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A 28-year-old woman presents with fever and a diffuse rash. One year ago, the woman was at the hospital dermatology department with fever, malar rash, arthralgia, and hematuria. Her medical history documented recurrent painful oral ulcers and photosensitivity. The diagnostic workup at that time revealed systemic lupus erythematosus (SLE) with cutaneous and renal involvement. The patient’s status improved following treatment with prednisolone and cyclophosphamide.
On the current visit, physical examination reveals diffuse, tender, erythematous to violaceous punctate lesions on the limbs, trunk, face, fingertips, and palms. The lesions do not blanch with pressure. In addition, the patient has no malar rash, oral ulcers, alopecia, or arthritis and the remainder of the physical examination is unremarkable. Laboratory investigation reveal anemia, a positive Coombs test, and leukopenia.
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Systemic lupus erythematosus (SLE) is a heterogeneous autoimmune disease characterized by a wide spectrum of clinical and immunologic manifestations that may affect any organ.1 Vasculitis is common in patients with SLE with a reported range of 11% to 20% in this population.2
The presentation of SLE varies based on the organ involved and the size of the affected vessels (arteries, veins, or capillaries).2 Medium- and large-vessel vasculitis may present with visceral involvement and life-threatening manifestations.3 Manifestations of cutaneous vasculitis, which represent small vessel involvement, are the most common sign of SLE and may include malar rash, palpable purpura, punctate lesions, urticaria, and tender erythematous plaques or macules with or without necrosis, livedo reticularis, and superficial mucosal ulceration.2-4
Small-vessel vasculitis is a leukocytoclastic vasculitis and is usually limited to skin involvement.4 The condition is believed to be mediated by circulating immune complexes or by the direct effects of antibodies on cell surface components. Most patients present with erythematous or violaceous punctuate lesions on the fingertips and/or palms, which do not blanch on pressure.5
Histology reveals vasculitis of superficial and mid-dermal small blood vessels, erythrocyte extravasation, slight leukocytoclastic vasculitis, neutrophilic infiltration of vessel walls, and fibrinoid necrosis. It is often accompanied by thrombosis, with resultant cutaneous infarction.5,6
Discoid rash, Raynaud phenomenon, myositis, anemia, Coombs’ positivity and leukopenia are associated with the development of cutaneous vasculitis.3 Early diagnosis and adequate treatment are crucial to avoid complications.
Faten Rabhi, MD, Refka Frioui, MD, Kahena Jaber, MD, and Mohamed Raouf Dhaou, MD, are from the department of dermatology, Military Hospital of Tunis, Tunisia. Stephen Schleicher, MD, is director of the DermDox Center for Dermatology in Pennsylvania, as well as an associate professor of medicine at Commonwealth Medical College and a clinical instructor of dermatology at Arcadia University and Kings College.
References
1. Zhizho Y, Bo L, Jianso L, Fenlia G, Xun W, Zhihu Y. Clinical characteristics of cutaneous vasculitis secondary to systemic lupus erythematosus. Ped Rheumatol. 2008;6(Suppl 1):P239. doi:10.1186/1546-0096-6-S1-P239
2. Ramos-Casals M, Nardi N, Lagrutta M, et al. Vasculitis in systemic lupus erythematosus: prevalence and clinical characteristics in 670 patients. Medicine (Baltimore). 2006;85(2):95-104. doi:10.1097/01.md.0000216817.35937.70.
3. Kallas R, Goldman D, Petri MA. Cutaneous vasculitis in SLE. Lupus Sci Med. 2020;7(1):e000411. doi:10.1136/lupus-2020-000411
4. Uva L, Miguel D, Pinheiro C, Freitas JP, Marques Gomes M, Filipe P. Cutaneous manifestations of systemic lupus erythematosus. Autoimmune Dis. 2012;2012:834291. doi:10.1155/2012/834291
5. Barile-Fabris L, Hernandez-Cabrera MF, Barragan-Garfias JA. Vasculitis in systemic lupus erythematosus. Curr Rheumatol Rep. 2014;16(9):440. doi:10.1007/s11926-014-0440-9
6. Sanchez-Perez J, Penas PF, Rios-Buceta L, Fernandez-Henera J, Fraga J, Garcia-Diez A. Leukocytoclastic vasculitis in subacute cutaneous lupus erythematosus: clinicopathologic study of three cases and review of the literature. Dermatology. 1996;193(3):230-235. doi:10.1159/000246251
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