Coxsackie B3 Virus-induced Acute Hemorrhagic Edema of Infancy

Acute hemorrhagic edema of infancy (AHEI) is a cutaneous leukocytoclastic small vessel vasculitis of unknown incidence. It affects mostly infants aged 4 to 24 months. The distinctive features of AHEI include a generally healthy-appearing child with low-grade or absent fever and rarely painful targetoid purpuric edematous lesions. The disease usually resolves spontaneously within 3 weeks without late sequelae. The main differential diagnosis of AHEI is Henoch-Schönlein purpura (HSP). Initially, purpura fulminans should also be ruled out. We report the case of a 5-year-old girl with low fever and rapidly progressive skin lesions who had been admitted to the pediatric clinic. The child presented with palpable annular targetoid and purpuric plaques of different size predominantly affecting the face and extremities. In addition, there was a painful, hemorrhagic edema on the dorsum of her hands and feet. Based on the course of the disease and the typical clinical presentation, i.e., extensive characteristic skin lesions in a young child in a good general health condition, a diagnosis of AHEI was established. A virus serology test showed increased titers of enterovirus and coxsackievirus. Isolation of virus from feces confirmed an infection with coxsackie B3 virus. To our knowledge, this is the first report linking coxsackie B3 virus infection to AHE

Coxsackie B3 Virus-induced Acute Hemorrhagic Edema of Infancy

Acute hemorrhagic edema of infancy (AHEI) is a cutaneous leukocytoclastic small vessel vasculitis of unknown incidence. It affects mostly infants aged 4 to 24 months. The distinctive features of AHEI include a generally healthy-appearing child with low-grade or absent fever and rarely painful targetoid purpuric edematous lesions. The disease usually resolves spontaneously within 3 weeks without late sequelae. The main differential diagnosis of AHEI is Henoch-Schönlein purpura (HSP). Initially, purpura fulminans should also be ruled out. We report the case of a 5-year-old girl with low fever and rapidly progressive skin lesions who had been admitted to the pediatric clinic. The child presented with palpable annular targetoid and purpuric plaques of different size predominantly affecting the face and extremities. In addition, there was a painful, hemorrhagic edema on the dorsum of her hands and feet. Based on the course of the disease and the typical clinical presentation, i.e., extensive characteristic skin lesions in a young child in a good general health condition, a diagnosis of AHEI was established. A virus serology test showed increased titers of enterovirus and coxsackievirus. Isolation of virus from feces confirmed an infection with coxsackie B3 virus. To our knowledge, this is the first report linking coxsackie B3 virus infection to AHE

Association of Clinical and Demographic Factors With the Severity of Palmoplantar Pustulosis.

Importance: Although palmoplantar pustulosis (PPP) can significantly impact quality of life, the factors underlying disease severity have not been studied. Objective: To examine the factors associated with PPP severity. Design, Setting, and Participants: An observational, cross-sectional study of 2 cohorts was conducted. A UK data set including 203 patients was obtained through the Anakinra in Pustular Psoriasis, Response in a Controlled Trial (2016-2019) and its sister research study Pustular Psoriasis, Elucidating Underlying Mechanisms (2016-2020). A Northern European cohort including 193 patients was independently ascertained by the European Rare and Severe Psoriasis Expert Network (2014-2017). Patients had been recruited in secondary or tertiary dermatology referral centers. All patients were of European descent. The PPP diagnosis was established by dermatologists, based on clinical examination and/or published consensus criteria. The present study was conducted from October 1, 2014, to March 15, 2020. Main Outcomes and Measures: Demographic characteristics, comorbidities, smoking status, Palmoplantar Pustulosis Psoriasis Area Severity Index (PPPASI), measuring severity from 0 (no sign of disease) to 72 (very severe disease), or Physician Global Assessment (PGA), measuring severity as 0 (clear), 1 (almost clear), 2 (mild), 3 (moderate), and 4 (severe). Results: Among the 203 UK patients (43 men [21%], 160 women [79%]; median age at onset, 48 [interquartile range (IQR), 38-59] years), the PPPASI was inversely correlated with age of onset (r = -0.18, P = .01). Similarly, in the 159 Northern European patients who were eligible for inclusion in this analysis (25 men [16%], 134 women [84%]; median age at onset, 45 [IQR, 34-53.3] years), the median age at onset was lower in individuals with a moderate to severe PGA score (41 years [IQR, 30.5-52 years]) compared with those with a clear to mild PGA score (46.5 years [IQR, 35-55 years]) (P = .04). In the UK sample, the median PPPASI score was higher in women (9.6 [IQR, 3.0-16.2]) vs men (4.0 [IQR, 1.0-11.7]) (P = .01). Likewise, moderate to severe PPP was more prevalent among Northern European women (57 of 134 [43%]) compared with men (5 of 25 [20%]) (P = .03). In the UK cohort, the median PPPASI score was increased in current smokers (10.7 [IQR, 4.2-17.5]) compared with former smokers (7 [IQR, 2.0-14.4]) and nonsmokers (2.2 [IQR, 1-6]) (P = .003). Comparable differences were observed in the Northern European data set, as the prevalence of moderate to severe PPP was higher in former and current smokers (51 of 130 [39%]) compared with nonsmokers (6 of 24 [25%]) (P = .14). Conclusions and Relevance: The findings of this study suggest that PPP severity is associated with early-onset disease, female sex, and smoking status. Thus, smoking cessation intervention might be beneficial

