Long-Term Efficacy of Treatment with Intravenous Immunoglobulin in Scleromyxedema

Scleromyxedema or generalized lichen myxedematosus is a rare depositional disorder. Diagnostic criteria encompass a generalized papular and sclerodermoid eruption, monoclonal gammopathy (paraproteinemia), most often with G-lambda type immunoglobulin, a characteristic microscopic triad (mucin deposition, fibroblast proliferation, fibrosis), and absence of thyroid disease. Many internal manifestations of scleromyxedema have been described to date, leading to high mortality and morbidity. Because the disease is rare, the etiology is not fully understood and there is a lack of well-designed studies, so no optimal treatment exists so far. This paper reports the follow-up on a patient in 5.5-year remission after successful intravenous immunoglobulin therapy 10.5 years since initial diagnosis

Long-Term Efficacy of Treatment with Intravenous Immunoglobulin in Scleromyxedema

Scleromyxedema or generalized lichen myxedematosus is a rare depositional disorder. Diagnostic criteria encompass a generalized papular and sclerodermoid eruption, monoclonal gammopathy (paraproteinemia), most often with G-lambda type immunoglobulin, a characteristic microscopic triad (mucin deposition, fibroblast proliferation, fibrosis), and absence of thyroid disease. Many internal manifestations of scleromyxedema have been described to date, leading to high mortality and morbidity. Because the disease is rare, the etiology is not fully understood and there is a lack of well-designed studies, so no optimal treatment exists so far. This paper reports the follow-up on a patient in 5.5-year remission after successful intravenous immunoglobulin therapy 10.5 years since initial diagnosis

Clinical and Demographic Characteristics of Patients with Molluscum Contagiosum Treated at the University Dermatology Clinic Maribor in a 5-year period

Molluscum contagiosum virus (MCV) is a common skin pathogen in both adults and children. In this prospective study, we clinically evaluated consecutive patients with molluscum contagiosum (MC) who had been examined during a 5-year period at the second-largest dermatology clinic in Slovenia and described their main demographic and clinical characteristics, concomitant diseases, and treatment success. The study included 188 patients, of which 121 (64%) were men and 67 (36%) were women. A total of 135 (72%) patients were adults, with lesions that were most commonly located in the anogenital region (98%) and were probably sexually acquired. Two adult patients were diagnosed with concurrent human immunodeficiency virus (HIV) infection. Fifty-three (28%) patients were children with a mean age of 5.7 years, most commonly presenting with lesions on the torso and extremities (85%). In adults, the infection most commonly occurred in male patients, while in children it was slightly more common in female patients. At presentation, 58% of patients had more than 5 MC lesions. A total of 30% of the included children had concomitant atopic dermatitis. We did not observe an increased occurrence of MCV infection in patients with atopic dermatitis. All patients were treated with curettage of the lesions. The cure rate at the first follow-up visit after 2 months was relatively high (63%), and recurrences were not associated with the number or site of lesions at presentation or with concomitant atopic dermatitis. </p

Ulcus vulvae acutum

A 29-year old female presented with a history of sudden onset of genital ulcers with fever and malaise. The clinical picture and course of the disease corresponded to the diagnosis of acute vulvar ulcer. No relevant etiologic factors could be ascertained. Treatment with doxycycline and systemic corticosteroids led to the resolution of the ulcerations after three weeks. Although the disease is rare, it is important to include it in the differential diagnosis of genital ulcers

Idiopathic eruptive macular pigmentation

We presented the case of a 10-year-old girl with a six months history of disseminated asymptomatic, brown pigmented macules on the trunk and proximal parts of the extremities. The clinical picture, histological findings, and the course of disease were similar to those of idiopathic eruptive macular pigmentation. The cutaneous lesions gradually disappeared over the next two years without any treatment, and no relapse occurred. The knowledge of this disease is important in order to avoid unnecessary treatment as spontaneous resolution of the lesions may be expected within months or a few years. The spontaneous regression without any treatment is an additional diagnostic criterion

New insights into the evolutionary and genomic landscape of molluscum contagiosum virus (MCV) based on nine MCV1 and six MCV2 complete genome sequences

Molluscum contagiosum virus (MCV) is the sole member of the Molluscipoxvirus genus and the causative agent of molluscum contagiosum (MC), a common skin disease. Although it is an important and frequent human pathogen, its genetic landscape and evolutionary history remain largely unknown. In this study, ten novel complete MCV genome sequences of the two most common MCV genotypes were determined (five MCV1 and five MCV2 sequences) and analyzed together with all MCV complete genomes previously deposited in freely accessible sequence repositories (four MCV1 and a single MCV2). In comparison to MCV1, a higher degree of nucleotide sequence conservation was observed among MCV2 genomes. Large-scale recombination events were identified in two newly assembled MCV1 genomes and one MCV2 genome. One recombination event was located in a newly identified recombinant region of the viral genome, and all previously described recombinant regions were re-identified in at least one novel MCV genome. MCV genes comprising the identified recombinant segments have been previously associated with viral interference with host T-cell and NK-cell immune responses. In conclusion, the two most common MCV genotypes emerged along divergent evolutionary pathways from a common ancestor, and the differences in the heterogeneity of MCV1 and MCV2 populations may be attributed to the strictness of the constraints imposed by the host immune response

New Insights into the Evolutionary and Genomic Landscape of Molluscum Contagiosum Virus (MCV) based on Nine MCV1 and Six MCV2 Complete Genome Sequences

