Tumid lupus erythematosus

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Tumid lupus erythematosus
Other names"Lupus erythematosus tumidus" [1]
Specialty Dermatology

Tumid lupus erythematosus is a rare, but distinctive entity in which patients present with edematous erythematous plaques, usually on the trunk. [2]

Contents

Lupus erythematosus tumidus (LET) was reported by Henri Gougerot and Burnier R. in 1930. It is a photosensitive skin disorder, a different subtype of cutaneous lupus erythematosus (CLE) from discoid lupus erythematosus (DLE) or subacute CLE (SCLE). [3] LET is usually found on sun-exposed areas of the body. Skin lesions are edematous, urticarialike annular papules and plaques. Topical corticosteroids are not effective as treatment for LET, but many will respond to chloroquine. LET resolves with normal skin, no residual scarring, no hyperpigmentation or hypopigmentation. Cigarette smokers who have LET may not respond very well to chloroquine. [4] [5]

It has been suggested that it is equivalent to Jessner lymphocytic infiltrate of the skin. [6]

Signs and symptoms

The characteristic presentation of tumid lupus erythematosus is erythematous, edematous plaques that lack ulceration or scaling. [4] In contrast to discoid lupus erythematosus (DLE), there is no atrophy, scarring, or follicular plugging. Skin exposed to the elements, such as the face, upper chest (V-neck distribution),  upper back, extensor arms, and shoulders, is typically affected by tumid lupus erythematosus. [7] Rare cases of tumid lupus erythematosus affecting the lower extremities have been documented, nevertheless. [8] Tumid lupus erythematosus typically manifests itself in the summer in temperate climates. [7]

Papules and plaques of tumid lupus erythematosus can create an annular pattern in certain patients, resembling annular subacute cutaneous lupus erythematosus (SCLE), with less edema at the center. A Blaschkoid distribution, [9] [10] scalp involvement resembling alopecia areata, [11] and periorbital edema are less frequent signs of tumid lupus erythematosus. [12]

Causes

There is currently no known unique etiology for tumid lupus erythematosus. However, it has been shown that triggering variables like ultraviolet (UV) exposure can exacerbate tumid lupus erythematosus lesions. [13] Its link to autoimmune disease has generated debate; an autoimmune workup may be started if an autoimmune disease is suspected. [14] [15] It is suggested that immune dysregulation results in T cell suppression. [16] There has been evidence of a correlation between smoking and medications such as thiazide diuretics, monoclonal antibodies, angiotensin-converting enzyme inhibitors, tumor necrosis factor antagonists, and highly active antiretroviral therapy. [17] [18]

Diagnosis

The identification of consistent clinical symptoms and histopathologic findings is the basis for the diagnosis of tumid lupus erythematosus. Provocative phototesting results and antimalarial medication response are additional tests that are not usually required but can confirm a diagnosis of tumid lupus erythematosus. [7]

Proposed diagnostic criteria reflect key findings in tumid lupus erythematosus: [4]

  1. Clinical - Smooth-surfaced, succulent, urticarial-like, erythematous plaques in sun-exposed areas. [4]
  2. Histologic - There is no epidermal involvement or modification of the dermoepidermal interface; instead, there is perivascular and periadnexal lymphocytic infiltration, interstitial mucin deposition, and, in certain instances, dispersed neutrophils. [4]
  3. Phototesting - Skin lesion proliferation following exposure to ultraviolet A (UVA) and/or ultraviolet B (UVB) radiation. [4]
  4. Treatment - Quick and efficient systemic antimalarial medication treatment. [4]

Treatment

First-line treatments include photoprotection, topical calcineurin inhibitors, and intralesional and/or topical corticosteroids. Antimalarial medications like hydroxychloroquine or chloroquine should be used as part of a systemic treatment for patients who do not respond to conservative therapy or who have a severe illness. Methotrexate or mycophenolate mofetil along with folic acid supplements are examples of second-line therapy. [19] If all previous treatments are ineffective, third-line treatments such as thalidomide or lenalidomide may be considered. [4] [20] Another effective treatment for suppressive, non-curative conditions is pulse dye laser. [21] In order to keep the lesions from relapsing in these patients, trigger avoidance measures including wearing sunscreen and abstaining from smoking are essential. [19]

See also

Related Research Articles

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<span class="mw-page-title-main">Discoid lupus erythematosus</span> Autoimmune skin condition

Discoid lupus erythematosus is the most common type of chronic cutaneous lupus (CCLE), an autoimmune skin condition on the lupus erythematosus spectrum of illnesses. It presents with red, painful, inflamed and coin-shaped patches of skin with a scaly and crusty appearance, most often on the scalp, cheeks, and ears. Hair loss may occur if the lesions are on the scalp. The lesions can then develop severe scarring, and the centre areas may appear lighter in color with a rim darker than the normal skin. These lesions can last for years without treatment.

