrent histiocytic, cytophagic panniculitis, cytopenia, abnormal liver function tests, and a terminal, febrile bleeding diathesis. Originally thought to have Weber-Christian disease, these patients, we believe, represent a unique syndrome: lobular, histiocytic, cytophagic panniculitis. Erythrophagocytosis and cytophagocytosis are readily observed, but the cells do not show malignant features. Histiocytosis can be found at times in the bone marrow, lymph nodes, liver, spleen, and serosal tissues, as well as in the skin and subcutaneous tissues. The terminal hemorrhage in these patients is characterized by features of pancytopenia, liver failure, and intravascular coagulation. This disease may be separated from malignant histiocytosis by the chronic course, the primary involvement of the adipose tissue, and the benign histiocytes in the infiltrate. It has some similarities to other regional histiocytoses such as sinus histiocytosis, intestinal histiocytosis, and splenic histiocytosis.

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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.
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¹: From the Department of Dermatology, Mayo Clinic and Mayo Foundation.

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Cytophagic histiocytic panniculitis with fever, cytopenia, liver failure, and terminal hemorrhagic diathesis

Hum Pathol

Cytophagic panniculitis

G Ital Dermatol Venereol

Transient histiocytosis with striking phagocytosis of platelets, leukocytes, and erythrocytes

Arch Intern Med

An unusual case which began with subcutaneous panniculitis followed by fever, severe hepatic involvement and hyperlipidemia

Acta Pathol Jpn

Generalized proteolysis in a young woman with Weber-Christian disease (nodular nonsuppurative panniculitis)

Scand J Haematol

Reacción leucoeritroblástica, trombopenia y células gigantes multinucleadas en médula ósea en un caso de paniculitis recidivante de Weber-Christian

Rev Clin Esp

Fatal panniculitis

Arch Pathol

Congenital hemophagocytic reticulosis

Am J Clin Pathol

Familial haemophagocytic reticulosis

Arch Dis Child

Malignant histiocytosis: Report of five Chinese patients

Cancer