Emerging Insights into Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis Induced by Immune Checkpoint Inhibitor and Tumor-Targeted Therapy.

Objective: Anticancer drugs have revolutionized tumor therapy, with cutaneous toxicities such as Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) being common immune-related adverse events. The debate over the efficacy of systemic corticosteroids in treating these conditions persists, while tumor necrosis factor (TNF)-alpha inhibitors show promise. This study aims to evaluate the effectiveness and safety of combination therapy involving the TNF-α inhibitor adalimumab for SJS/TEN induced by anticancer drugs. Methods: A literature review of SJS/TEN cases induced by anticancer drugs from 1992 to 2023 was conducted, alongside an analysis of patients admitted to the First Affiliated Hospital of Fujian Medical University during the same period. Clinical characteristics, skin healing time, mortality, and adverse events were evaluated in two treatment groups: SJS/TEN patients treated with targeted anticancer therapies and immunotherapies. Results: Among the 27 patients studied (18 with SJS or SJS-TEN overlapping and 9 with TEN), combination therapy with adalimumab significantly reduced mucocutaneous reepithelization time and healing duration compared to corticosteroid monotherapy. Patients receiving adalimumab combined with corticosteroids had lower actual mortality rates than those on corticosteroid monotherapy. The combination therapy also showed a trend towards reducing standardized mortality rates based on the Score of Toxic Epidermal Necrolysis (SCORTEN). Conclusion: The findings suggest that adalimumab in combination with corticosteroids provides significant clinical benefits and is safer than corticosteroids alone for treating SJS/TEN induced by targeted anticancer therapies and immunotherapies. This study contributes valuable insights into potential treatment strategies for severe cutaneous adverse reactions to anticancer drugs, highlighting the importance of exploring alternative therapies such as TNF-α inhibitors in managing these conditions effectively.

Multiple cutaneous myxofibrosarcoma on the right arm: Clinicopathological features and differential diagnosis.

The clinical presentations and pathological features of low-grade myxofibrosarcoma can be misleading, frequently resulting in diagnostic errors. An accurate diagnosis requires the application of immunohistochemistry techniques and the discerning diagnostic acumen of experienced pathologists. A 62-year-old male patient visited our outpatient clinic with multiple painful and rapidly enlarging subcutaneous nodules on his right forearm. Initially, the condition was misdiagnosed as multiple lipomas. The final pathology revealed characteristics consistent with low-grade myxofibrosarcoma.

APOA4 as a novel predictor of prognosis in Stevens-Johnson syndrome/toxic epidermal necrolysis: A proteomics analysis from two prospective cohorts.

BACKGROUND: Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are rare but life-threatening adverse drug reactions. Conventional systemic therapies are of limited efficacy and often exhibit strong side effects. OBJECTIVE: To assess the efficacy and safety of the combination treatment with a tumor necrosis factor-α antagonist adalimumab and delineate the underlying mechanisms. METHODS: We evaluated the efficacy and safety of the combination therapy with adalimumab by comparing 2 treatment cohorts of SJS/TEN patients. Patient plasma samples were collected for proteomics analysis. RESULTS: The combination therapy with adalimumab significantly shortened the time to mucocutaneous re-epithelization and healing, with reduced side effects caused by corticosteroids. Plasma proteomic profiling showed that apolipoprotein A-IV (APOA4) was one of the most significant differentially expressed proteins. Multivariate regression analysis revealed that APOA4 level was significantly associated with prognosis parameter of SJS/TEN (P = .004), but not with disease severity score (severity-of-illness score for toxic epidermal necrolysis [SCORTEN]) (P = .118). Thus further research will be helpful to effectively incorporate APOA4 into current SCORTEN-driven protocols. LIMITATIONS: The cohort size is relatively small. Both cohorts had low overall SCORTEN scores. CONCLUSION: Adalimumab in combination with corticosteroids demonstrates significant clinical benefits over corticosteroids alone in SJS/TEN patients. Moreover, APOA4 may serve as a novel prognostic marker of SJS/TEN.

Challenge of Nail Psoriasis: An Update Review.

Nail psoriasis is a refractory disease that affects 50-79% skin psoriasis patients and up to 80% of patients with psoriatic arthritis (PsA). The pathogenesis of nail psoriasis is still not fully illuminated, although some peculiar inflammatory cytokines and chemokines seems to be the same as described in psoriatic skin lesions. Psoriatic nail involving matrix can cause pitting, leukonychia, red spots in lunula, and nail plate crumbling, while nail bed involvement can result in onycholysis, oil-drop discoloration, nail bed hyperkeratosis, and splinter hemorrhages. The common assessment methods of evaluating nail psoriasis includes Nail Psoriasis Severity Index (NAPSI), Nail Assessment in Psoriasis and Psoriatic Arthritis (NAPPA), Nail Psoriasis Quality of life 10 (NPQ10), and so on. Treatment of nail psoriasis should be individualized according to the number of involving nail, the affected site of nail and presence of skin and/or joint involvement. Generally, topical therapies are used for mild nail psoriasis, while biologic agents such as etanercept are considered for severe nail disease and refractory nail psoriasis. Even though the current literature has shown some support for the pathogenesis, clinical presentation, or therapies of nail psoriasis, systemic review of this multifaceted disease is still rare to date. We elaborate recent developments in nail psoriasis epidemiology, pathogenesis, anatomy, clinical manifestation, diagnosis, differential diagnosis, and therapies to raise better awareness of the complexity of nail psoriasis and the need for early diagnosis or intervention.

Case Report: Successful Treatment of Acute Generalized Exanthematous Pustulosis With Secukinumab.

Acute generalized exanthematous pustulosis is a severe, usually drug-related reaction, characterized by an acute onset of mainly small non-follicular pustules on an erythematous base. Most cases of acute generalized exanthematous pustulosis (AGEP) clear quickly with a systemic corticosteroid, but severe or recalcitrant cases may need other systemic therapies. In this case, a man in his 40 s with a history of psoriasis consulted a physician about widespread erythema, pustules, target lesions, and fever after the administration of a quadruple antituberculosis drug. Routine laboratory testing revealed elevated white blood cell count and C-reactive protein. The histopathology showed subcorneal pustules, spongiosis as well as lymphocyte and eosinophils infiltration in the dermis. The patient was diagnosed with definitive AGEP according to the diagnostic score from the EuroSCAR study. Cutaneous lesions especially pustules and erythema multiforme-like lesions on the upper arms and palms are crucial for distinguishing AGEP from Generalized pustular psoriasis. The patient was treated with secukinumab as a result of his failure to respond to topical corticosteroids and constrain of systemic steroids. Remission with secukinumab therapy was safe without increased risks of infections. This case indicates that secukinumab is a potential therapy that can rapidly improve the clinical symptoms of AGEP.