J of IMAB. 2019 Jan-Mar;25(1) https://www.journal-imab-bg.org 2443

Case report

LUPUS VASCULITIS

Desislava Tsvetanova1, Ivelina Yordanova1, Hristina Haidudova1, KlimentinaGospodinova1, Verka Pavlova1, Maya Alexieva1, Milen Karaivanov2, DimitarGospodinov1

1) Department of Dermatology, Venereology and Allergology, Faculty ofMedicine, Medical University-Pleven, Bulgaria2) Department of Pathoanatomy, Faculty of Medicine, Medical University-Pleven, Bulgaria.

Journal of IMAB - Annual Proceeding (Scientific Papers). 2019 Jan-Mar;25(1)Journal of IMABISSN: 1312-773Xhttps://www.journal-imab-bg.org

SUMMARY:Combination between Leucocytoclastic vasculitis

and Subacute cutaneous lupus erythematosus has beenonly rarely reported in the literature. Pathological cutane-ous lesions are presented by purpura, erythematous macules,urticarial, nodules and necroses.We present a 58-year-oldwoman with painful nodular lesions on the left thigh.Thedisease started in June 2015 with photosensitivity and pso-riasiform rashes on the back. Pathological cutaneouschanges affected lateral and dorsal surface of the left thigh.It was presented by painful, indurated in base, ulcerativeplaques, with unclear borders and necrotic surface. Therewere no deviations from complete blood count and bio-chemistry. Immunological investigations revealed elevatedlevels of MPO(pANCA) – 1.38 U/ml, antinuclear antibod-ies (ANA)- 134.1 U/ml, SS-A(Ro) antibodies – 233.7 U/mlCRP- 12.7mg/l. Urine analysis revealed positive proteinresults.Escherichia coli was isolated from microbiology test-ing. Histopathological result from ulcerative lesions fromthe left thigh of the patient revealed necrotizing leuko-cytoclastic vasculitis. The result from direct immunofluo-rescence microscopy of lesional skin corresponded to lu-pus erythematosus.The diagnosis lupus vasculitis wasmade. Systemic therapy with chloroquine phosphate, meth-ylprednisolone, methotrexate was administered. Topicaltreatment included proteolytic enzyme in dressings andvacuum therapy with good effect.

Keywords: Lupus vasculitis, antinuclear antibodies(ANA), SS-A(Ro) antibodies, vacuum therapy, chloroquinephosphate, methylprednisolone, methotrexate.

INTRODUCTION:Leukocytoclastic vasculitis associated with suba-

cute cutaneous lupus erythematosus (SCLE) has been de-scribed for the first time by Sontheimer. Purpura, erythema,urticaria, nodules and necrosis affect the skin of the trunk,palmoplantar areas and lower limbs. The histopathologi-cal changes are presented by leukocytoclastic vasculitis ofthe small blood vessels in the deep dermis. The prognosisof the disease is good [1].

MATERIALS AND METHODS:We present a 58-year-old female patient with pain-

ful nodular lesions affecting the area of the left thigh. Shereported about tension of the skin and photosensitivity.The patient suffers from arterial hypertension, hydroneph-rosis IV grade of the left kidney and carcinoma of uterinecervix. The patient was admitted at our department for thefirst time in June 2015 with normal status of cardiovascu-lar and pulmonary systems. Pathological cutaneouschanges affected the back of the trunk and were presentedby psoriasiform rashes. Cutaneous appendages and mucousmembranes were without pathological changes. Peripherallymph nodes were not pathologically enlarged. There wereno deviations from complete blood count and biochemis-try. Elevated levels of MPO(pANCA) – 1.38 U/ml, antinu-clear antibodies (ANA)- 134.1 U/ml (normal value up to 1U/ml), SS-A(Ro) antibodies – 233.7 U/ml (normal value upto 7.5 U/ml) and CRP- 12.7mg/l were found. Direct immun-ofluorescence (DIF) from lesional skin revealed granularband of IgG, IgM and C3 along the dermo-epidermal base-ment membrane. This result corresponds with Lupus ery-thematosus. Histopathological result from lesion on theback of the patient confirmed the diagnosis Lupus erythe-matosus. One month therapy with systemic metylpred-nisolonei.m. in tapering doses from 60 to 20 mg and chlo-roquine phosphate tablets 3x250 mg per day was conductedwithout satisfactory effect. Because of the worsening of thedisease and appearance of ulcerative lesions on the thighsthe patient was readmitted to our Department in July 2015(fig.1a, b). At the time of the second hospitalization thelaboratory results showed elevated levels of leucocytes(11.9x109/L), ASAT – 51.1 U/L, urea – 8.99 mmol/L, C-reactive protein- 12.7mg/l and positive protein result fromurine analysis. From microbiological examination, Es-cherichia coli was isolated. Serology tests for hepatic mark-ers for HBsAg (0.534), anti-HCV (0.037), Ebstein Bar vi-rus and Borrelia Burgdorferi were negative. Histopatho-logical result revealed pathologic alterations in small-sizeddermal vessels consisting of inflammatory infiltrate ofleukocytes, red blood cells and fibrin extravasation, neu-trophilic inflammation of the vessel wall, with fibrinoid

