Successful Treatment with Bosentan of Lower Extremity Ulcers in a Scleroderma Patient

Digital ulcers are a well-known problem in patients with systemic sclerosis. Lower extremity ulcers are less prevalent but are also a challenging and underestimated complication of the disease causing important pain and morbidity. Bosentan, an oral dual endothelin receptor antagonist, has been shown to be effective in preventing digital ulcers in patients with systemic sclerosis. A few recent observations showed the efficacy of bosentan for accelerating the healing of nondigital ulcers in scleroderma patients. This report deals with a 48-year-old patient with systemic sclerosis who developed painful ulcers on the left ankle and hallux. The ulcers were refractory to a combination of vasodilator therapy with a calcium antagonist and several courses of intravenous prostanoids, low molecular weight heparin, aspirin, simvastatin, and intensive local treatment. Bosentan treatment showed spectacular healing of the ulcers already after 4 months of therapy. This case supports the previous few observations of accelerating wound healing of lower extremity ulcers in systemic sclerosis patients with bosentan treatment

Patients Perceptions on Teledermatology Through Video-consulting During the Covid19-pandemic: Report on a Belgian Cohort

Objectives: In light of the Covid19-pandemic the Belgian government announced a quarantine period. Following the imposed guidelines, UZ Leuven responded swiftly by canceling all non-urgent ambulant consultations for a period of three weeks. Later, this was prolonged to a period of three months. This setting urged implementation of telemedicine. In this pilot study, we invited patients followed in our department for inflammatory skin diseases (psoriasis and atopic dermatitis) to substitute their cancelled appointment by a video-consult. Preparation of the consultation as well as the video-consultation itself were assessed by the patient through an online questionnaire. Material and methods:  A live-interaction teledermatology service was offered by email to patients in follow-up for inflammatory skin diseases who had an appointment scheduled that was cancelled because of the Covid19-pandemic. Patients who accepted the invitation received instructions on how to prepare for the video-consultation. After the video-consultation they received a link to an anonymous online questionnaire. Results: In total, 100 patients received an invitation by email to replace the cancelled appointment by a video-consultation. 42 patients agreed to participate in video-consultation. The questionnaire was completed by 30 patients. Overall, patients did not report technical difficulties before or during the video-consultation. Satisfaction with the video-consult was high. Most of the participants were inclined to accept another invitation in the future. Conclusion: The Covid19 pandemic prompted rapid implementation of video-consultation in the UZ Leuven dermatology department. It was assessed as an acceptable replacement given the circumstances. Most patients estimated the visual support an added value and were open to repeat video-consulting in the future

Huidaandoeningen bij systeemziekten

Skin manifestations are frequent in systemic connective tissue diseases. These skin lesions are often chronic and disfiguring and can profoundly affect the quality of life of the patient. For the health care workers concerned, these skin manifestations can constitute an important clue to the diagnosis. Indeed, skin lesions are immediately visible and biopsies can easily be taken. The clinical pictures and pathological features can be very characteristic and thus diagnostically very valuable. This review describes and illustrates the most important skin manifestations observed in lupus erythematosus, dermatomyositis and scleroderma. In addition, a practical diagnostic approach in case of a suspected cutaneous vasculitis is proposed.status: publishe

Thalidomide: Still an important second-line treatment in refractory cutaneous lupus erythematosus?

BACKGROUND: Cutaneous lupus erythematosus (CLE) can be a severe disease, characterized by extensive, disfiguring lesions and a relapsing course. Thalidomide is known as an effective treatment for CLE, however, its use is restricted by its potential side-effects. Nevertheless, it remains a valuable option to consider. Therefore, it is important to report new clinical experiences. METHODS: The data of 30 patients with refractory CLE, who were treated with thalidomide, were retrospectively analyzed. The response rate was categorized as complete, partial or no response. The relapse rate and the occurrence of side-effects were registered. RESULTS: Six patients prematurely discontinued treatment because of side-effects. The response rate was 100% in the remaining 24 patients, including 20 patients (83%) with complete response and 4 (27%) with partial response. Clinical relapse was frequent (73%) and occurred between 3 and 24 weeks after withdrawal of thalidomide. Nine patients (30%) developed peripheral neuropathy. In the majority, there was no complete resolution of the neuropathy-associated symptoms after stopping thalidomide. One patient developed a thrombosis in an artery stent. CONCLUSION: Because of high risk of polyneuropathy, low-dose thalidomide should be used and long-term therapy should be avoided. Therefore, it should be recommended to combine thalidomide with other treatments for CLE.peerreview_statement: The publishing and review policy for this title is described in its Aims & Scope. aims_and_scope_url: http://www.tandfonline.com/action/journalInformation?show=aimsScope&journalCode=ijdt20status: publishe

