Acta Dermato-Venereologica 99-3CompleteContent | Page 22

335 SHORT COMMUNICATION Anetoderma and Systemic Lupus Erythematosus: Case Report and Literature Review Álvaro IGLESIAS-PUZAS 1 , Ana BATALLA 1 , Gonzalo PEÓN 1 , Carlos ÁLVAREZ 2 and Ángeles FLÓREZ 1 Dermatology Department, and 2 Pathology Department, Pontevedra University Hospital, EOXI Pontevedra-Salnés, Centro de Especialidades Mollavao, c/ Simón Bolívar s/n., ES-36001 Pontevedra, Spain. E-mail: [email protected] 1 Accepted Dec 6, 2018; E-published Dec 6, 2018 Anetoderma is a rare skin condition marked by circum­ scribed areas of slack skin due to the loss of dermal elastic fibres. Based on aetiology, anetoderma can be classified into 2 forms. Primary anetoderma (PA) develops in clini- cally normal skin (Schweninger-Buzzi type) or after a non-specific inflammatory process (Jadassohn-Pellizzari type), whereas secondary anetoderma arises from an ab- normal reparative mechanism of well-defined preceding skin diseases (1, 2). Anetoderma has been related to different immunolo- gical diseases, ranging from simple serological findings to well-defined pathologies (2). Although the connection may not be so much with systemic lupus erythematosus (SLE) as with the presence of antiphospholipid antibodies (APA) (3), patients with both conditions (anetoderma and SLE) in the absence of APA have been described (4, 5). Moreover, the pathophysiology, as well as a temporary relationship between APA, SLE development and onset of anetoderma, have not been well established. CASE REPORT A 43-year-old man presented with a 1-year history of gradually spreading, non-itching skin lesions that had appeared on his trunk and left arm. Regarding his previous medical history, he had been diagnosed with SLE 8 years previously due to the presence of erythema after sun exposure, discoid facial lesions, oral ulcers and consistent positive serology (antinuclear antibodies (ANA) >1/640 speckled pattern and anti-double-stranded antibodies (dsDNA) > 1/10). There was no history of vascular thrombosis, neurological disorders or heart valve defects. His condition had been treated with hydroxychloroquine for 3 years. Since he became asymptomatic, he had decided to drop out of the treatment and was lost to follow-up. Physical examination showed well-circumscribed, erythematous round plaques with overlying wrinkled skin and herniation phe- nomenon on his left arm and upper and lower back. These lesions had developed on previously healthy skin according to the patient’ medical records (Fig. 1). Laboratory studies demonstrated no abnormalities except per- sistent lymphopaenia (lymphocytes <1,200/mm 3 ). Immunological studies revealed a negative extractable nuclear antigen (ENA) panel, low serum levels of C3 (80 mg/dl, normal range 88–201 mg/dl) and negative APA. Serological tests, including for HIV, syphilis and Borrelia, were also negative. A skin biopsy from a lesion on his back showed superficial and deep perivascular lymphocytic infiltrate with no thrombi detected in dermal blood vessels. Verhoeff-van Gieson staining evidenced depletion and fragmentation of elastic fibres, which was more evident in the upper dermis (Fig. 2). Direct immunofluorescence (DIF) study detected granular IgG (+++), IgM (++) and C3 (+++) deposits in the basement membrane and blood vessels of involved skin. These findings confirmed the diagnosis of anetoderma in a patient with SLE. Fig. 1. Well-circumscribed, erythematous, round plaques with overlying wrinkled skin and herniation phenomenon on the upper and lower back. Based on the association of anetoderma with APA, the patient was tested for APA with a 6-month periodicity. One year later, positive results for lupus anticoagulant test (dilute Russell viper venom time (DRVVT) ratio >1.2) and beta-2-glycoprotein 1 anti- bodies (IgG > 46.7 IU, positive > 20 IU) were found. Regarding the absence of criteria for antiphospholipid syndrome, the patient was advised not to smoke or gain weight, and hydroxychloroquine was prescribed again. DISCUSSION Anetoderma is an elastolytic disorder characterized by lo- calized areas of wrinkled or flaccid skin due to a decrease in the amount of normal elastic tissue (5, 6). This condition has classically been connected with autoimmune diseases, such as SLE or lupus-like syndromes (2, 3). However, the Fig. 2. (a) Superficial and deep perivascular lymphocytic infiltrate with no thrombi in dermal blood vessels (haematoxylin and eosin ×40). (b) Verhoeff-van Gieson staining depletion and fragmentation of the elastic fibres, which was more evident in the upper dermis (Verhoeff-van Gieson staining ×40). This is an open access article under the CC BY-NC license. www.medicaljournals.se/acta Journal Compilation © 2019 Acta Dermato-Venereologica. doi: 10.2340/00015555-3100 Acta Derm Venereol 2019; 99: 335–336