Cutaneous Lupus Erythematosus Presenting as Persistent Periorbital Edema: A Report of Two Cases
DOI:
https://doi.org/10.12970/2310-9874.2020.08.04Keywords:
Autoimmune diseases, lupus erythematosus tumidus, cutaneous lupus, periorbital edema, lupus tumidus treatment.Abstract
Background: Cutaneous lupus erythematosus (CLE) is a chronic, autoimmune, inflammatory disease characterized by a multi-factorial etiology and a spectrum of cutaneous manifestations with variable evolution. Several CLE variants exist with varying clinical and histopathologic features, depending on the extent of epidermal and/or dermal involvement. Persistent, periorbital edema as the sole clinical manifestation of CLE is rarely reported. We report two cases of lupus tumidus presenting with periorbital edema.
Case Presentations: This is the case of two, unrelated, middle-aged patients with a chronic history of periorbital edema whose review of systems were negative for systemic involvement. Physical examination in both cases revealed periorbital, non-tender, erythematous plaques associated with significant non-pitting edema. No epidermal changes were noted. Rheumatologic and thyroid studies were unremarkable. Histopathology in both patients showed a superficial and deep perivascular lymphocytic infiltrate as well as mucin deposition. After a clinical and histopathologic correlation, a diagnosis of CLE in the spectrum of lupus tumidus was made. In both cases, clinical improvement over the course of several weeks was observed with a taper of oral prednisone 40 mg daily and hydrochloroquinine 200 mg twice daily.
Discussion: Several CLE variants exist in a clinical spectrum where cutaneous and histopathologic findings range from predominant epidermal involvement to mostly dermal involvement. Although uncommon, periorbital edema may be the sole manifestation of CLE. It is important to suspect CLE in cases of persistent, periocular edema in order to avoid delays in diagnosis and treatment.
References
Lee L, Werth V. Lupus Erythematosus. In: Bolognia JL, Jorizzo JL, Rapini RP, eds. Dermatology. St Louis: Mosby Elsevier; 2012. p. 615-29.
Durosaro O, Davis M, Reed K, Rohlinger, A. Incidence of cutaneous lupus erythematosus, 1965-2005: a population-based study. Arch Dermatol 2009; 145: 249-53. https://doi.org/10.1001/archdermatol.2009.21
Smith C, Pinals R. Periorbital Edema in Systemic Lupus Erythematosus. Arch Intern Med 1982; 142: 1711-12. https://doi.org/10.1001/archinte.1982.00340220135022
Erras S, Benjilali L, Essaadouni L. Periorbital edema as initial manifestation of chronic cutaneous lupus erythematosus. Pan African Medical Journal 2012; 12: 57-58.
Kuhn A, Richter-Hintz D, Oslislo C, Ruzicka T, Megahed M, Lehmann P. Lupus erythematosus tumidus - a neglected subset of cutaneous Lupus erythematosus: report of 40 cases. Arch Dermatol 2000; 136: 1033-34. https://doi.org/10.1001/archderm.136.8.1033
Cozzani E, Christana K, Rongiletti F, Rebora A, Parodi A. Lupus erythematosus tumidus: clinical, histopathological and serological aspects and therapy response of 21 patients. Eur J Dermatol 2010; 20: 797.
Schepis C, Lentini M, Siragusa M, Batolo D. ACE-inhibitor-induced drug eruption resembling lymphocytic infiltration and lupus erythematosus tumidus. Dermatology 2004; 208: 354-6. https://doi.org/10.1159/000077849
Cyran S, Douglass MC, Silverstein JL. Chronic cutaneous lupus erythematosus presenting as periorbital edema and erythema. J Am Acad Dermatol 1992; 26: 334-38. https://doi.org/10.1016/0190-9622(92)70049-L
Williams WL, Ramos-Caro FA. Acute periorbital mucinosis in discoid lupus erythematosus. J Am Acad Dermatol 1999; 41: 871-3. https://doi.org/10.1016/S0190-9622(99)70348-8
Vasallo C, Colombo G, Canevari R, Brazzelli V, Ardigo M, Carrera C, et al. Monolateral severe eyelid erythema and edema as unique manifestation of lupus tumidus. Int J Dermatol 2005; 44: 858-60. https://doi.org/10.1111/j.1365-4632.2005.02210.x
Donzis PB, Insler MS, Buntin DM, et al. Discoid lupus erythematosus involving the eyelids. Am J Opthalmol 1984; 98(1): 32-36. https://doi.org/10.1016/0002-9394(84)90185-5
Giménez-García R, Sánchez-Ramón S, De Andrés A. Discoid lupus erythematosus involving the eyelids. J Eur Acad Dermatol Venereol 2005; 1: 138-9. https://doi.org/10.1111/j.1468-3083.2004.01076.x
Ghaninejad H, Kavusi S, Safar F, Asgari M, Naraghi ZS. Persistent periorbital edema as a sole manifestation of cutaneous lupus erythematosus: report of two cases. Dermatol Online J 2006; 12-14.
