Lupus erythematosus panniculitis

Lupus erythematosus panniculitis
Other namesLupus erythematosus profundus,[1] Lupus panniculitis,[2] "Lupus profundus",[2] and Subcutaneous lupus erythematosus[2]
SpecialtyDermatology
Not to be confused with Subacute cutaneous lupus erythematosus or Lupus erythematosus.

Lupus erythematosus panniculitis presents with subcutaneous nodules that are commonly firm, sharply defined and nontender.[1]

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.[3] Anemia, lowered C4 levels, positive rheumatoid factor, lymphopenia, and anemia are additional potential abnormalities in the laboratory.[6]

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

References

  1. ^ a b William D. James; Timothy G. Berger; Dirk M. Elston (2015). Andrews' Diseases of the Skin: Clinical Dermatology (12th ed.). Elsevier. p. 156. ISBN 978-0-323-31967-6.
  2. ^ a b c Rapini, Ronald P.; Bolognia, Jean L.; Jorizzo, Joseph L. (2007). Dermatology: 2-Volume Set. St. Louis: Mosby. ISBN 978-1-4160-2999-1.
  3. ^ a b c d e f Fraga, Javier; García-Díez, Amaro (2008). "Lupus Erythematosus Panniculitis". Dermatologic Clinics. 26 (4). Elsevier BV: 453–463. doi:10.1016/j.det.2008.06.002. ISSN 0733-8635. PMID 18793977.
  4. ^ Nousari, Hossein C.; Kimyai-Asadi, Arash; Provost, Thomas T. (1999). "Generalized lupus erythematosus profundus in a patient with genetic partial deficiency of C4". Journal of the American Academy of Dermatology. 41 (2). Elsevier BV: 362–364. doi:10.1016/s0190-9622(99)70389-0. ISSN 0190-9622. PMID 10426934.
  5. ^ Cribier, Bernard (2005). "Panniculite lupique". La Presse Médicale (in French). 34 (3). Elsevier BV: 243–248. doi:10.1016/s0755-4982(05)88257-0. ISSN 0755-4982.
  6. ^ a b c d Martens, P B; Moder, K G; Ahmed, I (January 1999). "Lupus panniculitis: clinical perspectives from a case series". The Journal of Rheumatology. 26 (1): 68–72. PMID 9918242.
  7. ^ Callen, Jeffrey P (January 5, 2006). "Cutaneous lupus erythematosus: A personal approach to management". Australasian Journal of Dermatology. 47 (1). Wiley: 13–27. doi:10.1111/j.1440-0960.2006.00217.x. ISSN 0004-8380. PMID 16405478. S2CID 27073889.
  8. ^ Bednarek, Agata; Bartoszak, Leszek; Samborski, Włodzimierz (2015). "on a patient with lupus panniculitis". Advances in Dermatology and Allergology. 1. Termedia Sp. z.o.o.: 59–62. doi:10.5114/pdia.2014.40958. ISSN 1642-395X. PMID 25821430.
  9. ^ Peters, Margot S.; Su, W. P. Daniel (1991). "Eosinophils in lupus panniculitis and morphea profunda". Journal of Cutaneous Pathology. 18 (3). Wiley: 189–192. doi:10.1111/j.1600-0560.1991.tb00151.x. ISSN 0303-6987. PMID 1918506. S2CID 29826100.
  10. ^ Peters, Margot S.; Daniel Su, W.P. (1989). "Lupus Erythematosus Panniculitis". Medical Clinics of North America. 73 (5). Elsevier BV: 1113–1126. doi:10.1016/s0025-7125(16)30622-8. ISSN 0025-7125. PMID 2671535.
  11. ^ Galindo, E Cuerda; Paz, F Sánchez de; Pérez, I Mansilla; Magdalena, O Poza; Dorado, M Martín (June 2001). "Lupus erythematosus profundus: case reports". Cutis. 67 (6): 465–467. PMID 11419017.
