Association between maternal dupilumab exposure and pregnancy outcomes in patients with moderate-to-severe atopic dermatitis: A nationwide retrospective cohort study.

BACKGROUND: There is limited epidemiological evidence on outcomes associated with dupilumab exposure during pregnancy; monitoring pregnancy outcomes in large populations is required. OBJECTIVE: To investigate the potential association between exposure to dupilumab in pregnant women with atopic dermatitis and any adverse pregnancy, neonatal, congenital and post-partum outcomes. METHODS: We performed a multicentre retrospective cohort study across 19 Italian tertiary referral hospital. Childbearing women were eligible if aged 18-49 years and carried out the pregnancy between 1 October 2018 and 1 September 2022. RESULTS: We retrospectively screened records of 5062 patients receiving dupilumab regardless of age and gender, identifying 951 female atopic dermatitis patients of childbearing age, 29 of whom had been exposed to the drug during pregnancy (3%). The median duration of dupilumab treatment prior to conception was 22.5 weeks (range: 3-118). The median time of exposure to the drug during pregnancy was 6 weeks (range: 2-24). All the documented pregnancies were unplanned, and the drug was discontinued in all cases once pregnancy status was reported. The comparison of the study cohort and the control group found no significant drug-associated risk for adverse pregnancy, congenital, neonatal or post-partum outcomes. The absence of a statistically significant effect of exposure on the event was confirmed by bivariate analysis and multivariate analysis adjusted for other confounding factors. CONCLUSIONS: This cohort of pregnant patients exposed to dupilumab adds to the existing evidence concerning the safety of biologic agents in pregnancy. No safety issues were identified regarding the primary outcome assessed. In clinical practice, these data provide reassurance in case of dupilumab exposure during the first trimester. However, the continuous use of dupilumab throughout pregnancy warrants further research.

Vulvar vitiligo and lichen sclerosus in children: A clinical challenge.

Vulvar vitiligo (VV) and vulvar lichen sclerosus (VLS), both feature skin and mucosal hypo-/depigmentation. The aim of this study was to describe the clinical and dermoscopic features of VV and VLS in the pediatric population, providing diagnostic clues, and to define their association. We performed a systematic literature review of the clinical and dermoscopic features of pediatric VV and VLS. An observational study was conducted on children affected by VLS associated with VV, referred to the Dermatology Unit of the Sant'Orsola Polyclinic in Bologna, Italy. Medical history, age at diagnosis, ethnicity, clinical and dermoscopic features, and symptoms were recorded for all patients. 124 cases of VLS and 10 cases of VV were reviewed. Clinical manifestations included hypo-/depigmented patches in both conditions, while ecchymosis/purpura and fissures/erosion were observed in VLS. Symptoms including pruritus, pain, or burning were reported only by VLS patients. In our study five patients with VLS associated with VV were retrieved. Clinical features included well-demarcated depigmented patches in VV and translucent areas, erythema, ecchymoses/purpura, and labial fusion in VLS. Dermoscopy showed white structureless areas with a whipped cream-like appearance, linear or dotted vessels, white chrysalis-like structures, erosion and red-purpuric blotches in VLS and reduced pigment network or pigment absence, intralesional spots of residual pigmentation and telangiectasias in VV. Symptoms were present in all patients. Both VV and VLS show hypo-/depigmented patches. In the presence of associated symptoms, possible VLS should be investigated with clinical and dermoscopic examination to achieve a prompt diagnosis.

Short anagen syndrome: A case series and algorithm for diagnosis.

BACKGROUND: The short anagen syndrome (SAS) is a rare idiopathic pediatric disorder characterized by the short duration of the anagen phase. SAS mainly affects Caucasian children. Parents complain of their child's inability to grow long hair. Topical minoxidil may be an effective treatment for SAS; however, a slow spontaneous improvement is typical. OBJECTIVE: Our aim was to collect data on out cases of SAS and create an algorithm to facilitate diagnosis of SAS. METHODS: A retrospective review of 25 patients with SAS was performed within the Dermatology Department of the University of Bologna. We collected data regarding symptoms, pull test, hair card test, trichoscopy, trichogram, treatments, including biotin and minoxidil, and clinical outcome. RESULTS: Characteristic findings included parental reporting that the hair had not required a haircut, hair card test showing hairs with conical-shaped tips, and hair shafts of different diameters, with more 10%-20% of hair shafts less than 60 µm thick on trichoscopy. Trichogram revealed an increased percentage of telogen hair with normal hair shafts and tapering ends. The mean anagen-to-telogen ratio was 66:34 (normal ratio 90:10). CONCLUSION: We developed an algorithm to facilitate the diagnosis of this rare hair disease using clinical examination and invasive and non-invasive testing to differentiate SAS from other forms of pediatric alopecia. In conclusion, the collected data of the therapy showed that biotin alone or in combination with topical minoxidil is an effective treatment for SAS.

Basaloid follicular hamartomas in pediatric Basal Cell Nevus Syndrome: A diagnostic challenge.

