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Cytophagic histiocytic panniculitis

MedGen UID:
590528
•Concept ID:
C0406594
•
Disease or Syndrome
Synonyms: CHP; cytophagic histiocytic panniculitis; Winkelmann cytophagic panniculitis
SNOMED CT: Cytophagic histiocytic panniculitis (238883003)
Modes of inheritance:
Unknown inheritance
MedGen UID:
989040
•Concept ID:
CN307042
•
Finding
Source: Orphanet
Hereditary clinical entity whose mode of inheritance is unknown.
See: This record
Unknown inheritance (Orphanet)
 
Monarch Initiative: MONDO:0019789
Orphanet: ORPHA94087

Definition

Cytophagic histiocytic panniculitis (CHP) is a very rare form of panniculitis manifesting as recurrent multiple subcutaneous nodules (which may progressively become ecchymotic and ulcerated), and histologically characterized by lobular panniculitis with lymphocytic and histiocytic infiltration in the subcutaneous adipose tissue. [from ORDO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVCytophagic histiocytic panniculitis
Follow this link to review classifications for Cytophagic histiocytic panniculitis in Orphanet.

Professional guidelines

PubMed

Treatment of T-cell non-Hodgkin's lymphoma.
Evens AM, Gartenhaus RB
Curr Treat Options Oncol 2004 Aug;5(4):289-303. doi: 10.1007/s11864-004-0020-8. PMID: 15233906

Recent clinical studies

Etiology

Cytophagic histiocytic panniculitis and hemophagocytic lymphohistiocytosis: an overview.
Aronson IK, Worobec SM
Dermatol Ther 2010 Jul-Aug;23(4):389-402. doi: 10.1111/j.1529-8019.2010.01339.x. PMID: 20666826
Panniculitis in childhood and adolescence.
Moraes AJ, Soares PM, Zapata AL, Lotito AP, Sallum AM, Silva CA
Pediatr Int 2006 Feb;48(1):48-53. doi: 10.1111/j.1442-200X.2006.02169.x. PMID: 16490070
Treatment of T-cell non-Hodgkin's lymphoma.
Evens AM, Gartenhaus RB
Curr Treat Options Oncol 2004 Aug;5(4):289-303. doi: 10.1007/s11864-004-0020-8. PMID: 15233906
Cytophagic histiocytic panniculitis improved by combined CHOP and cyclosporin A treatment.
Ito M, Ohira H, Miyata M, Suzuki T, Sato Y, Kaise S, Nishimaki T, Sakuma H, Nihei Y, Iwatsuki K, Kasukawa R
Intern Med 1999 Mar;38(3):296-301. doi: 10.2169/internalmedicine.38.296. PMID: 10337947
Hemophagocytic lymphohistiocytosis: a hemophagocytic syndrome.
Favara BE
Semin Diagn Pathol 1992 Feb;9(1):63-74. PMID: 1561489

Diagnosis

Cytophagic histiocytic panniculitis leading to a diagnosis of acute myeloid leukemia with monocytic differentiation: A case report and literature review.
Arnoff TE, Mirza FN, Yumeen S, Ben Khadra S, George DD, Robinson-Bostom L
J Cutan Pathol 2024 Sep;51(9):658-661. Epub 2024 May 20 doi: 10.1111/cup.14659. PMID: 38769706
Panniculitis: A Cardinal Sign of Autoinflammation.
Borges T, Silva S
Curr Rheumatol Rev 2024;20(4):350-360. doi: 10.2174/0115733971254702231020060633. PMID: 37921131
Adult-onset Still's Disease Secondary to Cytophagic Histiocytic Panniculitis.
Yoshida A, Sugita K, Yamada N, Goto H, Yamamoto O
Acta Derm Venereol 2022 Jan 26;102:adv00639. doi: 10.2340/actadv.v101.568. PMID: 34806757Free PMC Article
Cytophagic histiocytic panniculitis with haemophagocytosis in a patient with familial multiple lipomatosis and review of the literature.
Krilis M, Miyakis S
Mod Rheumatol 2012 Feb;22(1):158-62. Epub 2011 Jul 6 doi: 10.1007/s10165-011-0487-7. PMID: 21732050
Cytophagic histiocytic panniculitis and hemophagocytic lymphohistiocytosis: an overview.
Aronson IK, Worobec SM
Dermatol Ther 2010 Jul-Aug;23(4):389-402. doi: 10.1111/j.1529-8019.2010.01339.x. PMID: 20666826