  12. ^ Grossberg, E; Scherschun, L; Fivenson, D P (2001). "Lupus profundus: not a benign disease". Lupus. 10 (7). SAGE Publications: 514–516. doi:10.1191/096120301678416105. ISSN 0961-2033. PMID 11480852. S2CID 20725776.
  13. ^ a b Housman, Tamara Salam; Jorizzo, Joseph L.; McCarty, Martha A.; Grummer, Sarah E.; Fleischer, Alan B.; Sutej, Paul G. (January 1, 2003). "Low-Dose Thalidomide Therapy for Refractory Cutaneous Lesions of LupusErythematosus". Archives of Dermatology. 139 (1). American Medical Association (AMA): 50–54. doi:10.1001/archderm.139.1.50. ISSN 0003-987X. PMID 12533164.
  14. ^ Morgan, K. W.; Callen, J. P. (2001-09-01). "Calcifying lupus panniculitis in a patient with subacute cutaneous lupus erythematosus: response to diltiazem and chloroquine". The Journal of Rheumatology. 28 (9): 2129–2132. ISSN 0315-162X. PMID 11550987. Retrieved February 2, 2024.
  15. ^ Yell, J.A.; Burge, S.M. (1993). "Lupus erythematosus profundus treated with clobetasol propionate under a hydrocolioid dressing". British Journal of Dermatology. 128 (1). Oxford University Press (OUP): 103. doi:10.1111/j.1365-2133.1993.tb00158.x. ISSN 0007-0963. S2CID 41328138.
  16. ^ BURROWS, N. P.; WALPORT, M. J.; HAMMOND, A. H.; DAVEY, N.; JONES, R. RUSSELL (1991). "Lupus erythematosus profundus with partial C4 deficiency responding to thalidomide". British Journal of Dermatology. 125 (1). Oxford University Press (OUP): 62–67. doi:10.1111/j.1365-2133.1991.tb06042.x. ISSN 0007-0963. PMID 1873207. S2CID 8861366.
  17. ^ Magro, Cynthia M.; Crowson, A. Neil; Kovatich, Al J.; Burns, Frank (2001). "Lupus profundus, indeterminate lymphocytic lobular panniculitis and subcutaneous T-cell lymphoma: a spectrum of subcuticular T-cell lymphoid dyscrasia". Journal of Cutaneous Pathology. 28 (5). Wiley: 235–247. doi:10.1034/j.1600-0560.2001.028005235.x. ISSN 0303-6987. PMID 11401667. S2CID 35276450.
  18. ^ Watanabe, T.; Tsuchida, T. (1996). "Lupus erythematosus profundus: a cutaneous marker for a distinct clinical subset?". British Journal of Dermatology. 134 (1): 123–125. doi:10.1046/j.1365-2133.1996.d01-735.x. PMID 8745897. S2CID 40612152.
  19. ^ Ng, Patricia Pei-Lin; Tan, Suat Hoon; Tan, Tulip (2002). "Lupus erythematosus panniculitis: a clinicopathologic study". International Journal of Dermatology. 41 (8). Wiley: 488–490. doi:10.1046/j.1365-4362.2002.01510.x. ISSN 0011-9059. PMID 12207763. S2CID 42594091.
  20. ^ Wimmershoff, M. B.; Hohenleutner, U.; Landthaler, M. (2003). "Discoid Lupus Erythematosus and Lupus Profundus in Childhood: A Report of Two Cases". Pediatric Dermatology. 20 (2). Wiley: 140–145. doi:10.1046/j.1525-1470.2003.20210.x. ISSN 0736-8046. PMID 12657012. S2CID 6348757.
  21. ^ Bacanli, A; Uzun, S; Ciftcioglu, M A; Alpsoy, E (2005). "A case of lupus erythematosus profundus with unusual manifestations". Lupus. 14 (5). SAGE Publications: 403–405. doi:10.1191/0961203305lu2088cr. ISSN 0961-2033. PMID 15934442. S2CID 8871456.
  22. ^ Nitta, Y. (1997). "Lupus erythematosus profundus associated with neonatal lupus erythematosus". British Journal of Dermatology. 136 (1): 112–114. doi:10.1046/j.1365-2133.1997.d01-1154.x. PMID 9039307. S2CID 2491393.

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