Bowel-associated dermatosis-arthritis syndrome, a diagnostic challenge in a young child with very early-onset inflammatory bowel disease.

A 14-month-old girl with very early-onset inflammatory bowel disease (VEO-IBD) was admitted with a flare of her bowel disease and subsequently developed high fevers, joint pain, and skin lesions during her hospitalization. Workup demonstrated bowel-associated dermatosis-arthritis syndrome in the setting of VEO-IBD, a neutrophilic dermatosis rarely reported in children that can be challenging to diagnose and treat, with limited literature for patients under 2 years of age.

Effectiveness and Safety of Dupilumab in Children Under 6 Years of Age with Moderate-to-Severe Atopic Dermatitis: A Retrospective Real-World Study.

INTRODUCTION: Dupilumab has recently been shown to be effective in children under 6 years of age with atopic dermatitis (AD). Nevertheless, real-life and long-term follow-up data are scarce. We aimed to assess the effectiveness, safety, and long-term outcomes of dupilumab in a daily-practice setting in this age group. METHOD: This was a retrospective observational cohort study. Only patients with 16 or more weeks of treatment were included in the analysis. The proportion of patients who achieved ≥75% or 90% reduction from baseline EASI (EASI75 and EASI90, respectively) and the percentage of patients who achieved vIGA 0-1 were analyzed at 4, 16, 48, 72, and 96 weeks (when available). Adverse events were recorded during follow-up. RESULTS: A total of 19 patients <6 years old with moderate-to-severe AD were included in the cohort, with a median age of 4.7 years (range: 2.6-5.9). The median weeks on dupilumab were 51.3 (IQR: 24.6-79.3). EASI75 was achieved in 11/19 patients (57.9%) at w4, 16/19 (84.2%) at w16, 9/12 (75%) at w48, 6/6 (100%) at w72, and in 2/2 (100%) at week 96. The objective of vIGA 0-1 was reached by 10/19 patients (51.6%) at w4, by 14/19 (73.7%) at w16, by 9/12 (75%) at w48, by 5/6 (83.3%) at w72, and by 2/2 (100%) at w96. Dupilumab was discontinued in 3 patients (15.8%) due to loss of response. One patient developed a paradoxical palmo-plantar eruption. We found no cases of conjunctivitis, facial erythema, or injection-site reactions related to dupilumab. CONCLUSIONS: Dupilumab was effective and safe in our cohort of patients with moderate-to-severe AD under 6 years of age. Response was maintained in the long term in most patients with longer follow-up. Its adverse effect profile was similar to that found in older children and adults.

Monkeypox outbreak in Spain: clinical and epidemiological findings in a prospective cross-sectional study of 185 cases.

BACKGROUND: Since May 2022, a new outbreak of monkeypox has been reported in several countries, including Spain. The clinical and epidemiological characteristics of the cases in this outbreak may differ from those in earlier reports. OBJECTIVES: To document the clinical and epidemiological characteristics of cases of monkeypox in the current outbreak. METHODS: We conducted a prospective cross-sectional study in multiple medical facilities in Spain to describe the cases of monkeypox in the 2022 outbreak. RESULTS: In total, 185 patients were included. Most cases started with primarily localized homogeneous papules, not pustules, in the probable area of inoculation, which could be cutaneous or mucous, including single lesions. Generalized small pustules appeared later in some of them. Heterogeneous lesions occurred during this generalized phase. All patients had systemic symptoms. Less common lesions included mucosal ulcers (including pharyngeal ulcers and proctitis) and monkeypox whitlows. Four patients were hospitalized, none died. Smallpox vaccination and well-controlled HIV disease were not associated with markers of severity. Contact during sex is the most likely mechanism of transmission. In this outbreak, cases have been described in men who have sex with men and are strongly associated with high-risk sexual behaviours. Seventy-six per cent of the patients had other sexually transmitted diseases upon screening. CONCLUSIONS: The clinical findings in this outbreak differ from previous findings and highly suggest contact transmission and initiation at the entry site. The characterization of the epidemiology of this outbreak has implications for control. What is already known about this topic? Monkeypox eruption is described as consisting of pustules. The roles of HIV and previous smallpox vaccination in the prognosis are unknown. The transmission route was initially described as respiratory droplets and was later suggested to be via sexual contact. What does this study add? Initial lesions at the probable inoculation area were homogeneous and papular (pseudopustules). Generalized small pustules appeared later in some of them. Heterogeneous lesions occurred during this generalized phase. All patients had systemic symptoms. Less common signs included mucosal ulcers (including pharyngeal ulcers and proctitis) and monkeypox whitlows. Well-controlled HIV and previous smallpox vaccination were not associated with severity. No patient died. The data support the hypothesis of transmission via contact during sex. Although this might change, the outbreak is currently limited mostly to men who have sex with men, with high-risk factors for sexually transmitted diseases.