Wells syndrome following vaccination: A pediatric case with positive patch test to gelatin.

We report a 12-month-old boy with a skin eruption that developed 15 days after receiving the measles, mumps, rubella (MMR), pneumococcal, and meningococcal vaccines, consistent with the diagnosis of Wells syndrome. Patch testing showed a positive reaction to gelatin, which is used as a stabilizer for both live and inactivated vaccines. Gelatin was only present in the MMR vaccine.

Dupilumab in Patients with Atopic Dermatitis: A Multicentric, Long-Term, Real-World Portuguese Study.

INTRODUCTION: Several clinical trials have established the efficacy and safety of dupilumab for treating atopic dermatitis (AD). However, literature remains scarce in reporting the long-term effectiveness, safety, and drug survival of dupilumab in real-world settings. This study aimed to describe the latter outcomes of dupilumab in patients with AD. METHODS: This Portuguese, multicentric, observational, retrospective study included consecutive adult patients with AD who initiated dupilumab between January 2019 and September 2023, with a follow-up period up to 30 months. Drug discontinuation and adverse effects data were used to estimate drug survival. Clinical assessments included the Eczema Area and Severity Index (EASI), pruritus numerical rating scale (NRS), and Dermatology Life Quality Index (DLQI). RESULTS: A total of 312 patients were included in the study, with 56.4% being male (median age of 30 years, range 18-83). The 30-month drug survival rate was 82.0%. During the study period, 12.5% of the sample (n = 39 patients) discontinued treatment: 7.3% due to treatment failure, 2.9% due to safety concerns, 1.3% due to complete disease control, 0.6% due to pregnancy, and 0.3% due to lack of compliance. Adverse events not leading to drug discontinuation were noted in 25.6% of the sample (n = 80). Conjunctivitis was the most frequently reported adverse event (17%), followed by facial erythema (9%). At 30 months, the mean EASI decreased significantly from 27.30 ± 11.89 at baseline to 2.92 ± 3.96 (p < 0.001), reflecting an overall improvement of 89.3%. Similarly, pruritus NRS decreased from 7.36 ± 1.90 at baseline to 1.74 ± 2.16 at month 30 (p < 0.001), improving by 76.4%, and mean DLQI changed from 18.0 ± 7.09 at baseline to 2.67 ± 3.95 at month 30 (p < 0.001), decreasing by 85.2%. CONCLUSIONS: This study increases our current understanding of dupilumab in real-world settings, demonstrating its long-term effectiveness and safety in treating AD.

Comparison of non-criteria antiphospholipid syndrome with definite antiphospholipid syndrome: A systematic review.

Objectives: Patients with laboratory or clinical manifestations suggestive of antiphospholipid syndrome (APS) but not fulfilling the classification criteria constitute a clinical challenge. This study aims to compare non-criteria APS (NC-APS) with definite APS in terms of clinical manifestations, therapies, and outcomes. Methods: A systematic review of observational studies comparing definite and NC-APS was performed searching four electronic databases. Data on clinical manifestations, therapies and clinical outcomes was extracted. Results: Sixteen studies, assessing a total of 3,798 participants, were included. Seven out of 10 studies found no significant difference in the prevalence of arterial or venous thrombosis between definite and NC-APS, with two studies on seronegative APS also finding no difference in thrombosis recurrence. Seven out of 12 studies found no significant difference in the prevalence of obstetric manifestations between groups, with the remaining exhibiting conflicting results. In 9 studies comparing treatment frequency in obstetric patients, all but one described similar treatment frequency, with the percentage of NC-APS treated during pregnancy ranging from 26% to 100%. In 10 studies comparing pregnancy outcomes of NC-APS versus definite APS, 7 found similar successful pregnancies/live births. Additionally, 5 studies described improvement of live births in both groups with treatment, with three signalling aspirin monotherapy as efficacious as combination therapy in NC-APS. Conclusion: This review hints at an absence of marked differences in most evaluated parameters between definite and NC-APS, emphasizing the value of a more active follow-up of these patients. The low-quality available evidence highlights the need for well-defined NC-APS populations in future studies. Systematic Review Registration: https://www.crd.york.ac.uk/prospero, identifier CRD42020210674.

Adverse Cutaneous Drug Reactions in Children: A Retrospective Study in a Tertiary Hospital.

Adverse reactions to drugs are a major concern in health, and children seem to be particularly vulnerable to these reactions. Cutaneous reactions account for 35% of the drug-related adverse effects in children. We conducted a retrospective study to characterize the pediatric population having a diagnosis of cutaneous adverse drug reactions (CADRs) in children admitted in a tertiary hospital during 6 years. (SKINmed. 2022;20:126-129).

