Search PubMed⌕ Search

SEARCH · Search PubMed

Results for “Stevens-Johnson Syndrome”

Search indexed PubMed citations on genomics, clinical trials, systematic reviews and public health. Explore titles, authors and supplied subject terms, then open the PubMed record.

Quote a phrase for an exact phrase match. Source license links do not imply unrestricted reuse.

At least 19 recordsLinked to original sources
PubMed2026

Crosstalk mediators implicated in the Stevens-Johnson Syndrome through gene regulatory network analysis.

Stevens-Johnson syndrome (SJS) is a rare and severe mucocutaneous disorder often triggered by medications or infections. Our previous research identified that four key genes, Ikzf1, Ptger3, Mavs, and Tlr3 are involved in SJS susceptibility and the conjunctival epithelial innate immune response, demonstrating their role in regulating interferon-stimulated genes. However, the interplay among these regulatory factors remains unclear. This study aimed to elucidate the crosstalk mechanisms between the pathways regulated by these four genes in conjunctival epithelial cells. We constructed a comprehensive gene regulatory network using transcriptomic data from murine conjunctival epithelial cells under 16 distinct conditions, including polyI:C stimulation across wild-type, knockout, and transgenic backgrounds for the key genes. A targeted network analysis systematically identified numerous candidate genes mediating the crosstalk between the regulatory pathways initiated by Ikzf1, Ptger3, Mavs, and Tlr3. The identified candidates suggest the involvement of diverse signaling pathways previously unlinked to SJS pathology. Our findings suggest that the pathogenesis of SJS may arise not from the dysfunction of isolated genes but from the disruption of a balance maintained by intricate pathway crosstalk.

Animals↗
PubMed2026

Non-genetic Risk Factors for Allopurinol-Induced Severe Cutaneous Adverse Reaction (SCAR): A Systematic Review.

BACKGROUND: Allopurinol-induced severe cutaneous adverse reactions (SCARs) are rare but potentially life threatening, particularly in Asian populations. While the genetic marker HLA-B*58:01 is a well-established risk factor, non-genetic factors may also contribute. This systematic review synthesizes evidence on associations between non-genetic risk factors and allopurinol-induced SCAR. METHODS: We searched MEDLINE, Scopus, Cochrane Library and Web of Science from inception to 29 June 2026 for observational studies examining non-genetic risk factors for SCAR, defined as Stevens-Johnson Syndrome, Toxic Epidermal Necrolysis, Acute Generalised Exanthematous Pustulosis, or Hypersensitivity Syndrome/Drug Reaction with Eosinophilia and Systemic Symptoms. Adults aged ≥ 18 years were included. Pooled odds ratios (ORs) with 95% confidence intervals (CIs) were calculated using a random-effects model; heterogeneity was assessed with I2. Mean differences were calculated for continuous variables. NIH Study Quality Assessment Tool was used for quality assessment of the studies. RESULTS: Twenty-six studies were included. Female sex (20 studies; 3340 SCAR cases, 562,647 controls) was associated with an increased risk of allopurinol-induced SCAR (OR 2.06; 95% confidence interval (CI) 1.25-3.38). Chronic kidney disease (16 studies; 1625 SCAR cases, 553,804 controls) was also significantly associated with SCAR (OR 3.78; 95% CI 1.99-7.17). Five studies (177 SCAR cases, 1368 controls) reported higher allopurinol doses among SCAR cases than tolerant controls (mean difference 19.61 mg; 95% CI 2.97-36.24). No significant associations were observed for age or concomitant diuretic use in the primary meta-analyses. Substantial heterogeneity was observed across studies. Sensitivity analyses demonstrated consistent findings for most factors, although concomitant diuretic use became significantly associated with SCAR after exclusion of non-Asian and zero-event studies. CONCLUSION: CKD, female sex, and higher allopurinol dose were identified as significant non-genetic risk factors for allopurinol-induced SCAR. These findings support consideration of non-genetic factors alongside pharmacogenomic screening in future risk-stratification strategies. However, substantial heterogeneity and potential publication bias limit the certainty of the available evidence. Well-designed studies evaluating non-genetic predictors as primary outcomes are needed to develop robust integrated risk prediction models for clinical decision making.

Journal Article↗
PubMed2025

HLA-B Alleles With Shared Peptide Binding Specificities Define Global Risk of Co-trimoxazole-Induced Severe Cutaneous Adverse Drug Reactions.