Molluscum contagiosum virus (MCV) is the sole member of the Molluscipoxvirus genus and the causative agent of molluscum contagiosum (MC), a common skin disease. Although it is an important and frequent human pathogen, its genetic landscape and evolutionary history remain largely unknown. In this study, ten novel complete MCV genome sequences of the two most common MCV genotypes were determined (five MCV1 and five MCV2 sequences) and analyzed together with all MCV complete genomes previously deposited in freely accessible sequence repositories (four MCV1 and a single MCV2). In comparison to MCV1, a higher degree of nucleotide sequence conservation was observed among MCV2 genomes. Large-scale recombination events were identified in two newly assembled MCV1 genomes and one MCV2 genome. One recombination event was located in a newly identified recombinant region of the viral genome, and all previously described recombinant regions were re-identified in at least one novel MCV genome. MCV genes comprising the identified recombinant segments have been previously associated with viral interference with host T-cell and NK-cell immune responses. In conclusion, the two most common MCV genotypes emerged along divergent evolutionary pathways from a common ancestor, and the differences in the heterogeneity of MCV1 and MCV2 populations may be attributed to the strictness of the constraints imposed by the host immune response

Apremilast Use in Severe Psoriasis: Real-World Data from Central and Eastern Europe

Introduction: The broad and sustained efficacy of apremilast for psoriasis has been demonstrated in randomized and real-world observational studies. Data from Central and Eastern Europe (CEE) are lacking. Moreover, apremilast use in this region is limited by country-specific reimbursement criteria. This is the first study to report data on the real-world use of apremilast in the region. Methods: APPRECIATE (NCT02740218) was an observational, retrospective, cross-sectional study assessing psoriasis patients 6 (± 1) months after apremilast treatment initiation. The study aimed to describe the characteristics of patients with psoriasis receiving apremilast, estimate treatment outcomes, including Psoriasis Area Severity Index (PASI), Body Surface Area (BSA), and Dermatology Life Quality Index (DLQI), and assess dermatologists' and patients' perspectives on treatment using questionnaires including the Patient Benefit Index (PBI). Adverse event reports were taken from the medical records. Results: Fifty patients (Croatia: 25; Czech Republic: 20; Slovenia: 5) were enrolled. In patients continuing apremilast at 6 (± 1) months, mean (± SD) PASI score was reduced from 16.2 ± 8.7 points at treatment initiation to 3.1 ± 5.2 at 6 (± 1) months; BSA from 11.9% ± 10.3% to 0.8% ± 0.9%; DLQI from 13.7 ± 7.4 points to 1.6 ± 3.2. PASI 75 was reached by 81% of patients. Physicians reported that the overall treatment success fulfilled their expectations in more than two thirds of patients (68%). At least three-quarters of patients reported apremilast had a quite or very high benefit on the needs they identified as being most important. Apremilast was well tolerated; no serious or fatal adverse events were identified. Conclusion: Apremilast was effective in reducing skin involvement and improving quality of life in CEE patients having severe disease. Treatment satisfaction among physicians and patients was very high. These data add to the growing body of evidence showing consistent effectiveness of apremilast across the continuum of psoriasis disease severity and manifestations

International eDelphi Study to Reach Consensus on the Methotrexate Dosing Regimen in Patients with Psoriasis

Importance: A clear dosing regimen for methotrexate in psoriasis is lacking, and this might lead to a suboptimal treatment. Because methotrexate is affordable and globally available, a uniform dosing regimen could potentially optimize the treatment of patients with psoriasis worldwide. Objective: To reach international consensus among psoriasis experts on a uniform dosing regimen for treatment with methotrexate in adult and pediatric patients with psoriasis and identify potential future research topics. Design, Setting, and Participants: Between September 2020 and March 2021, a survey study with a modified eDelphi procedure that was developed and distributed by the Amsterdam University Medical Center and completed by 180 participants worldwide (55 [30.6%] resided in non-Western countries) was conducted in 3 rounds. The proposals on which no consensus was reached were discussed in a conference meeting (June 2021). Participants voted on 21 proposals with a 9-point scale (1-3 disagree, 4-6 neither agree nor disagree, 7-9 agree) and were recruited through the Skin Inflammation and Psoriasis International Network and European Academy of Dermatology and Venereology in June 2020. Apart from being a dermatologist/dermatology resident, there were no specific criteria for participation in the survey. The participants worked mainly at a university hospital (97 [53.9%]) and were experienced in treating patients with psoriasis with methotrexate (163 [91.6%] had more than 10 years of experience). Main Outcomes and Measures: In a survey with eDelphi procedure, we tried to reach consensus on 21 proposals. Consensus was defined as less than 15% voting disagree (1-3). For the consensus meeting, consensus was defined as less than 30% voting disagree. Results: Of 251 participants, 180 (71.7%) completed all 3 survey rounds, and 58 participants (23.1%) joined the conference meeting. Consensus was achieved on 11 proposals in round 1, 3 proposals in round 2, and 2 proposals in round 3. In the consensus meeting, consensus was achieved on 4 proposals. More research is needed, especially for the proposals on folic acid and the dosing of methotrexate for treating subpopulations such as children and vulnerable patients. Conclusions and Relevance: In this eDelphi consensus study, consensus was reached on 20 of 21 proposals involving methotrexate dosing in patients with psoriasis. This consensus may potentially be used to harmonize the treatment with methotrexate in patients with psoriasis.