Clinicopathological features of head and neck cutaneous basal cell carcinoma observed at the Centro Hospitalar de Trás-os-Montes e Alto Douro ENT Department.

Background: Cutaneous basal cell carcinoma (cBCC) incidence has been increasing, but there are no available data on its epidemiological, clinical, and pathological patterns in Northeast Portugal. cBCC is mainly located in the head and neck, where the ear, neck, and throat (ENT) surgeon may have a major role. We aimed to verify the clinicopathological characteristics of basal cell carcinomas diagnosed in an ENT department. Methods: We performed a retrospective clinicopathological evaluation of the head and neck cBCC cases followed up at the Centro Hospitalar de Trás-os-Montes e Alto Douro (CHTMAD) ENT Department between January 2007 and April 2021. Results: One hundred seventy-four patients with 293 cBCCs were included in this retrospective study. We observed that about one-third of the patients had multiple cBCCs (30.5%) and an infiltrative-type growth pattern (39.3%), both features considered as patterns that are more aggressive. Infiltrative-type growth pattern cBCCs were significantly larger when compared with the indolent-type growth pattern (16.2 mm vs 10.8 mm). Conclusions: To the best of our knowledge, this is the first study about cBCC in a patient population followed up at an ENT hospital department. This study has shown that these patients had cBCCs with more aggressive features, making these tumors an important issue for the ENT surgeon.

Langerhans Cell Histiocytosis-A Benign Presentation Heralding a Serious Disease.

A 3-year old White boy was referred to our dermatology department with a papular disseminated eruption, evolving for 7 months. Several topical antibiotics and corticosteroids were used without improvement. The dermatosis was locally asymptomatic, and systemic symptoms were absent. Examination revealed multiple, skin-colored to pinkish monomorphic papules with a generalized distribution involving the face, trunk, and limbs (Figure 1). The lesions spared the scalp, palms, and soles. Cervical, axillary, and inguinal lymphatic nodes were not palpable. Cutaneous biopsy of one of the abdominal lesions revealed an unremarkable epidermis but a reticular dermis with clusters of histiocytic, lymphocytic, and rare eosinophil cells. In the immunohistochemical study, expression of CD1a was observed in the histiocytic cells and S100 in the antigen-presenting cells of the dermal infiltrate (Figures 2 and 3). Taking into account the clinical presentation and the histopathologic result, a diagnosis of Langerhans cell histiocytosis (LCH) was established.

TERTp mutations and p53 expression in head and neck cutaneous basal cell carcinomas with different aggressive features.

Cutaneous basal cell carcinoma (cBCC) is an economic burden to health services, due to its great morbidity and increasing incidence in old people. Infiltrative cBCCs and cBCCs with micronodular pattern are considered as more aggressive. The role of p53 expression and TERTp mutation on cBCC behavior remains to be clarified. We aimed to assess TERTp mutations and p53 expression in relation to the cBCC histological subtype in a cohort of patients referred to an ENT Department of a tertiary Hospital of Northern Portugal. We performed a retrospective clinicopathological and histological review of the head and neck cBCCs followed-up at the otorhinolaryngology department of Trás-os-Montes e Alto Douro hospital (January 2007-June 2018). We assessed TERTp mutations in 142 cBCCs and p53 protein expression, through immunohistochemistry, in 157 cBCCs. We detected TERTp mutations in 43.7% of cBCCs and p53 overexpression in 60.5% of cBCCs. We spotted association of p53 overexpression and TERTp mutation with necrosis. In the infitrative-growth pattern cBCCs, there was no significant association with the clinical and histological features evaluated, except for necrosis. In the indolent-growth cBCCs, we identified a significant association of TERTp mutation status with female sex, necrosis, multiple cBCCs, and p53 positive expression. Our results suggest that TERTp mutation may be useful to identify more aggressive features in the indolent-growth pattern cBCCs (nodular and superficial subtypes). Further studies with larger cohorts are warranted to clarify the relevance of TERTp mutation in cBCCs.

Diffuse Large B-Cell Lymphoma in the Setting of Chronic Lymphocytic Leukemia.

A 77-year-old man was referred to our department with a 6-month history of a painless, rapidly enlarging tumor on the left leg. Fourteen years ago, he was diagnosed with chronic lymphocytic leukemia (CLL), and he had presented with lymphocytosis and multiple lymphadenopathies (Rai stage II). Both bone marrow aspiration and peripheral blood immunophenotyping identified a monoclonal B-cell population expressing surface CD5, CD20, CD23, CD43, and IgG kappa. At that time, he underwent eight courses of treatment with oral fludarabine, followed by complete remission, and he had been clinically stable ever since (Rai stage 0).