Clinical and Experimental Dermatology最新文献

Background: The clinical, epidemiological and immunopathological profiles of atypical forms of pemphigus remain only partially known.

Objectives: To define the clinical, epidemiological and immunological characteristics, therapies and outcomes in patients with atypical pemphigus variants.

Methods: This was a 14-year multicentre retrospective observational study (VARIANT_P) on atypical variants of pemphigus across Italy. We collected demographic, immunopathological and clinical data, as well as information on comorbidities and prescribed treatments.

Results: We enrolled 61 patients [female/male sex ratio 1.77; 13 paraneoplastic pemphigus (PNP), 26 IgA pemphigus (PIgA), 22 pemphigus herpetiformis (PH)]. The median ages at onset and diagnosis were 70.6 (range 43.1-86.8) and 71.1 (range 46.9-86.9) for PNP; 62.2 (range 3.8-81.0) and 63.6 (range 4.0-82.4) for PIgA; and 49.4 (range 5.4-84.4) and 52.3 (range 5.9-85.9) for PH, respectively. The median diagnostic delay was 3.0 (range 0.0-45.6) months for PNP, 9.5 (range 1.0-140.0) months for PIgA and 2.0 (range 0-30.4) months for PH. The mortality rate was 55% (6/11) for PNP, 4% (1/26) for PIgA and 6% (1/17) for PH. Cutaneous involvement was present in all patients with PIgA and PH, and in 83% (10/12) of the patients with PNP. In contrast, oral mucosal involvement was observed in all patients with PNP with data (n = 12), but only in 8% (2/26) of those with PIgA and 21% (4/19) of those with PH. Histology, direct immunofluorescence, indirect immunofluorescence and enzyme-linked immunosorbent assay data demonstrated variable concordance with previously known data. Comorbidities included mainly solid malignancies for people with PNP, whereas cardiovascular and metabolic diseases were the most prevalent for those with PIgA and PH. Treatment mostly relied on systemic steroids and rituximab.

Conclusions: The VARIANT_P study contributes to data collection relating to atypical pemphigus variants in order to promote the development of specific therapeutical guidelines in the future.

Atopic dermatitis (AD) is a chronic, relapsing inflammatory skin disease with a big impact on quality of life. Despite major therapeutic advances, the involvement of difficult-to-treat areas such as the head and neck, hands and feet, and genital region remains a significant clinical challenge. Dupilumab, the first biologic approved for moderate-to-severe AD, has shown remarkable efficacy in clinical trials and real-world settings, but variable outcomes have been reported on sensitive areas of the body. This narrative review synthesizes evidence from real-world studies evaluating dupilumab in patients with AD affecting difficult-to-treat areas. Data indicate that dupilumab improves signs and symptoms for the majority of patients, with substantial benefits for quality of life and disease control. However, a subset of patients may experience suboptimal responses or paradoxical reactions, such as dupilumab-associated head and neck dermatitis (DAHND), often linked to Malassezia sensitization or alternative immune pathways. Similarly, although many studies confirm dupilumab effectiveness in chronic hand and foot eczema, treatments that have failed occur, sometimes requiring transition to Janus kinase inhibitors. Involvement of the genital area, a frequently underrecognized manifestation, generally responds well to dupilumab, although alternative biologics or small molecules may be needed in patients with resistant cases. Overall, real-world data highlight both the strengths and limitations of dupilumab in challenging anatomical sites, stressing the need for individualized therapeutic decisions. Awareness of variable responses, adverse event profiles and emerging alternatives is crucial for optimizing outcomes in patients with moderate-to-severe AD.

Infantile scabies can be challenging to treat because of behavioural and anatomical factors that reduce the efficacy of topical therapies. In this small prospective cohort study, there were 22 neonates and infants with persistent palmoplantar scabies. Patients were treated exclusively with permethrin 5% cream. All patients initially failed the standard regimen consisting of one full-body application, followed by a second -application after 7 days. In 1 group, 11 infants received an intensified regimen with 3 additional nights of targeted application to the hands and feet (intervention group), while in a second group, 11 continued with the standard regimen only (control group). Complete resolution was observed in all 11 patients in the intervention group, compared with none in the control group. Frequent infant behaviours, including spontaneous kicking, leg movements and habitual fist clenching, probably reduced drug contact time on the palms and soles. These findings indicate that standard topical regimens may be insufficient in this population and that targeted reapplication, combined with caregiver education, can optimize treatment outcomes in infants.