Ricotti C, Tozman E, Fernandez A, Nousari CH. Unilateral eyelid discoid lupus erythematosus. Am J Dermatopathol 2008; 512-3. https://doi.org/10.1097/DAD.0b013e318181593c
Yi-Fang W, Tseng-Tong K, Pei-Hsuan L, Ming-Jing C, Chih-Hsun Y. Cutaneous lupus erythematosus manifesting as unilateral eyelid erythema and swelling. Dermatologica Sinica 2012; 30: 25-28. https://doi.org/10.1016/j.dsi.2011.09.008
Galeone M, Arunachalam M, Scarfi F, Palleschi GM, Massi D, Difonzo EM. Unilateral eyelid involvement in discoid lupus erythematosus. Int J Dermatol 2014: 53: 894-6. https://doi.org/10.1111/j.1365-4632.2012.05730.x
Makeeva V, Seminario-Vidal L, Beckum K, Naveed S. Bilateral periorbital swelling as the initial presentation of cutaneous lupus erythematosus. J Am Acad Dermatol 2016; 2: 72-76. https://doi.org/10.1016/j.jdcr.2015.12.009
Darjani A, Rafiei R, mesbah A, et al. Discoid lupus erythematosus presenting as upper eyelid edema and erythema. Acta Med Iran 2017; 55(7): 474-476.
Chomiciene A, Stankeviciute R, Malinauskiene L, et al. Rare cause of periorbital and eyelids lesions: discoid lupus erythematosus misdiagnosed as allergy. Ann Allergy Asthma Immunol 2017; 119(6): 568-569. https://doi.org/10.1016/j.anai.2017.09.062
Wu MY, Wang CH, Ng CY, et al. Periorbital edema and swelling as a presenting sign of lupus erythematosus in tertiary referrals center and literature review. Lupus 2018; 27: 1828-1837. https://doi.org/10.1177/0961203318792358
Serarslan G, Atik E, Sarikaya G. Periorbital edema and erythema: An unusual localization of DLE in a patient with psoriasis. J Dermatol 2011; 38: 486-488. https://doi.org/10.1111/j.1346-8138.2010.01096.x
Wu YF, Kuo TT, Lu PH, Chen MJ, Yang CH. Cutaneous lupus erythematosus manifesting as unilateral eyelid erythema and swelling. Dermatologica Sinica 2012; 30: 25-28. https://doi.org/10.1016/j.dsi.2011.09.008
Silva LL, Romiti R, Nico MM. Persistent eyelid edema and erythema as manifestation of lupus erythematosus: A series of six cases. J Dtschn Dermatol Ges 2015; 13: 917-920. https://doi.org/10.1111/ddg.12632
Jefferson GD, Aakalu VK, Braniecki M. Tumid lupus: An unexpected diagnosis for the otolaryngologist. Am J Otolaryngol 2017; 38: 257-259. https://doi.org/10.1016/j.amjoto.2017.01.003
Al-Holou SN, Elner VM, Douglas RS, Joseph SS. Eyelid swelling and proptosis with systemic implications. Am J Med 2016; 129: e51-e53. https://doi.org/10.1016/j.amjmed.2016.01.027
Arrico L, Abbouda A, Abicca I, Malagola R. Ocular complications in cutaneous lupus erythematosus: A systematic review with a meta-analysis of reported cases. J Ophthalmol 2015; 2015: 254260. https://doi.org/10.1155/2015/254260
Yaghoobi R, Feily A, Behrooz B, Yaghoobi E, Mokhtarzadeh S. Palpebral involvement as a presenting and sole manifestation of discoid lupus erythematosus. Scientific World Journal 2010; 10: 2130-2131. https://doi.org/10.1100/tsw.2010.209
Cakici O, Karadag R, Bayramlar H, Ozkanli S, Uzuncakmak TK, Karadag AS. Periorbital discoid lupus: A rare localization in a patient with systemic lupus erythematosus. An Bras Dermatol 2016; 91(5 Suppl 1): 122-124. https://doi.org/10.1590/abd1806-4841.20164708
Vieira V, Del Pozo J, Yebra-Pimentel MT, Martínez W, Fonseca E. Lupus erythematosus tumidus: A series of 26 cases. Int J Dermatol 2006; 45: 512-17. https://doi.org/10.1111/j.1365-4632.2004.02574.x
Smith ES, Hallman JR, DeLuca AM, Goldenberg G, Jorizzo JL, Sangueza OP. Dermatomyositis: a clinicopathological study of 40 patients. Am J Dermatopathol 2009; 31: 61-67. https://doi.org/10.1097/DAD.0b013e31818520e1
Elston DM, Ferringer T. Dermatopathology. Philadelphia: Elsevier Limited, 2009.
Morales A, Sanchez J, Gonzalez-Chavez J, Vega J, Justiniano H. Periorbital mucinosis: A variant of cutaneous lupus erythematosus? J Am Acad Dermatol. 2010; 62: 667-71. https://doi.org/10.1016/j.jaad.2009.05.043
Braun RP, French LE, Massouye´ I, Saurat JH. Periorbital edema and erythema as a manifestation of discoid lupus erythematosus. Dermatology 2002; 205: 194-7. https://doi.org/10.1159/000063915