Sodium thiosulfate as a treatment for calciphylaxis: A case series

BACKGROUND: Calciphylaxis is a rare disorder predominantly seen in patients with chronic kidney disease. Calcification of cutaneous vessels leads to extremely painful plaques and ulcerations. Mortality is high, sepsis being the leading cause of death. There is no standardized treatment. In the last years, administration of sodium thiosulfate (STS) has yielded some promising results. METHODS: We retrospectively collected data of eight calciphylaxis patients treated with STS at the University Hospital of Leuven between June 2009 and June 2014. RESULTS: Eight patients, of whom two without related renal risk factors, received STS treatment in different dosing schemes. Four patients (50%) achieved complete healing of skin lesions; two patients had only stabilization of the disease while experiencing pain relief. In the two remaining patients who had further progression of the disease, STS treatment led to analgesia in one patient. Overall, seven patients (87.5%) had some benefit from STS treatment. However, four patients (50%) eventually died due to causes related to calciphylaxis. STS was generally well tolerated, although almost all patients (87.5%) had metabolic acidosis. CONCLUSIONS: Treatment of calciphylaxis remains challenging. Although STS therapy, together with other treatment modalities, seems to improve disease outcome, more studies focusing on optimal dosing schemes and duration of treatment are necessary.peerreview_statement: The publishing and review policy for this title is described in its Aims & Scope. aims_and_scope_url: http://www.tandfonline.com/action/journalInformation?show=aimsScope&journalCode=ijdt20status: publishe

Myositis-specific autoantibodies: an important diagnostic tool in amyopathic dermatomyositis

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IgG4-related skin lesions: case report and review of the literature

IgG4-related disease (IgG4-RD) is a systemic disease, mostly affecting the pancreas. It presents as accumulation of IgG4-producing plasma cells in various tissues. Other possible affected organs include the lacrimal glands, salivary glands, lungs, kidneys, liver, bile duct, retroperitoneum, breast, aorta, pituitary gland and prostate. A cutaneous presentation has also been described in the literature, and might be the initial presenting feature of IgG4-RD. We describe a 73-year-old white man who presented with two infiltrated, erythematous nodules on his abdomen. The histopathological characteristics were highly suggestive of IgG4-related cutaneous disease. Immunohistochemical stains were positive for IgG4. In the past, the patient's other organs had also been affected by IgG4-RD. Cutaneous presentation of IgG4-RD has been described previously in the literature but only in Asian patients (both East and South Asian). We also provide an overview of previously reported cutaneous manifestations of IgG4-RD.status: publishe

Detection of circulating anti-skin antibodies by indirect immunofluorescence and by ELISA: a comparative systematic review and meta-analysis

Background Both enzyme-linked immunosorbent assays (ELISAs) and indirect immunofluorescence (IIF) are available for the diagnosis of autoimmune bullous diseases (AIBD). Many studies have reported on the performance of ELISAs and concluded that ELISAs could replace IIF. This study compares the diagnostic accuracy of ELISA and IIF for the detection of autoantibodies to desmoglein 1 (DSG1), desmoglein 3 (DSG3), bullous pemphigoid antigen 2 (BP180) and bullous pemphigoid antigen 1 (BP230) to support the diagnosis of pemphigus vulgaris (PV), pemphigus foliaceus (PF) and bullous pemphigoid (BP). Methods A literature search was performed in the PubMed database. The meta-analysis was performed using summary values and a bivariate random effect model. Results The five included studies on PV did not demonstrate significant differences between IIF and DSG3-ELISA (sensitivity 82.3% vs. 81.6%, p = 0.9284; specificity 95.6% vs. 93.9%, p = 0.5318; diagnostic odds ratio [DOR] 101.60 vs. 67.760, p = 0.6206). The three included studies on PF did not demonstrate significant differences between IIF and DSG1-ELISA (sensitivity 80.6% vs. 83.1%, p = 0.8501; specificity 97.5% vs. 93.9%, p = 0.3614; DOR 160.72 vs. 75.615, p = 0.5381). The eight included studies on BP showed that BP230-ELISA differed significantly from both IIF on monkey esophagus (MO) and BP180-ELISA with regard to DOR (11.384 vs. 68.349, p = 0.0008; 11.384 vs. 41.699, p = 0.0125, respectively) Conclusions Our meta-analysis shows that ELISA performs as well as IIF for diagnosing PV, PF and BP.status: publishe

Dermal fibroblasts pretreated with a sterol Delta7-reductase inhibitor produce 25-hydroxyvitamin D3 upon UVB irradiation

As dermis is a physiological site of vitamin D3 photoproduction, the photo-endocrine vitamin D3 system was studied in dermal fibroblasts. Dermal fibroblasts contain the vitamin D receptor and induce 1alpha,25-dihydroxyvitamin D3-24-hydroxylase [CYP24] mRNA upon stimulation with 1,25-dihydroxyvitamin D3 [1,25(OH)2D3]. In addition, dermal fibroblasts contain mRNA of the vitamin D3-25-hydroxylases (CYP2R1 and CYP27A1). However, we could not detect any 25-hydroxyvitamin D3 [25OHD3]-1alpha-hydroxylase mRNA in dermal fibroblasts and no CYP24 mRNA was induced upon ultraviolet [UVB] irradiation, even when endogenous 7-dehydrocholesterol content was elevated by pretreatment with the sterol Delta7-reductase inhibitor BM15766. Nevertheless, dermal fibroblasts produce inactive vitamin D3 metabolites that can be activated by epidermal keratinocytes as CYP24 mRNA is induced in epidermal keratinocytes but not in dermal fibroblasts after transfer of medium or cellular suspensions from BM15766-pretreated, UVB-irradiated fibroblasts. This CYP24 induction was UVB-dose dependent and was inhibited by ketoconazole. As revealed in a competitive binding assay, BM15766-pretreated dermal fibroblasts are able to produce 25OHD3 upon UVB irradiation, but no 1,25(OH)2D3 was detected via combined high-performance liquid chromatography radioimmunoassay. The physiological relevance of dermal vitamin D3 photoproduction and its subsequent conversion into 25OHD3 remains elusive.status: publishe