Treating axial spondyloarthritis and peripheral spondyloarthritis, especially psoriatic arthritis, to target: 2017 update of recommendations by an international task force

Therapeutic targets have been defined for axial and peripheral spondyloarthritis (SpA) in 2012, but the evidence for these recommendations was only of indirect nature. These recommendations were re-evaluated in light of new insights. Based on the results of a systematic literature review and expert opinion, a task force of rheumatologists, dermatologists, patients and a health professional developed an update of the 2012 recommendations. These underwent intensive discussions, on site voting and subsequent anonymous electronic voting on levels of agreement with each item. A set of 5 overarching principles and 11 recommendations were developed and voted on. Some items were present in the previous recommendations, while others were significantly changed or newly formulated. The 2017 task force arrived at a single set of recommendations for axial and peripheral SpA, including psoriatic arthritis (PsA). The most exhaustive discussions related to whether PsA should be assessed using unidimensional composite scores for its different domains or multidimensional scores that comprise multiple domains. This question was not resolved and constitutes an important research agenda. There was broad agreement, now better supported by data than in 2012, that remission/inactive disease and, alternatively, low/minimal disease activity are the principal targets for the treatment of PsA. As instruments to assess the patients on the path to the target, the Ankylosing Spondylitis Disease Activity Score (ASDAS) for axial SpA and the Disease Activity index for PSoriatic Arthritis (DAPSA) and Minimal Disease Activity (MDA) for PsA were recommended, although not supported by all. Shared decision-making between the clinician and the patient was seen as pivotal to the process. The task force defined the treatment target for SpA as remission or low disease activity and developed a large research agenda to further advance the field

Clinical Case Reports / De novo mutation of emopamil binding protein (EBP ) gene in a girl with ConradiHünermannHapple syndrome

ConradiHünermannHapple syndrome is a rare Xlinked dominant syndrome affecting the skin, skeletal system, and eyes. Here, we report on a female patient with a de novo heterozygous missense mutation c.301C>T (p.Trp101Arg) of the EMP (emopamil binding protein) gene.(VLID)510155

Acta Dermato-Venereologica / Anogenital Human Papillomavirus Prevalence is Unaffected by Therapeutic Tumour Necrosis Factor-alpha Inhibition

Patients receiving tumour necrosis factor alpha (TNF-) inhibitors are at increased risk of exacerbation of (myco-)bacterial and some viral infections. However, in- formation on anogenital human papillomavirus (HPV) infection in these patients is sparse or conflicting. In this study 222 patients with psoriasis or inflammatory bowel disease (IBD), who received either anti-TNF- inhibitors or alternatives (purine-, folic acid analogues, photothe- rapy, fumaric ester, mesalazine) continuously for at least 6 months, were evaluated for the presence of anogeni- tal HPV-induced lesions, mucosal HPV DNA, and sero- logical status of mucosal low-risk HPV6 and high-risk HPV16/HPV18. Hallmarks of anogenital HPV infection were more frequently detected in patients with psoriasis than in those with IBD. HPV-induced lesions, viral DNA, and seroprevalence were not elevated in participants with psoriasis or IBD, who received TNF- inhibitors for a mean duration of 31.4 months (range 696 months) compared with recipients of alternative or no treatment. TNF- blockade for a mean period of 31.4 months does not increase detectable anogenital HPV infection or dis- ease.(VLID)330858