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necrosis and fragmented neutrophilic nuclei (nuclear “dust”) - histological pattern of necrotizing leukocytoclastic vas-culitis (fig. 2a, b, c).

Fig. 1. a, b. Ulcerative plaques with undermined borders and necrotic surface on the dorsal (a) and dorsal-medialsurface (b) of the left thigh.

Chest X-ray was without deviations. Ultrasound ofthe kidneys established hydronephrosis IV grade. Afterconsultation with cardiologist and echocardiography 10mmpericardial effusion and concentric left ventricular hyper-trophy were found. Based on the data from the medical his-tory, clinical picture results from the laboratory investiga-tions and the consultations,the diagnosis lupus vasculitiswas made. Systemic treatment with chloroquine phosphatetabl. 250mg 3 times daily, methylprednisolone in taperingdoses from 60 mg to 20 mg daily and methotrexate 15 mg.weekly p.o. was administrated for one month.Maintainancetherapy with chloroquine phosphate 250mg daily andMethotrexate 15 mg. weekly was conducted. Two-monthsvacuum therapy course was performed, and the result wascomplete epithelization of the lesions with atrophic scars(fig. 3a, b).

Fig. 2. a, b, c. Histological pattern of necrotizing leukocytoclastic vasculitis - fibrinoid necrosis and fragmentedneutrophilic nuclei (nuclear “dust”) with inflammation of the small-sized vessels in deep dermis. (a) H&Ex100, (b)H&Ex25, (c) H&Ex40

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Fig. 3. a, b. Postlesional atrophic scars on the dorsal-lateral (a) and dorsal surface (b) of the left thigh - after thetreatment

DISCUSSION:SCLE was first described by Sontheimer et al. in

1979 [2]. Gilliam and Sontheimercreated a classificationthat divided Lupus erythematosus into specific and non-specific forms. Specific forms include chronic, subacute andacute cutaneous lupus erythematosus. The non-specificforms comprise leukocytoclastic vasculitis, livedo race-mosa, thrombophlebitis, occlusive vasculopathy, Ray-naud’s syndrome, periungual telangiectasia, diffuse non-cicatricial alopecia, erythema multiforme, calcinosis cutisand papular mucinosis [3].

The combination of SCLE with histological vascu-lar changes is rarely observed [1]. The most common non-specific findings affect mainly small-sized blood vesselsin the dermis. The histopathological picture correspondsto leukocytoclastic vasculitis and is presented by perivas-cular neutrophilic infiltration, edema and fibrinoid necro-sis in the walls of the vessels. The same changes have beendescribed in the patient observed by us. According to theimmunological studies elevated ANA values have been re-ported in the majority of patients with SCLE. There are alsoincreased anti-Ro antibodies and elevated levels of anti-La antibodies [5, 6]. The immunological results of our pa-tient support the diagnosis SCLE. Purpura, erythema, urti-caria, nodules and necrosis on the trunk, palmoplantar ar-eas and lower extremities have been described in patientswith SCLE in combination with vasculitis. This clinicalpicture was observed in the case described by us.

Vascular lesions may precede SCLE, but more com-monly vasculitis develops in patients with prolonged SCLE[7]. According to a histopathological study by Cribier etal. that the degree of vascular necrosis and the depth ofvascular changes were not severe in patients with systemic

complications such as glomerulonephritis and gastrointe-stinal involvement [8].

The systemic antimalarial drugs such as hydro-xychloroquine, chloroquine and mepakine are the mostcommonly used. Hydroxychloroquine is best tolerated, andtherefore it is administered as first-line medication in thetreatment of SCLE. Its therapeutic effect could be seen 6weeks after the first dose [9].

