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).

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.

Use of anifrolumab in systemic lupus erythematosus, cutaneous lupus erythematosus, and other autoimmune dermatoses. / "Uso del anifrolumab en el lupus eritematoso sistémico, lupus eritematoso cutáneo y otras dermatosis autoinmunes".

Anifrolumab is an inhibitor of the type I interferon receptor subunit 1 (IFNAR1) recently approved for the management of moderate-to-severe systemic lupus erythematosus (SLE). In 2 clinical trials, it has proven effective to treat cutaneous signs. Although anifrolumab has not been indicated for cutaneous lupus erythematosus (CLE), multiple cases and case series (20 publications with a total of 78 patients) have shown good and rapid responses with this drug, both in subacute CLE and discoid lupus erythematosus, as well as in lupus panniculitis and perniosis. Two case reports of dermatomyositis have also experienced clinical improvement with anifrolumab. Clinical trials of this drug are ongoing for subacute CLE and discoid lupus erythematosus, systemic sclerosis, and progressive vitiligo. Its most common adverse effects are respiratory infections and herpes zoster. Anifrolumab may be a well-tolerated alternative in the management of CLE.

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.

Mpox Pharyngitis.

A case of mpox pharyngitis in absence of cutaneous lesions is reported. Usually, clinical presentation is either a cutaneous eruption or a combination of cutaneous and mucosal lesions. In patients with atypical pharyngitis, regardless of the presence of skin lesions, pharyngeal swabs should be collected to rule out mpox.

Solid Facial Edema.

This case report describes nonpitting erythematous edema on the forehead, glabella, nose, and cheeks.

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.

Biologics Versus JAK Inhibitors. Part I: Cancer Risk. A Narrative Review.

INTRODUCTION: Biological drugs (BD) and Janus kinase inhibitors (JAKi) have revolutionized the treatment of diverse dermatoses. However, there are concerns regarding their safety, especially the risk of cancer and opportunistic infections. Here, we discuss the risk of cancer associated with the BD and JAKi used in dermatology. METHODS: A narrative review was carried out. All relevant articles evaluating the risk of cancer associated with BD or JAKi and published between January 2010 and February 2024 were selected. RESULTS: Multiple large studies have evaluated the association between BD, JAKi and cancer risk. However, there is a lack of prospective, comparative studies. Overall, patients undergoing BD and JAKi present a cutaneous cancer incidence similar to that in the general population. The drugs more strongly associated with non-skin cancer risk were anti-tumor necrosis factor (anti-TNFs) agents and JAKi (especially tofacitinib and oral ruxolitinib). This risk appears to increase with age, the presence of other factors (such as chronic immunosuppression from previous drugs or other comorbidities), and specific diseases such as rheumatoid arthritis (RA) and myelodysplastic syndrome. Conversely, BD such as interleukin (IL)-17 and IL-23 inhibitors may even reduce the risk of some visceral and hematological malignancies. In patients with dermatological conditions such as psoriasis and atopic dermatitis, the risk of malignancies may be lower than in other subgroups, and probably comparable to the general population. CONCLUSIONS: The incidence of cancer in patients undergoing BD or JAKi is generally low. This incidence can be higher in elderly patients with RA or myelodysplastic syndrome, and in those undergoing prolonged therapy with tofacitinib or ruxolitinib (oral), or anti-TNF agents.

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.

Certolizumab pegol effectiveness, survival and safety in patients with psoriasis: A multicenter retrospective analysis in daily clinical practice by the Spanish Psoriasis Group.

BACKGROUND: Certolizumab is an Fc-free PEGylated tumor necrosis factor-alpha (TNFα) inhibitor recently approved for the treatment of moderate-to-severe plaque psoriasis, although there is limited real-world evidence on the effectiveness and safety in patients with plaque psoriasis treated with certolizumab. The objective of this article is to determine the effectiveness, drug survival, and safety, including pregnancy, childbirth, and lactation, of certolizumab in moderate-to-severe plaque psoriasis under real-world conditions. METHODS: This is a retrospective, multicenter, observational study performed in 15 hospitals in Spain. It evaluates the effectiveness and safety of certolizumab in plaque psoriasis in the clinical practice setting. RESULTS: A total of 67 patients (73% female) were evaluated with a mean baseline Psoriasis Area Severity Index (PASI) of 8.9. At Week 12, the mean PASI was 2.3 (n = 67), 1.3 (n = 57) at Week 24 and 1.3 at Week 52 (n = 34). Absolute PASI < 3 was achieved in 69, 86, and 92% of patients at Weeks 12, 24, and 52, respectively, as observed. For its part, using the under-response imputation analysis, PASI < 3 at Weeks 12, 24, and 52 were achieved by 69, 73, and 49% of the patients, respectively. A total of 35 patients (52%) had concomitant psoriatic arthritis, and, in 24 of them, Disease Activity in Psoriatic Arthritis Score (DAPSA) was recorded at baseline, with a mean value of 17.9 which decreased to 8.2 at Week 12 (n = 22) and to 3.6 at Week 24 (n = 18). Certolizumab treatment was discontinued in 14 out of 67 patients (21%), due to lack/loss of cutaneous or articular effectiveness (n = 11) or patient decision (n = 2) or adverse event in only one patient who developed active tuberculosis. A lower baseline PASI [hazard ratio (HR): 1.12 (1.02-1.23); P = 0.023] and a more significant reduction in PASI at Week 12 [HR: 1.16 (1.07-1.27); P < 0.001] and Week 52 [HR: 1.47 (1.11-1.96); P = 0.007] was shown to be significantly related with better survival for the entire follow-up period. Fourteen patients were treated during pregnancy and/or lactation without reporting adverse events in either the patient or the newborn. CONCLUSIONS: Certolizumab consistently showed high effectiveness and drug survival rates in this real-life cohort. The safety demonstrated in clinical trials during pregnancy and lactation seems to be confirmed in clinical practice.

Gianotti-Crosti syndrome mimicking scabies.

This case report highlights a case of a 65-year-old woman who presented to our clinic with suspicion of refractory scabies. She had undergone multiple treatments without improvement. However, the clinical condition was not scabies but rather Gianotti-Crosti Syndrome (GCS), leading to rapid clinical improvement and avoiding the need for further unnecessary tests and treatments. GCS is a postviral exanthem characterized by symmetrical, red papules on the extremities and buttocks, typically occurring in children but can also affect adults. It is crucial for every physician to distinguish it from other causes of rash, including scabies, to ensure an accurate diagnosis and appropriate management.