Successful Treatment of Stimulator of Interferon Genes-Associated Vasculopathy of Infantile Onset SAVI Syndrome With Anifrolumab.

This case report describes a woman in her 30s who presented with cutaneous lesions since age of 2 to 3 years with erythema and pain on her nose, both cheeks, and ears when exposed to cold temperatures that progressed to nasal and auricular cartilage necrosis and was diagnosed with stimulator of interferon genes­associated vasculopathy with onset in infancy (SAVI syndrome).

Histological deep margins in cutaneous squamous cell carcinoma of the scalp and risk of recurrence.

BACKGROUND: Consensus is lacking on adequate deep histological margins in cutaneous squamous cell carcinoma (cSCC). Deep clearance for tumours located on the scalp is limited by anatomic constraints. OBJECTIVE: To determine whether clear but close deep histological margins (<1 mm) confer a higher risk of recurrence in cSCCs of the scalp treated by wide local excision, compared to deep histological margins ≥1 mm. METHODS: Multicentre retrospective observational cohort study and multivariate competing risk analysis to evaluate risk factors for recurrence. RESULTS: In total, 295 patients with 338 cSCCs were included. Close deep histological margins were not associated with an increased cumulative incidence of recurrence (subhazard ratio [SHR] 1.96 [95% CI 0.87-4.41]). However, an increased risk of recurrence was observed for those tumours that presented concurrent invasion of the galea aponeurotica and close deep margins, as opposed to patients without these factors (SHR 3.52 [1.24-10.01]). Tumours with clear but close peripheral margins (<1 mm) also had higher risk of recurrence (SHR 5.01 [1.68-14.97]). LIMITATIONS: Retrospective observational study based on pathology reports. CONCLUSION: Deep histological margins <1 mm do not confer a greater risk of recurrence as long as the tumour is completely excised and the galea aponeurotica is not involved. Surgical excision of cSCC on the scalp should include the galea to ensure proper assessment of deep margins.

Skin cancer risk after hematopoietic stem cell transplantation: a systematic review and meta-analysis.

Hematopoietic stem cell transplantation (HSCT) has improved outcomes for severe hematologic, malignant, and immune disorders, yet poses an increased risk of subsequent malignancies. This study aimed to examine the risk of skin cancer following HSCT and identify potential risk factors. The search was conducted in MEDLINE, EMBASE, and CINAHL databases until December 2023. Cohort studies reporting standardized incidence ratios (SIRs) for post-HSCT skin cancer or investigating risk factors were included. SIRs, or hazard ratios (HRs) with 95% confidence interval (CI), were calculated using random-effects inverse-variance models. Outcome endpoints were SIRs of skin cancer post-HSCT and risk factors, including gender, chronic graft-versus-host disease (cGVHD), voriconazole exposure, and total body irradiation (TBI). Twenty-six studies involving 164,944 HSCT recipients (allogeneic HSCT, n = 68,637; autologous HSCT, n = 95,435; mean age: 38.5 ± 13.8 years; 71,354 females [43.3%]) were analyzed. Overall, SIR for skin cancer post-HSCT was 7.21 (95% CI 3.98-13.08), with SIRs of 2.25 (95% CI: 1.37-3.68) for autologous HSCT, and 10.18 (95% CI 5.07-20.43) for allogeneic HSCT. Risk factors for skin cancer risk included cGVHD (HR = 2.86 [95% CI: 2.01-4.07]), specifically for basal cell and squamous cell carcinoma (SCC) (HR = 1.80 [95% CI: 1.31-2.46] and HR = 3.68 [95% CI: 2.39-5.68], respectively), male gender (HR = 1.56 [95% CI: 1.15-2.13]), especially for SCC (HR = 1.70 [95% CI: 1.03-2.80]), and voriconazole exposure (HR = 2.01 [95% CI: 1.12-3.61]). TBI showed no statistically significant association with subsequent skin cancer (HR = 1.12 [95% CI: 0.73-1.71]). These findings highlight the importance of rigorous skin cancer surveillance and preventive strategies in HSCT recipients, particularly in male individuals undergoing allogeneic transplants and those with identifiable risk factors, to enable early detection and intervention.

Real-world effectiveness and safety of bimekizumab for hidradenitis suppurativa: An ambispective observational study.

In this original research, we present the results in terms of effectiveness and safety of bimekizumab for hidradenitis suppurativa in real clinical practice. Results indicated significant improvement in all activity scores and patient-reported outcomes at week 16, including a notable decrease in mean IHS4 from 27.1 to 15.6 (p < 0.001), HS-PGA from 5.1 to 3.2 (p < 0.001), VAS pain from 8.3 to 4.7 (p < 0.001) and DLQI from 21.6 to 12.6 (p < 0.001). Bimekizumab, administered every 2 or 4 weeks, was well-tolerated with no discontinuations and no new safety concerns identified. These findings corroborate the drug's effectiveness and favourable safety profile observed in phase 3 clinical trials, supporting its use in real-world clinical practice for treating HS.

Scaling and periorificial crusts in a pediatric patient.

This case reports a 14-month-old child with Staphylococcal Scalded Skin Syndrome (SSSS). The child presented generalized scaling erythema accompanied by skin pain, and perioral crusts and fissures and she required hospital admission for antibiotic treatment with intravenous cloxacillin and hidroelectrolyte replacement.SSSS is a blistering skin disorder, mainly affecting children, caused by specific Staphylococcus aureus strains producing exfoliative toxins. It shows erythema in skin folds progressing to blisters within 48 h, often with perioral crusts and fissures. Its diagnosis relies on clinical assessment and it often requires intravenous antibiotics for its treatment.

Mohs micrographic surgery in immunosuppressed vs immunocompetent patients: Results of a prospective nationwide cohort study (REGESMOHS, Spanish registry of Mohs surgery).

BACKGROUND: Immunosuppressed (IS) patients, particularly solid organ transplant recipients and those on immunosuppressive therapy, face a higher incidence and recurrence of nonmelanoma skin cancers (NMSC), including basal cell carcinoma (BCC) and squamous cell carcinoma (SCC). Mohs micrographic surgery (MMS) is the preferred treatment for high-risk NMSC due to its high cure rate and margin examination capabilities. However, IS patients may experience more complications, such as surgical site infections, and a greater risk of recurrence, making their outcomes a subject of interest. OBJECTIVES: This study aimed to compare IS and immunocompetent (IC) patients undergoing MMS for NMSC in terms of baseline characteristics, intra- and post-surgical complications, and postoperative recurrence rates. METHODS: The study utilized data from the REGESMOHS registry, a 7-year prospective cohort study in Spain. It included 5226 patients, categorizing them into IC (5069) and IS (157) groups. IS patients included solid organ transplant recipients, those on immunosuppressive treatments, individuals with haematological tumours and HIV-positive patients. Patient data, tumour characteristics, surgical details and outcomes were collected and analysed. RESULTS: IS patients demonstrated a higher proportion of SCC, multiple synchronous tumours and tumours invading deeper structures. Complex closures, unfinished MMS and more surgical sections were observed in the IS group. Although intra-operative morbidity was higher among IS patients, this difference became non-significant when adjusted for other variables such as year of surgery, antiplatelet/anticoagulant treatment or type of closure. Importantly, IS patients had a substantially higher recurrence rate (IRR 2.79) compared to IC patients. CONCLUSIONS: This study suggests that IS patients may be at a higher risk of development of AE such as bleeding or tumour necrosis and are at a higher risk of tumour recurrence. Close follow-up and consideration of the specific characteristics of NMSC in IS patients are crucial. Further research with extended follow-up is needed to better understand the long-term outcomes for this patient group.