Therapy

Cytophagic histiocytic panniculitis with haemophagocytosis in a patient with familial multiple lipomatosis and review of the literature.
Krilis M, Miyakis S
Mod Rheumatol 2012 Feb;22(1):158-62. Epub 2011 Jul 6 doi: 10.1007/s10165-011-0487-7. PMID: 21732050
Cytophagic histiocytic panniculitis after H1N1 vaccination: a case report and review of the cutaneous side effects of influenza vaccines.
Pauwels C, Livideanu CB, Maza A, Lamant L, Paul C
Dermatology 2011;222(3):217-20. Epub 2011 Apr 13 doi: 10.1159/000326912. PMID: 21494027
Treatment of T-cell non-Hodgkin's lymphoma.
Evens AM, Gartenhaus RB
Curr Treat Options Oncol 2004 Aug;5(4):289-303. doi: 10.1007/s11864-004-0020-8. PMID: 15233906
Subcutaneous panniculitic T-cell lymphoma and cytophagic histiocytic panniculitis.
Yung A, Snow J, Jarrett P
Australas J Dermatol 2001 Aug;42(3):183-7. doi: 10.1046/j.1440-0960.2001.00511.x. PMID: 11488712
Successful treatment of severe cytophagic histiocytic panniculitis with cyclosporine A.
Ostrov BE, Athreya BH, Eichenfield AH, Goldsmith DP
Semin Arthritis Rheum 1996 Jun;25(6):404-13. doi: 10.1016/s0049-0172(96)80005-9. PMID: 8792512

Prognosis

Cytophagic Histiocytic Panniculitis Associated with Myelodysplastic Syndrome.
Kimura R, Sugita K, Goto H, Yamamoto O
Acta Derm Venereol 2019 Jan 1;99(1):97-98. doi: 10.2340/00015555-3043. PMID: 30226527
Cytophagic histiocytic panniculitis and hemophagocytic lymphohistiocytosis: an overview.
Aronson IK, Worobec SM
Dermatol Ther 2010 Jul-Aug;23(4):389-402. doi: 10.1111/j.1529-8019.2010.01339.x. PMID: 20666826
Treatment of T-cell non-Hodgkin's lymphoma.
Evens AM, Gartenhaus RB
Curr Treat Options Oncol 2004 Aug;5(4):289-303. doi: 10.1007/s11864-004-0020-8. PMID: 15233906
Cytophagic histiocytic panniculitis--a syndrome associated with benign and malignant panniculitis: case comparison and review of the literature.
Craig AJ, Cualing H, Thomas G, Lamerson C, Smith R
J Am Acad Dermatol 1998 Nov;39(5 Pt 1):721-36. doi: 10.1016/s0190-9622(98)70044-1. PMID: 9810888
Successful treatment of severe cytophagic histiocytic panniculitis with cyclosporine A.
Ostrov BE, Athreya BH, Eichenfield AH, Goldsmith DP
Semin Arthritis Rheum 1996 Jun;25(6):404-13. doi: 10.1016/s0049-0172(96)80005-9. PMID: 8792512

Clinical prediction guides

Bilateral ptosis as first presentation of cytophagic histiocytic panniculitis: a case report.
Liao W, Xiao S, Yong J, Fan S, Fang W, Zheng Y, Liu J
BMC Ophthalmol 2017 Jul 1;17(1):112. doi: 10.1186/s12886-017-0511-6. PMID: 28666424Free PMC Article
Efficacy of intravenous cyclosporine in a case of cytophagic histiocytic panniculitis complicated by haemophagocytic syndrome after visceral leishmania infection.
Merelli M, Quartuccio L, Bassetti M, Pecori D, Gandolfo S, Avellini C, De Vita S
Clin Exp Rheumatol 2015 Nov-Dec;33(6):906-9. Epub 2015 Nov 17 PMID: 26575530
Cytophagic histiocytic panniculitis and subcutaneous panniculitis-like T-cell lymphoma: report of 7 cases.
Marzano AV, Berti E, Paulli M, Caputo R
Arch Dermatol 2000 Jul;136(7):889-96. doi: 10.1001/archderm.136.7.889. PMID: 10890991
Cytophagic histiocytic panniculitis--a syndrome associated with benign and malignant panniculitis: case comparison and review of the literature.
Craig AJ, Cualing H, Thomas G, Lamerson C, Smith R
J Am Acad Dermatol 1998 Nov;39(5 Pt 1):721-36. doi: 10.1016/s0190-9622(98)70044-1. PMID: 9810888
Cytophagic histiocytic panniculitis with atypical lymphocytes in peripheral blood.
Makino S, Kamura J
Intern Med 1992 Nov;31(11):1317-21. doi: 10.2169/internalmedicine.31.1317. PMID: 1295632

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