Skin nontumor

Panniculitis

Cytophagic histiocytic panniculitis



Last author update: 1 July 2011
Last staff update: 18 November 2020

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PubMed Search: Cytophagic histiocytic panniculitis

Mowafak Hamodat, M.B.Ch.B., M.Sc.
Page views in 2024 to date: 180
Cite this page: Hamodat M. Cytophagic histiocytic panniculitis. PathologyOutlines.com website. https://www.pathologyoutlines.com/topic/skinnontumorcytophagichistiocyticpanniculitis.html. Accessed March 28th, 2024.
Definition / general
Terminology
  • Panniculitis associated with hemophagocytic syndrome
Etiology
  • 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 features
  • Erythematosus to violaceous or hemorrhagic nodules, often in lower limbs and trunk
  • Ulceration with localized or generalized edema
Microscopic (histologic) description
  • 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
Microscopic (histologic) images

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11 year old girl with perforin gene mutation

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