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Chilblain lupus erythematosus was initially described by Hutchinson in 1888 as an uncommon manifestation of chronic cutaneous lupus erythematosus. Chilblain lupus erythematosus is characterized by a rash that primarily affects acral surfaces that are frequently exposed to cold temperatures, such as the toes, fingers, ears, and nose. The rash is defined by oedematous skin, nodules, and tender plaques with a purple discoloration.

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References

  1. ↑ Rapini, Ronald P.; Bolognia, Jean L.; Jorizzo, Joseph L. (2007). Dermatology: 2-Volume Set. St. Louis: Mosby. ISBN   978-1-4160-2999-1.
  2. ↑ James, William; Berger, Timothy; Elston, Dirk (2005). Andrews' Diseases of the Skin: Clinical Dermatology. (10th ed.). Saunders. Page 159. ISBN   0-7216-2921-0.
  3. ↑ Gougerot H, Burnier R. Lupus érythémateux "tumidus". Bull Soc Fr Dermatol Syphiligr. 1930;37:1291-1292.
  4. 1 2 3 4 5 6 7 8 Kuhn, Annegret; Richter-Hintz, Dagmar; Oslislo, Claudia; Ruzicka, Thomas; Megahed, Mosaad; Lehmann, Percy (2000-08-01). "Lupus Erythematosus Tumidus". Archives of Dermatology. American Medical Association (AMA). 136 (8): 1033–1041. doi:10.1001/archderm.136.8.1033. ISSN   0003-987X. PMID   10926740.
  5. ↑ Callen, Jeffrey P. (2002). "Management of skin disease in patients with lupus erythematosus". Best Practice & Research Clinical Rheumatology. Elsevier BV. 16 (2): 245–264. doi:10.1053/berh.2001.0224. ISSN   1521-6942. PMID   12041952.
  6. ↑ "Jessner Lymphocytic Infiltration of the Skin: eMedicine Dermatology" . Retrieved 2010-05-22.
  7. 1 2 3 "UpToDate". UpToDate. Retrieved 2024-03-02.
  8. ↑ Stead, Jennifer; Headley, Catherine; Ioffreda, Michael; Kovarik, Carrie; Werth, Victoria (2008). "Coexistence of Tumid Lupus Erythematosus With Systemic Lupus Erythematosus and Discoid Lupus Erythematosus". JCR: Journal of Clinical Rheumatology. Ovid Technologies (Wolters Kluwer Health). 14 (6): 338–341. doi:10.1097/rhu.0b013e31817d1183. ISSN   1076-1608. PMC   2829660 . PMID   18664992.
  9. ↑ Pacheco, T R; Spates, S T; Lee, L A (2002). "Unilateral tumid lupus erythematosus". Lupus. SAGE Publications. 11 (6): 388–391. doi:10.1191/0961203302lu208cr. ISSN   0961-2033. PMID   12139378. S2CID   35133682.
  10. ↑ Hinz, Torsten; Hornung, Thorsten; Wenzel, Joerg; Bieber, Thomas (2012-03-27). "Lupus tumidus following the lines of Blaschko". International Journal of Dermatology. Wiley. 52 (12): 1615–1617. doi:10.1111/j.1365-4632.2011.05419.x. ISSN   0011-9059. PMID   22458246. S2CID   27225913.
  11. ↑ Werth, Victoria P. (1992-03-01). "Incidence of Alopecia Areata in Lupus Erythematosus". Archives of Dermatology. 128 (3): 368. doi:10.1001/archderm.1992.01680130082010. ISSN   0003-987X. PMID   1550369.
  12. ↑ Vassallo, Camilla; Colombo, Giovanni; Canevari, Frank Rikki; Brazzelli, Valeria; Ardigò, Marco; Carrera, Carlo; Cananzi, Raffaello; Borroni, Giovanni (2005-04-14). "Monolateral severe eyelid erythema and edema as unique manifestation of lupus tumidus". International Journal of Dermatology. Wiley. 44 (10): 858–860. doi:10.1111/j.1365-4632.2005.02210.x. ISSN   0011-9059. PMID   16207190. S2CID   45610534.
