Skin-nontumor / Clinical Dermatology - Cytophagic histiocytic panniculitis

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Other dermatoses

Cytophagic histiocytic panniculitis

Reviewer: Mowafak Hamodat, MB.CH.B, MSc., FRCPC, Eastern Health, St. Johns, Canada (see Reviewers page)
Revised: 20 July 2011, last major update July 2011
Copyright: (c) 2002-2011, PathologyOutlines.com, Inc.

Description
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● Rare panniculitis associated with hemophagocytic syndrome (Dermatol Ther 2010;23:389)

Terminology
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● Panniculitis associated with hemophagocytic syndrome

Clinical features
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● Associated with CMV, EBV, HIV, bacteria, fungi and parasites
● Occurs post-bone marrow transplant, with SLE, as adverse reaction to interferon–alpha therapy
● Associated with T cell lymphoma
● Presents with spiking fever, erythematous subcutaneous skin nodules, anemia, leukopenia
● May be fatal with multisystemic involvement

Clinical description
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● Erythematosus to violaceous or hemorrhagic nodules, often in lower limbs and trunk
● Ulceration with localized or generalized edema

Clinical images
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Various images

Micro description
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● Lobular or partially septal distribution of florid lobular panniculitis, with large histiocytes containing eosinophilic cytoplasm; also neutrophils and lymphocytes
● Red cell extravasation typically present with frank hemorrhage
● Erythrophagocytosis, phagocytosis of lymphocytes or nuclear debris
● Enlarged histiocytes may be described as "bean bag cell"
● Usually no giant cells / granulomas

Micro images
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Various images in 11 year old girl with perforin gene mutation

Differential diagnosis
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● Subcutaneous T cell panniculitis lymphoma
● Angiocentric lymphoma
● Cutaneous Rosai Dorfman disease

End of Skin-nontumor / Clinical Dermatology > Other dermatoses > Cytophagic histiocytic panniculitis

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