Lupus erythematosus panniculitis explained

Lupus erythematosus panniculitis
Synonyms:Lupus erythematosus profundus, Lupus panniculitis, "Lupus profundus",[1] and Subcutaneous lupus erythematosus
Field:Dermatology

Lupus erythematosus panniculitis presents with subcutaneous nodules that are commonly firm, sharply defined and nontender.[2]

Signs and symptoms

Lupus erythematosus panniculitis consists of soft, deep subcutaneous plaques or nodules that can occasionally appear in crops. Proximal extremities, in particular the lateral aspects of the arms and shoulders, face, trunk, buttocks breast, and scalp, are typically involved. Lesions can affect several areas at once or just one.[3] Very few generalized forms exist.[4] There is a preference for the face in pediatric patients.[5] In cases of discoid lupus erythematosus, the skin surface may exhibit scaling, atrophy, follicular plugging, telangiectasias, depigmentation, or ulceration. Erythema is a common clinical feature in the overlying skin.[6] The clinical course of lupus erythematosus panniculitis lesions is chronic and relapsing. There are areas of lipoatrophic depression left behind after the nodules resolve.[3]

Causes

Lupus erythematosus panniculitis may manifest independently or in conjunction with discoid lupus erythematosus (DLE) or systemic lupus erythematosus (SLE).[6] Lupus erythematosus panniculitis occurs independently twice as often as when combined with SLE or DLE.[7]

Diagnosis

The gold standard for diagnosing a lesioned skin specimen is its histopathological examination.[8] Two authors have outlines a histopathologic diagnostic criteria for lupus erythematosus panniculitis. The major criteria includes calcification, periseptal or lobular lymphocytic panniculitis, lymphocytic aggregates and lymphoid follicle formation, and hyaline fat necrosis. The minor criteria includes discoid lupus erythematosus alterations in the skin layer above, lymphocytic vascular inflammation, subepidermal zone hyalinization, mucin deposition, histiocytes and tiny granulomas, and plasma cell and eosinophil infiltrates.[9] [10]

Although serologic analyses are frequently normal, it is occasionally possible to show a positive antinuclear antibody titer. Antibodies against double-stranded DNA are less common. The serology for syphilis may give a false positive. Anemia, lowered C4 levels, positive rheumatoid factor, lymphopenia, and anemia are additional potential abnormalities in the laboratory.

Outlook

Despite being regarded as a benign form of lupus erythematosus,[11] lupus erythematosus panniculitis can occasionally cause significant morbidity due to the disease's damaging cosmetic effects and disability caused by painful lesions.[12]

Treatment

Antimalarials are regarded as the traditional course of care and, for certain patients, their first option.[13] Diltiazem in combination can be beneficial for patients with calcified nodules.[14] Topical corticosteroids, primarily clobetasol propionate, may be added occasionally.[15]

Thalidomide is regarded as the most effective treatment for lupus erythematosus panniculitis.[13] [16]

Epidemiology

Lupus erythematosus panniculitis may impact both sexes, but women are more likely to experience it. The percentages of frequency in case series vary, with different reports having female-to-male ratios of 2:1, 3:1, 4:1, and 9:1.[17] [3]

Although the age at which the disease manifests itself varies as well, most patients are between 30 and 60 years old.[3] The median age was 41 years old in a set of 40 cases,[6] and 42 years old in another Spanish series.[3] Asian patients appear to be affected by lupus erythematosus panniculitis at a slightly younger age group, with a mean age of 31.[18] [19] Cases involving children are uncommon.[20] [21] Rarely, neonatal lupus has been linked to lupus erythematosus panniculitis.[22]

