https://doi.org/10.5272/jimab.2019251.2443 Journal of IMAB Journal of IMAB - Annual Proceeding (Scientific Papers). 2019 Jan-Mar;25(1) ISSN: 1312-773X https://www.journal-imab-bg.org Case report

LUPUS

Desislava Tsvetanova1, Ivelina Yordanova1, Hristina Haidudova1, Klimentina Gospodinova1, Verka Pavlova1, Maya Alexieva1, Milen Karaivanov2, Dimitar Gospodinov1 1) Department of , Venereology and Allergology, Faculty of Medicine, Medical University-Pleven, Bulgaria 2) Department of Pathoanatomy, Faculty of Medicine, Medical University- Pleven, Bulgaria.

SUMMARY: MATERIALS AND METHODS: Combination between Leucocytoclastic vasculitis We present a 58-year-old female patient with - and Subacute cutaneous erythematosus has been ful nodular lesions affecting the area of the left thigh. She only rarely reported in the literature. Pathological cutane- reported about tension of the skin and photosensitivity. ous lesions are presented by purpura, erythematous macules, The patient suffers from arterial hypertension, hydroneph- urticarial, nodules and necroses.We present a 58-year-old rosis IV grade of the left kidney and carcinoma of uterine woman with painful nodular lesions on the left thigh.The cervix. The patient was admitted at our department for the started in June 2015 with photosensitivity and pso- first time in June 2015 with normal status of cardiovascu- riasiform on the back. Pathological cutaneous lar and pulmonary systems. Pathological cutaneous changes affected lateral and dorsal surface of the left thigh. changes affected the back of the trunk and were presented It was presented by painful, indurated in base, ulcerative by psoriasiform rashes. Cutaneous appendages and mucous plaques, with unclear borders and necrotic surface. There membranes were without pathological changes. Peripheral were no deviations from and bio- lymph nodes were not pathologically enlarged. There were chemistry. Immunological investigations revealed elevated no deviations from complete blood count and biochemis- levels of MPO(pANCA) – 1.38 U/ml, antinuclear antibod- try. Elevated levels of MPO(pANCA) – 1.38 U/ml, antinu- ies (ANA)- 134.1 U/ml, SS-A(Ro) – 233.7 U/ml clear antibodies (ANA)- 134.1 U/ml (normal value up to 1 CRP- 12.7mg/l. Urine analysis revealed positive U/ml), SS-A(Ro) antibodies – 233.7 U/ml (normal value up results.Escherichia coli was isolated from microbiology test- to 7.5 U/ml) and CRP- 12.7mg/l were found. Direct immun- ing. Histopathological result from ulcerative lesions from ofluorescence (DIF) from lesional skin revealed granular the left thigh of the patient revealed necrotizing leuko- band of IgG, IgM and C3 along the dermo-epidermal base- cytoclastic vasculitis. The result from direct immunofluo- ment membrane. This result corresponds with Lupus ery- rescence microscopy of lesional skin corresponded to lu- thematosus. Histopathological result from lesion on the pus erythematosus.The diagnosis lupus vasculitis was back of the patient confirmed the diagnosis Lupus erythe- made. Systemic therapy with chloroquine phosphate, meth- matosus. One month therapy with systemic metylpred- ylprednisolone, was administered. Topical nisolonei.m. in tapering doses from 60 to 20 mg and chlo- treatment included proteolytic enzyme in dressings and roquine phosphate tablets 3x250 mg per day was conducted vacuum therapy with good effect. without satisfactory effect. Because of the worsening of the disease and appearance of ulcerative lesions on the thighs Keywords: Lupus vasculitis, antinuclear antibodies the patient was readmitted to our Department in July 2015 (ANA), SS-A(Ro) antibodies, vacuum therapy, chloroquine (fig.1a, b). At the time of the second hospitalization the phosphate, , methotrexate. laboratory results showed elevated levels of leucocytes (11.9x109/L), ASAT – 51.1 U/L, urea – 8.99 mmol/L, C- INTRODUCTION: reactive protein- 12.7mg/l and positive protein result from Leukocytoclastic vasculitis associated with suba- urine analysis. From microbiological examination, Es- cute cutaneous (SCLE) has been de- cherichia coli was isolated. tests for hepatic mark- scribed for the first time by Sontheimer. Purpura, , ers for HBsAg (0.534), anti-HCV (0.037), Ebstein Bar vi- urticaria, nodules and affect the skin of the trunk, rus and Borrelia Burgdorferi were negative. Histopatho- palmoplantar areas and lower limbs. The histopathologi- logical result revealed pathologic alterations in small-sized cal changes are presented by leukocytoclastic vasculitis of dermal vessels consisting of inflammatory infiltrate of the small blood vessels in the deep . The leukocytes, red blood cells and fibrin extravasation, neu- of the disease is good [1]. trophilic of the vessel wall, with fibrinoid

J of IMAB. 2019 Jan-Mar;25(1) https://www.journal-imab-bg.org 2443 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-medial surface (b) of the left thigh.