  13. ↑ Saleh, Dahlia; Grubbs, Hailey; Koritala, Thoyaja; Crane, Jonathan S. (2023-06-28). "Tumid Lupus Erythematosus". StatPearls Publishing. PMID   29494121 . Retrieved 2024-03-02.
  14. ↑ Jefferson, Gina D.; Aakalu, Vinay K.; Braniecki, Marylee (2017). "Tumid lupus: An unexpected diagnosis for the otolaryngologist". American Journal of Otolaryngology. Elsevier BV. 38 (2): 257–259. doi:10.1016/j.amjoto.2017.01.003. ISSN   0196-0709. PMC   5826658 . PMID   28122678.
  15. ↑ Fogagnolo, L.; Soares, T. C. B.; Senna, C. G.; Souza, E. M.; Blotta, M. H. S. L.; Cintra, M. L. (2014-09-12). "Cytotoxic granules in distinct subsets of cutaneous lupus erythematosus". Clinical and Experimental Dermatology. Oxford University Press (OUP). 39 (7): 835–839. doi:10.1111/ced.12428. ISSN   0307-6938. PMID   25214407. S2CID   21127920.
  16. ↑ Gambichler, T.; Pätzholz, J.; Schmitz, L.; Lahner, N.; Kreuter, A. (2015-03-25). "<scp>FOXP</scp>3+ and <scp>CD</scp>39+ regulatory T cells in subtypes of cutaneous lupus erythematosus". Journal of the European Academy of Dermatology and Venereology. Wiley. 29 (10): 1972–1977. doi:10.1111/jdv.13123. ISSN   0926-9959. PMID   25808110. S2CID   30625226.
  17. ↑ Böckle, B C; Sepp, N T (2014-11-19). "Smoking is highly associated with discoid lupus erythematosus and lupus erythematosus tumidus: analysis of 405 patients". Lupus. SAGE Publications. 24 (7): 669–674. doi:10.1177/0961203314559630. ISSN   0961-2033. PMID   25411260. S2CID   43483915.
  18. ↑ Schneider, Stefan W.; Staender, Sonja; Schlüter, Bernhard; Luger, Thomas A.; Bonsmann, Gisela (2006-01-01). "Infliximab-Induced Lupus Erythematosus Tumidus in a Patient With Rheumatoid Arthritis". Archives of Dermatology. American Medical Association (AMA). 142 (1): 115–116. doi:10.1001/archderm.142.1.115. ISSN   0003-987X. PMID   16415403.
  19. 1 2 Liu, Evan; Daze, Robert P; Moon, Summer (2020-05-26). "Tumid Lupus Erythematosus: A Rare and Distinctive Variant of Cutaneous Lupus Erythematosus Masquerading as Urticarial Vasculitis". Cureus. Springer Science and Business Media LLC. doi: 10.7759/cureus.8305 . ISSN   2168-8184. PMC   7320659 . PMID   32607289.
  20. ↑ Gallitano, Stephanie M.; Haskin, Alessandra (2016). "Lupus erythematosus tumidus: A case and discussion of a rare entity in black patients". JAAD Case Reports. Elsevier BV. 2 (6): 488–490. doi:10.1016/j.jdcr.2016.05.022. ISSN   2352-5126. PMC   5149049 . PMID   27981226.
  21. ↑ Truchuelo, M.T.; Boixeda, P.; Alcántara, J.; Moreno, C.; de las Heras, E.; Olasolo, P.J. (2012). "Pulsed dye laser as an excellent choice of treatment for lupus tumidus: a prospective study". Journal of the European Academy of Dermatology and Venereology. 26 (10): 1272–1279. doi:10.1111/j.1468-3083.2011.04281.x. ISSN   0926-9959. PMID   21957901.

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