Basal Cell Nevus Syndrome (BCNS) is an autosomal dominant inherited disease caused by PTCH1 (9q22.3-q31) germline mutations. Skin manifestations are mainly characterized by hyperkeratosis of the palms and soles, palmoplantar pits and a strong predisposition to develop multiple basal cell carcinomas (BCCs). Recently, it has been hypothesized that basaloid follicular hamartomas (BFH) could be included in BCNS skin features. We present three pediatric cases of GS with BCCs and BFHs. Clinical, dermoscopic and immunohistochemical tools are reported.

Oral Manifestations in Melanoma Patients Treated with Target or Immunomodulatory Therapies.

BACKGROUND: BRAF (v-raf murine sarcoma viral oncogene homolog B1) and MEK (mitogen activated protein kinase) inhibitors, as well as immunotherapy against cytotoxic T-lymphocyte-associated antigen 4 (CTLA-4) and the programmed cell death 1 (PD-1) receptor and its ligand (PD-L1), have shown good results in improving the disease-free survival of patients with metastatic melanoma (MM). The aim of this review is to summarize the main oral adverse events (oAEs) occurring in patients undergoing target or immunotherapy. We proposed two separate sections: oAEs during the treatment with (1) target therapies with BRAF and MEK inhibitors and tyrosine kinase inhibitors (gingival hyperplasia, pigmentation disorders, squamo-proliferative lesions) and (2) immunotherapies with CTLA-4 or PD1 inhibitors (lichenoid reactions, immuno-bullous reactions, xerostomia and other reactions). Adverse events frequently include oAEs, although these are often misdiagnosed and under-reported. Indeed, the oral cavity is not routinely evaluated during clinical practice. The symptomatology related to oAEs is significant since it may represent the first manifestation of a severe systemic reaction, possibly leading to difficulties in nutrition with a consequent impact on patients' quality of life. A careful examination of the oral cavity is recommended during the evaluation of oncologic patients in order to promptly detect the onset of new manifestations.

Nevocentric erythema multiforme in a child.

A 9-year-old girl presented with multiple, ring-shaped, erythematous, nonscaly plaques on the trunk, face and arms, most surrounding preexisting melanocytic nevi. She had experienced recurring episodes of herpes simplex labialis over several years (average 4/year), the last occurring 10 days prior.

Head and neck dermatitis, a subtype of atopic dermatitis induced by Malassezia spp: Clinical aspects and treatment outcomes in adolescent and adult patients.

BACKGROUND AND OBJECTIVES: Head and neck dermatitis (HND) is a clinical variant of atopic dermatitis (AD), presenting in adolescence or adulthood and characterized by involvement of the head, neck, and superior part of the trunk. The role of Malassezia spp has been advocated in the pathogenesis of HND, and antifungal agents represent the treatment of choice. METHODS: A retrospective single-center study was performed to define the clinical features and treatment response of HND among adolescent and adult patients. RESULTS: Thirty-one patients were identified, 17 with "adolescent-onset" and 14 with "adult-onset" HND. Adolescent-onset HND positively correlated with a past history of AD and presented with exclusive head and neck involvement (P < .05). Adult-onset HND was associated with concomitant widespread atopic eczema, involving the flexural areas of the upper and lower limbs, trunk, nipples, or hands (P < .05). A positive response to itraconazole in combination with topical treatments was observed in both groups. CONCLUSIONS: This study delineates two HND clinical phenotypes: adolescent vs adult onset. Different characteristics were observed in terms of relationship to AD and eczema localization. A history of AD in childhood and presentation with exclusive involvement of head and neck regions was observed predominantly in the adolescent-onset form, while adult-onset HND often occurred in association with diffuse dermatitis and a past history of AD was less frequent than in the adolescent group. The study is limited by the single-center retrospective nature, which may lead to diagnostic and selection biases, and the small cohort of patients.

Congenital haemangiomas: a single-centre retrospective review.

OBJECTIVE: Congenital haemangiomas (CHs) are rare, benign vascular tumours that are fully developed at birth. Three subtypes of CHs have been described based on clinical behaviour: rapidly involuting CHs (RICHs), non-involuting CHs (NICHs) and partially involuting CHs (PICHs). We explore in our study clinical, evolutionary and paraclinical characteristics of the three CH subtypes. DESIGN: Children with CH attending our department of paediatric dermatology at Bordeaux University Hospital over a 13-year period were retrospectively included. Epidemiological, clinical and evolutionary data, photographs and imaging results were reviewed. All available tissue samples were histologically examined. RESULTS: We included 57 patients: 22 with RICH, 22 with NICH and 13 with PICH. Males predominated (ratio 1.7); the most common CH location was on the limbs. RICH, NICH and PICH exhibited overlapping characteristics; all were single telangiectatic lesions with pale peripheral halos. At birth, NICHs were flat but RICHs and PICHs bulky. The median age at complete RICH involution was 12 months. One-third of CHs that appeared RICH-like at birth underwent incomplete involution to become PICHs. Heart failure and thrombocytopenia were rare complications. PICHs were frequently ulcerated. Pain was common for NICH and PICH. The imaging and histological data of the three CH subtypes were rather similar. CONCLUSIONS: We describe the characteristics and evolution of the three CH subtypes using a case series. Certain overlapping features were apparent, reinforcing the hypothesis that RICH, NICH and PICH lie on the same pathological spectrum.