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Lupus erythematosus panniculitis

MedGen UID:
14589
•Concept ID:
C0030327
•
Disease or Syndrome
Synonyms: Lupus Erythematosus Panniculitides; Lupus Erythematosus Panniculitis; Lupus Erythematosus Profundus; Lupus Panniculitides; Lupus Panniculitis; Lupus Profundus; Panniculitides, Lupus; Panniculitides, Lupus Erythematosus; Panniculitis, Lupus; Panniculitis, Lupus Erythematosus
SNOMED CT: Lupus erythematosus profundus (15084002); Lupus panniculitis (239888002)
 
Monarch Initiative: MONDO:0019561
Orphanet: ORPHA90285

Definition

A rare form of chronic cutaneous lupus erythematosus characterized by recurrent, indurated, erythematous plaques and subcutaneous nodules with normal overlying epidermis, occurring predominantly on the face, upper arms, trunk, buttocks, and thighs. The lesions can ulcerate and lead to scarring. Histological findings include lobular lymphocytic panniculitis, hyaline fat necrosis, mucin deposition, and calcification. The condition may be associated with discoid or systemic lupus erythematosus. [from ORDO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVLupus erythematosus panniculitis
Follow this link to review classifications for Lupus erythematosus panniculitis in Orphanet.

Professional guidelines

PubMed

Treatment of Childhood-Onset Lupus Erythematosus Panniculitis With Rituximab.
Correll CK, Miller DD, Maguiness SM
JAMA Dermatol 2020 May 1;156(5):566-569. doi: 10.1001/jamadermatol.2019.4984. PMID: 32049306Free PMC Article
Update on management of connective tissue panniculitides.
Braunstein I, Werth VP
Dermatol Ther 2012 Mar-Apr;25(2):173-82. doi: 10.1111/j.1529-8019.2012.01489.x. PMID: 22741936Free PMC Article

Recent clinical studies

Etiology

Subcutaneous panniculitis-like T cell lymphoma arising in association with chronic lymphocytic leukaemia.
Kulumani Mahadevan LS, Ozdemirli M
BMJ Case Rep 2021 Sep 27;14(9) doi: 10.1136/bcr-2021-243490. PMID: 34580127Free PMC Article
Treatment of Childhood-Onset Lupus Erythematosus Panniculitis With Rituximab.
Correll CK, Miller DD, Maguiness SM
JAMA Dermatol 2020 May 1;156(5):566-569. doi: 10.1001/jamadermatol.2019.4984. PMID: 32049306Free PMC Article
Useful Parameters for Distinguishing Subcutaneous Panniculitis-like T-Cell Lymphoma From Lupus Erythematosus Panniculitis.
LeBlanc RE, Tavallaee M, Kim YH, Kim J
Am J Surg Pathol 2016 Jun;40(6):745-54. doi: 10.1097/PAS.0000000000000596. PMID: 26796503
Atypical lymphocytic lobular panniculitis.
Magro CM, Crowson AN, Byrd JC, Soleymani AD, Shendrik I
J Cutan Pathol 2004 Apr;31(4):300-6. doi: 10.1111/j.0303-6987.2004.0165.x. PMID: 15005686
Panniculitis: recent developments and observations.
White WL, Wieselthier JS, Hitchcock MG
Semin Cutan Med Surg 1996 Dec;15(4):278-99. doi: 10.1016/s1085-5629(96)80042-3. PMID: 9069596

Diagnosis

Useful Parameters for Distinguishing Subcutaneous Panniculitis-like T-Cell Lymphoma From Lupus Erythematosus Panniculitis.
LeBlanc RE, Tavallaee M, Kim YH, Kim J
Am J Surg Pathol 2016 Jun;40(6):745-54. doi: 10.1097/PAS.0000000000000596. PMID: 26796503
Lupus erythematosus panniculitis.
Fraga J, García-Díez A
Dermatol Clin 2008 Oct;26(4):453-63, vi. doi: 10.1016/j.det.2008.06.002. PMID: 18793977
Atypical lymphocytic lobular panniculitis.
Magro CM, Crowson AN, Byrd JC, Soleymani AD, Shendrik I
J Cutan Pathol 2004 Apr;31(4):300-6. doi: 10.1111/j.0303-6987.2004.0165.x. PMID: 15005686
Panniculitis: recent developments and observations.
White WL, Wieselthier JS, Hitchcock MG
Semin Cutan Med Surg 1996 Dec;15(4):278-99. doi: 10.1016/s1085-5629(96)80042-3. PMID: 9069596
Lupus erythematosus panniculitis.
Peters MS, Su WP
Med Clin North Am 1989 Sep;73(5):1113-26. doi: 10.1016/s0025-7125(16)30622-8. PMID: 2671535