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

Chest X-ray was without deviations. Ultrasound of the kidneys established hydronephrosis IV grade. After consultation with cardiologist and echocardiography 10mm 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 vasculitis was made. Systemic treatment with chloroquine phosphate tabl. 250mg 3 times daily, methylprednisolone in tapering doses from 60 mg to 20 mg daily and methotrexate 15 mg. weekly p.o. was administrated for one month.Maintainance therapy with chloroquine phosphate 250mg daily and Methotrexate 15 mg. weekly was conducted. Two-months vacuum therapy course was performed, and the result was complete epithelization of the lesions with atrophic scars (fig. 3a, b).

2444 https://www.journal-imab-bg.org J of IMAB. 2019 Jan-Mar;25(1) Fig. 3. a, b. Postlesional atrophic scars on the dorsal-lateral (a) and dorsal surface (b) of the left thigh - after the treatment

DISCUSSION: complications such as and gastrointe- SCLE was first described by Sontheimer et al. in stinal involvement [8]. 1979 [2]. Gilliam and Sontheimercreated a classification The systemic antimalarial drugs such as hydro- that divided Lupus erythematosus into specific and non- xychloroquine, chloroquine and mepakine are the most specific forms. Specific forms include chronic, subacute and commonly used. is best tolerated, and acute cutaneous lupus erythematosus. The non-specific therefore it is administered as first-line in the forms comprise leukocytoclastic vasculitis, livedo race- treatment of SCLE. Its therapeutic effect could be seen 6 mosa, thrombophlebitis, occlusive vasculopathy, Ray- weeks after the first dose [9]. naud’s syndrome, periungual telangiectasia, diffuse non- Systemic and immunosuppressive cicatricial alopecia, , calcinosis cutis agents are also used in patients suffering from SCLE. Aza- and papular mucinosis [3]. thioprine and have been reported as ef- The combination of SCLE with histological vascu- fective in the treatment of SCLE in small groups of patients. lar changes is rarely observed [1]. The most common non- Thalidomide has been used in severe course of the disease. specific findings affect mainly small-sized blood vessels Improvement is fast, although there are frequent recidives. in the dermis. The histopathological picture corresponds [10,11]. Vacuum therapy was reported as an effective to leukocytoclastic vasculitis and is presented by perivas- method for treatment of refractory for systemic therapy ul- cular neutrophilic infiltration, and fibrinoid necro- cers [12]. sis in the walls of the vessels. The same changes have been described in the patient observed by us. According to the CONCLUSION: immunological studies elevated ANA values have been re- We presented a clinical case of 58-year-old woman ported in the majority of patients with SCLE. There are also diagnosed with lupus vasculitis, treated with chloroquine increased anti-Ro antibodies and elevated levels of anti- phosphate, methotrexate and vacuum therapy, with good La antibodies [5, 6]. The immunological results of our pa- result. There was complete epithelization of the ulcerative tient support the diagnosis SCLE. Purpura, erythema, urti- lesions with atrophic scars without new vascular changes. caria, nodules and necrosis on the trunk, palmoplantar ar- The patient is monitored regularly for systemic manifesta- eas and lower extremities have been described in patients tions of lupus erythematosus and is under observation by with SCLE in combination with vasculitis. This clinical cardiologist, rheumatologist and ophthalmologist. There picture was observed in the case described by us. are no side effects from the systemic treatment so far. Vascular lesions may precede SCLE, but more com- monly vasculitis develops in patients with prolonged SCLE Acknowledgement: [7]. According to a histopathological study by Cribier et This publication was funded by Project al. that the degree of vascular necrosis and the depth of BG05M2OP001-2.009-0031-C01. vascular changes were not severe in patients with systemic

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Please cite this article as: Tsvetanova D, Yordanova I, Haidudova H, Gospodinova K, Pavlova V, Alexieva M, Karaivanov M, 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

Address for correspondence: Desislava Tsvetanova Department of Dermatology, venereology and allergology, Medical Universisty Pleven, Bulgaria 91, Gen. Vladimir Vazov str., Pleven, Bulgaria E-mail: [email protected] 2446 https://www.journal-imab-bg.org J of IMAB. 2019 Jan-Mar;25(1)