See also

Further reading

External links

Notes and References

  1. Book: Rapini, Ronald P. . Bolognia, Jean L. . Jorizzo, Joseph L. . Dermatology: 2-Volume Set . Mosby . St. Louis . 2007 . 978-1-4160-2999-1 .
  2. Book: William D. James . Timothy G. Berger . Dirk M. Elston . Andrews' Diseases of the Skin: Clinical Dermatology . 12th . . 2015 . 156 . 978-0-323-31967-6.
  3. Fraga . Javier . García-Díez . Amaro . Lupus Erythematosus Panniculitis . Dermatologic Clinics . Elsevier BV . 26 . 4 . 2008 . 0733-8635 . 10.1016/j.det.2008.06.002 . 453–463. 18793977 .
  4. Nousari . Hossein C. . Kimyai-Asadi . Arash . Provost . Thomas T. . Generalized lupus erythematosus profundus in a patient with genetic partial deficiency of C4 . Journal of the American Academy of Dermatology . Elsevier BV . 41 . 2 . 1999 . 0190-9622 . 10.1016/s0190-9622(99)70389-0 . 362–364. 10426934 .
  5. Cribier . Bernard . Panniculite lupique . La Presse Médicale . Elsevier BV . 34 . 3 . 2005 . 0755-4982 . 10.1016/s0755-4982(05)88257-0 . 243–248 . fr.
  6. Martens . P B . Moder . K G . Ahmed . I . Lupus panniculitis: clinical perspectives from a case series . The Journal of Rheumatology . January 1999 . 26 . 1 . 68–72 . 9918242.
  7. Callen . Jeffrey P . Cutaneous lupus erythematosus: A personal approach to management . Australasian Journal of Dermatology . Wiley . 47 . 1 . January 5, 2006 . 0004-8380 . 10.1111/j.1440-0960.2006.00217.x . 13–27. 16405478 . 27073889 .
  8. Bednarek . Agata . Bartoszak . Leszek . Samborski . Włodzimierz . on a patient with lupus panniculitis . Advances in Dermatology and Allergology . Termedia Sp. z.o.o. . 1 . 2015 . 1642-395X . 10.5114/pdia.2014.40958 . 59–62. 25821430 .
  9. Peters . Margot S. . Su . W. P. Daniel . Eosinophils in lupus panniculitis and morphea profunda . Journal of Cutaneous Pathology . Wiley . 18 . 3 . 1991 . 0303-6987 . 10.1111/j.1600-0560.1991.tb00151.x . 189–192. 1918506 . 29826100 .
  10. Peters . Margot S. . Daniel Su . W.P. . Lupus Erythematosus Panniculitis . Medical Clinics of North America . Elsevier BV . 73 . 5 . 1989 . 0025-7125 . 10.1016/s0025-7125(16)30622-8 . 1113–1126. 2671535 .
  11. Galindo . E Cuerda . Paz . F Sánchez de . Pérez . I Mansilla . Magdalena . O Poza . Dorado . M Martín . Lupus erythematosus profundus: case reports . Cutis . June 2001 . 67 . 6 . 465–467 . 11419017.
  12. Grossberg . E . Scherschun . L . Fivenson . D P . Lupus profundus: not a benign disease . Lupus . SAGE Publications . 10 . 7 . 2001 . 0961-2033 . 10.1191/096120301678416105 . 514–516. 11480852 . 20725776 .
  13. Housman . Tamara Salam . Jorizzo . Joseph L. . McCarty . Martha A. . Grummer . Sarah E. . Fleischer . Alan B. . Sutej . Paul G. . Low-Dose Thalidomide Therapy for Refractory Cutaneous Lesions of LupusErythematosus . Archives of Dermatology . American Medical Association (AMA) . 139 . 1 . January 1, 2003 . 50–54 . 0003-987X . 10.1001/archderm.139.1.50 . 12533164 .
  14. Morgan . K. W. . Callen . J. P. . Calcifying lupus panniculitis in a patient with subacute cutaneous lupus erythematosus: response to diltiazem and chloroquine. . The Journal of Rheumatology . 28 . 9 . 2001-09-01 . 0315-162X . 11550987 . 2129–2132 . February 2, 2024.
  15. Yell . J.A. . Burge . S.M. . Lupus erythematosus profundus treated with clobetasol propionate under a hydrocolioid dressing . British Journal of Dermatology . Oxford University Press (OUP) . 128 . 1 . 1993 . 0007-0963 . 10.1111/j.1365-2133.1993.tb00158.x . 103. 41328138 .
  16. BURROWS . N. P. . WALPORT . M. J. . HAMMOND . A. H. . DAVEY . N. . JONES . R. RUSSELL . Lupus erythematosus profundus with partial C4 deficiency responding to thalidomide . British Journal of Dermatology . Oxford University Press (OUP) . 125 . 1 . 1991 . 0007-0963 . 10.1111/j.1365-2133.1991.tb06042.x . 62–67. 1873207 . 8861366 .
  17. Magro . Cynthia M. . Crowson . A. Neil . Kovatich . Al J. . Burns . Frank . Lupus profundus, indeterminate lymphocytic lobular panniculitis and subcutaneous T-cell lymphoma: a spectrum of subcuticular T-cell lymphoid dyscrasia . Journal of Cutaneous Pathology . Wiley . 28 . 5 . 2001 . 0303-6987 . 10.1034/j.1600-0560.2001.028005235.x . 235–247. 11401667 . 35276450 .
  18. Watanabe . T. . Tsuchida . T. . Lupus erythematosus profundus: a cutaneous marker for a distinct clinical subset? . British Journal of Dermatology . 134 . 1 . 1996 . 10.1046/j.1365-2133.1996.d01-735.x . 123–125. 8745897 . 40612152 .
  19. Ng . Patricia Pei-Lin . Tan . Suat Hoon . Tan . Tulip . Lupus erythematosus panniculitis: a clinicopathologic study . International Journal of Dermatology . Wiley . 41 . 8 . 2002 . 0011-9059 . 10.1046/j.1365-4362.2002.01510.x . 488–490. 12207763 . 42594091 .
  20. Wimmershoff . M. B. . Hohenleutner . U. . Landthaler . M. . Discoid Lupus Erythematosus and Lupus Profundus in Childhood: A Report of Two Cases . Pediatric Dermatology . Wiley . 20 . 2 . 2003 . 0736-8046 . 10.1046/j.1525-1470.2003.20210.x . 140–145. 12657012 . 6348757 .
  21. Bacanli . A . Uzun . S . Ciftcioglu . M A . Alpsoy . E . A case of lupus erythematosus profundus with unusual manifestations . Lupus . SAGE Publications . 14 . 5 . 2005 . 0961-2033 . 10.1191/0961203305lu2088cr . 403–405. 15934442 . 8871456 .
  22. Nitta . Y. . Lupus erythematosus profundus associated with neonatal lupus erythematosus . British Journal of Dermatology . 136 . 1 . 1997 . 10.1046/j.1365-2133.1997.d01-1154.x . 112–114. 9039307 . 2491393 .

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