Therapy

Treatment of Childhood-Onset Lupus Erythematosus Panniculitis With Rituximab.
Correll CK, Miller DD, Maguiness SM
JAMA Dermatol 2020 May 1;156(5):566-569. doi: 10.1001/jamadermatol.2019.4984. PMID: 32049306Free PMC Article
Panniculitides of particular interest to the rheumatologist.
Morita TCAB, Trés GFS, García MSC, Halpern I, Criado PR, de Carvalho JF
Adv Rheumatol 2019 Aug 1;59(1):35. doi: 10.1186/s42358-019-0077-5. PMID: 31370889
Atypical lymphocytic lobular panniculitis.
Magro CM, Crowson AN, Byrd JC, Soleymani AD, Shendrik I
J Cutan Pathol 2004 Apr;31(4):300-6. doi: 10.1111/j.0303-6987.2004.0165.x. PMID: 15005686
Dermatologic diseases of the breast and nipple.
Whitaker-Worth DL, Carlone V, Susser WS, Phelan N, Grant-Kels JM
J Am Acad Dermatol 2000 Nov;43(5 Pt 1):733-51; quiz 752-4. doi: 10.1067/mjd.2000.109303. PMID: 11050577
Connective tissue panniculitis.
Winkelmann RK, Padilha-Goncalves A
Arch Dermatol 1980 Mar;116(3):291-4. PMID: 7369744

Prognosis

Lupus mastitis: a clinicopathologic review and addition of a case.
Summers TA Jr, Lehman MB, Barner R, Royer MC
Adv Anat Pathol 2009 Jan;16(1):56-61. doi: 10.1097/PAP.0b013e3181915ff7. PMID: 19098467
Atypical lymphocytic lobular panniculitis.
Magro CM, Crowson AN, Byrd JC, Soleymani AD, Shendrik I
J Cutan Pathol 2004 Apr;31(4):300-6. doi: 10.1111/j.0303-6987.2004.0165.x. PMID: 15005686
Lupus profundus, indeterminate lymphocytic lobular panniculitis and subcutaneous T-cell lymphoma: a spectrum of subcuticular T-cell lymphoid dyscrasia.
Magro CM, Crowson AN, Kovatich AJ, Burns F
J Cutan Pathol 2001 May;28(5):235-47. doi: 10.1034/j.1600-0560.2001.028005235.x. PMID: 11401667
Lupus erythematosus panniculitis.
Peters MS, Su WP
Med Clin North Am 1989 Sep;73(5):1113-26. doi: 10.1016/s0025-7125(16)30622-8. PMID: 2671535
Connective tissue panniculitis.
Winkelmann RK, Padilha-Goncalves A
Arch Dermatol 1980 Mar;116(3):291-4. PMID: 7369744

Clinical prediction guides

Treatment of Childhood-Onset Lupus Erythematosus Panniculitis With Rituximab.
Correll CK, Miller DD, Maguiness SM
JAMA Dermatol 2020 May 1;156(5):566-569. doi: 10.1001/jamadermatol.2019.4984. PMID: 32049306Free PMC Article
Panniculitis in childhood-onset systemic lupus erythematosus: a multicentric cohort study.
Verdier M, Anuardo P, Gormezano NWS, Romiti R, Campos LMA, Aikawa NE, Pereira RMR, Terreri MT, Magalhães CS, Ferreira JCOA, Silva MFC, Ferriani M, Sakamoto AP, Ferriani VPL, Centeville M, Sato J, Santos MC, Bonfá E, Silva CA
Adv Rheumatol 2019 Jan 18;59(1):3. doi: 10.1186/s42358-019-0049-9. PMID: 30658703
Panniculitis: recent developments and observations.
White WL, Wieselthier JS, Hitchcock MG
Semin Cutan Med Surg 1996 Dec;15(4):278-99. doi: 10.1016/s1085-5629(96)80042-3. PMID: 9069596
Lipomembranous (membranocystic) fat necrosis. Clinicopathologic correlation of 38 cases.
Snow JL, Su WP
Am J Dermatopathol 1996 Apr;18(2):151-5. doi: 10.1097/00000372-199604000-00007. PMID: 8739989
Lupus erythematosus panniculitis with partial genetic deficiency of C2 and C4 in a child.
Taïeb A, Hehunstre JP, Goetz J, Surlève Bazeille JE, Fizet D, Hauptmann G, Maleville J
Arch Dermatol 1986 May;122(5):576-82. doi: 10.1001/archderm.1986.01660170106030. PMID: 3707176

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