Systemic corticosteroids and immunosuppressiveagents are also used in patients suffering from SCLE. Aza-thioprine and cyclophosphamide have been reported as ef-fective in the treatment of SCLE in small groups of patients.Thalidomide has been used in severe course of the disease.Improvement is fast, although there are frequent recidives.[10,11]. Vacuum therapy was reported as an effectivemethod for treatment of refractory for systemic therapy ul-cers [12].

CONCLUSION:We presented a clinical case of 58-year-old woman

diagnosed with lupus vasculitis, treated with chloroquinephosphate, methotrexate and vacuum therapy, with goodresult. There was complete epithelization of the ulcerativelesions with atrophic scars without new vascular changes.The patient is monitored regularly for systemic manifesta-tions of lupus erythematosus and is under observation bycardiologist, rheumatologist and ophthalmologist. Thereare no side effects from the systemic treatment so far.

Acknowledgement:This publication was funded by Project

BG05M2OP001-2.009-0031-C01.

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1. Sanchez-Perez J, Penas PF, Ríos-Buceta L, Fernandez-Herrera J, FragaJ, García-Díez A. Leukocytoclasticvasculitis in subacute cutaneous lupuserythematosus: clinicopathologicstudy of three cases and review of theliterature. Dermatology. 1996;193(3):230-5. [PubMed] [Crossref]

2. Sontheimer RD, Thomas JR,Gilliam JN. Subacute cutaneous lupuserythematosus: a cutaneous marker fora distinct lupus erythematosus subset.Arch Dermatol. 1979 Dec;115(12):1409-15. [PubMed]

3. Gronhagen CM, Nyberg F. Cuta-neous lupus erythematosus: An up-date. Indian Dermatol Online J. 2014Jan;5(1):7-13. [PubMed] [Crossref]

4. Berti S, Moretti S, Lucin C,Amato L, Massi D, Fabbri P.Urticarialvasculitis and subacute cutaneous lu-pus erythematosus. Lupus. 2005;14(6):489-92. [PubMed] [Crossref]

5. Sontheimer RD. Subacute cuta-

neous lupus erythematosus: a decade’sperspective. Med Clin North Am. 1989Sep;73(5):1073-90.

6. Watson RM, Talwar P, AlexanderE, Bias WB, Provost TT. Subacute cu-taneous lupus erythematosus-immuno-genetic associations. J Autoimmunity.1991; 4(1):73-85.

7. Fabbri P, Cardinali C, Giomi B,Caproni M.Cutaneous lupus erythema-tosus Diagnosis and management. AmJ Clin Dermatol. 2003; 4(7):449-65.[PubMed] [Crossref]

8. Cribier B, Couilliet D, Meyer P,Grosshans E. The severity of his-topathological changes of leukocy-toclastic vasculitis is not predictive ofextracutaneous involvement. Am JDermatopathol. 1999 Dec; 21(6):532-6. [PubMed]

9. Duna GF, Cash JM. Treatment ofrefractory cutaneous lupus erythema-tosus. Rheum Dis Clin North Am. 1995Feb;21(1):99-115. [PubMed]

REFERENCES:

Address for correspondence:Desislava TsvetanovaDepartment of Dermatology, venereology and allergology, Medical UniversistyPleven, Bulgaria91, Gen. Vladimir Vazov str., Pleven, BulgariaE-mail: desi_tzvetanova@abv.bg

10. Callen JP, Spencer LV, BurrussJB,Holtman J.Azathioprine an effec-tive, corticosteroid-sparing therapy forpatients with recalcitrant cutaneouslupus erythematosus or with recalci-trant cutaneous leukocytoclastic vas-culitis. Arch Dermatol.1991 Apr;127(4):515-22

11. Stevens RJ, Andujar C,Edwards CJ, Ames PR, Barwick AR,Khamashta MA, et al.Thalidomide inthe treatment of the cutaneous mani-festations of lupus erythematosus: ex-perience in sixteen consecutive pa-tients. Br J Rheumatol. 1997 Mar;36(3):353-59. [PubMed]

12. Zutt M, Haas E, Kruger U,Distler M, Neumann C. Successful useof vacuum-assisted closure therapy forleg ulcers caused by occluding vascu-lopathy and inflammatory vasculardiseases—a case series. Dermatology.2007; 214(4):319-24. [PubMed][Crossref]

Please cite this article as: Tsvetanova D, Yordanova I, Haidudova H, Gospodinova K, Pavlova V, Alexieva M, KaraivanovM, Gospodinov D. Lupus vasculitis. J of IMAB. 2019 Jan-Mar;25(1):2443-2446.DOI: https://doi.org/10.5272/jimab.2019251.2443

Received: 12/11/2018; Published online: 22/03/2019