Cytophagic histiocytic panniculitis

Author: Dr Mark Duffill, Hamilton, New Zealand, 2008.


Cytophagic histiocytic panniculitis — codes and concepts
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What is panniculitis?

Panniculitis is a term usually referring to a disorder of the subcutaneous fat.

What is cytophagic histiocytic panniculitis?

Cytophagic histiocytic panniculitis is an inflammatory condition of the subcutaneous fat. It is diagnosed after a biopsy has detected abnormal histiocytes which are engulfing other cells (such as red and white blood cells).

Cytophagic histiocytic panniculitis also affects other organs and is a life-threatening condition.

Cytophagic histiocytic panniculitis

What are the clinical features of cytophagic histiocytic panniculitis?

Cytophagic histiocytic panniculitis presents with painful subcutaneous nodules at multiple sites and systemic symptoms.

  • The patient feels unwell and has a fever.
  • There is a decrease in the cells and platelets in the blood.
  • The liver and spleen are enlarged.
  • Progressive liver failure develops.

Death, when it occurs, is associated with bleeding from the gastrointestinal, urinary and respiratory tracts and into the skin.  

What is the cause of cytophagic histiocytic panniculitis?

Cytophagic histiocytic panniculitis is triggered by an infection in about 50% of cases. This is usually one of the herpes viruses. The disease occurs in patients with altered immune function due to immunodeficiency, an autoimmune disease, or haematological disease.

Subcutaneous T-cell lymphoma can also present with cytophagic histiocytic panniculitis.

How is cytophagic histiocytic panniculitis diagnosed?

Biopsy of a subcutaneous nodule shows cytophagic histiocytes and inflammatory cells associated with breakdown and haemorrhage in subcutaneous fat. Abnormal lymphocytes may lead to a diagnosis of T-cell lymphoma. 

Further investigations may show damage to other organs and a bleeding tendency. There may be evidence of a preceding infection, such as a herpesvirus, and of altered immune function. If T-cell lymphoma is diagnosed, staging may find evidence of lymphoma in the lymph nodes and elsewhere.

What is the treatment of cytophagic histiocytic panniculitis?

Cytophagic histiocytic panniculitis is usually lethal if untreated.  

Symptomatic treatment consists of transfusion and corticosteroid therapy. Etoposide and ciclosporin have been reported to be helpful in some cases. Some authors recommend that cytophagic histiocytic panniculitis should be treated with CHOP-like polychemotherapy. Good results have been obtained with high dose intravenous immunoglobulin.

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Related information

 

References

  • Marzano AV, Berti E, Paulli M, Caputo R. Cytophagic histiocytic panniculitis and subcutaneous panniculitis-like T-cell lymphoma: report of 7 cases. Arch Dermatol 2000 Jul;136(7):889-96. Comment in: Arch Dermatol. 2000 Jul;136(7):922-4. PubMed.
  • Li MT, Zeng XF, Zhang FC, Tang FL. Cytophagic histiocytic panniculitis: a report of 6 cases with literature review. Zhonghua Nei Ke Za Zhi. 2004 Aug;43(8):576-9. PubMed.

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