BACKGROUND: Co-trimoxazole is a leading global cause of severe cutaneous adverse drug reactions (SCAR) including Stevens-Johnson syndrome/toxic epidermal necrolysis (SJS/TEN) and drug reaction with eosinophilia and systemic symptoms (DRESS). Co-trimoxazole-induced SCAR are associated with HLA class I alleles including HLA-B&#x2217;13:01 and HLA-B&#x2217;38:02 in Southeast Asian (SEA) populations. However, the global generalizability of these associations is unknown but critical for population-appropriate risk stratification and diagnosis. OBJECTIVE: To determine HLA risk factors associated with co-trimoxazole-induced SJS/TEN and DRESS in populations from the United States and South Africa. METHODS: We performed high-resolution HLA typing on dermatologist-adjudicated co-trimoxazole-induced patients with SCAR in the United States (n = 63) and South Africa (n = 26) compared with population controls. Peptide binding and docking analyses were performed using MHCcluster2.0 and CB-Dock2. RESULTS: In a multiple logistic regression model, HLA-B&#x2217;44:03 (corrected P [Pc] < .001; odds ratio [OR] = 4.08), HLA-B&#x2217;38:01 (Pc < .001; OR = 5.66), and HLA-C&#x2217;04:01 (Pc = .003; OR = 2.50) were independently associated with co-trimoxazole-induced SJS/TEN in the United States. HLA-B&#x2217;44:03 was also associated with co-trimoxazole-induced DRESS in South Africa (Pc = .019; OR = 10.69). Distinct HLA-B variants with shared peptide binding specificities (SPBS) and HLA-C&#x2217;04:01 identified 94% and 78% of co-trimoxazole-induced SJS/TEN and DRESS in the United States, respectively. The SEA risk allele HLA-B&#x2217;13:01, with SPBS to HLA-B&#x2217;44:03, was identified in just one of 63 US patients with SCAR. CONCLUSIONS: HLA alleles with SPBS to SEA-related risk alleles, including HLA-B&#x2217;44:03 (SPBS with HLA-B&#x2217;13:01) and HLA-B&#x2217;38:01 (SPBS with HLA-B&#x2217;38:02) but also HLA-C&#x2217;04:01, predisposed to co-trimoxazole-induced SCAR in the United States and South Africa. These findings provide biological plausibility and strategies for global risk prediction and diagnosis of co-trimoxazole-induced SCAR.

Humans↗
PubMed1978

Toxic epidermal necrolysis from graft-vs-host disease. Occurrence in a patient with thymic hypoplasia.

An infant with diarrhea, failure to thrive, and a seborrhea-like skin eruption was thought to have fatal familial Leiner's syndrome. Treatment with nonirradiated plasma was followed by graft-vs-host disease and fatal toxic epidermal necrolysis; thymic hypoplasia was found at autopsy. Accurate diagnosis of immunodeficiency syndromes is essential to avoid potentially harmful therapy.

DiGeorge Syndrome↗
PubMed1976

Mucocutaneous lymph node syndrome in the United States.

Sixteen patients with an unusual and distinct symptom complex were encountered during a four-year period. Principal features of this syndrome are (1) fever lasting more than seven days; (2) conjunctival injection; (3) changes in the mouth consisting of erythema of the oropharynx, "strawberry tongue", and erythema of the lips; (4) indurative edema of hands and feet with palm and sole erythema followed by desquamation of the fingertips; and (5) an erythematous rash. Associated features were lymphadenopathy, pyuria, aseptic meningitis, diarrhea, arthritis, and arthralgia. Although usually a self-limited illness, one patient died with massive coronary artery thrombosis on the 19th day of illness. This syndrome appears to be clinically and pathologically similar to mucocutaneous lymph node syndrome, an illness prevalent in Japan but previously unrecognized by American clinicians. Pathologic features suggest a relationship to infantile periarteritis nodosa.

Arthritis, Juvenile↗
PubMed1975

Graft versus host reaction. An ultrastructural study.

Graft versus host reaction (GvH) following leukocytic transfusions occurred in a 34-year-old man with a generalized lymphosarcoma. Histologic and ultrastructural studies were performed, with special reference to dyskeratotic cells scattered in the epidermis. These cells are usually considered to be a constant and important feature of GvH reaction. Dense aggregation of tonofilaments, including cytoplasmic organelles and loss of desmosomes, were seen in dyskeratotic cells. Several intracellular desmosomes with tonofilaments bound to their attachment plaque were observed. Some of these cells were able to reach the horny layer; some others were phagocytosed by neighboring keratinocytes. These cells could be the result of a toxic damage to the epidermis, provoked by the immunologic phenomenon implicated in GvH reaction. Later in the clinical course, bullae formations occurred, showing some features of toxic epidermal necrolysis (TEN), as well as features of GvH reaction.

Adult↗
PubMed1978

Burn unit management of toxic epidermal necrolysis.

Toxic epidermal necrolysis is the name given to a group of dermatologic disorders characterized by a separation of epidermis and dermis with a subsequent skin slough. The denuded areas have the appearance of a second-degree burn. The complications of infection, negative nitrogen balance, severe pain, and emotional instability are identical to those seen in the patient with major burns. There are difficulties in patient management and advantages in burn unit care. As with the major burn, care of the patient with skin loss from toxic epidermal necrolysis is extremely complex, requiring the expertise of a burn team along with that of the dermatologist.

Adult↗
PubMed1976

Graft-versus-host reaction: a pathogenetic principle for the development of drug allergy, autoimmunity, and malignant lymphoma in non-chimeric individuals. Hypothesis.

It is proposed that the same diseases as those induced by the graft-versus-host reaction (GVHR) will arise in nonchimeric individuals, if structures of the major histocompatibility complex (MHC) on lymphocytes are altered, either by viral infection or by chemicals, in such a way that autologous T lymphocytes react against them as the react in the GVHR to semiallogeneic (F1) lymphocytes differing at the MHC. Diseases in which a GVHR-like pathogenesis is suspected will be discussed.

Animals↗
PubMed1976

Fibrinoid necrosis of the epithelial cells of the skin.

It has been shown in previous studies that fibrinoid necrosis not only occurs in connective tissue and in vessel walls, but can also be observed in the liver cells under special conditions and can also be provoked experimentally. It was observed in the present study that certain dermatoses (cases of the herpes group, erythema multiforme, drug eruptions, Lyell's toxic epidermal necrolysis, pityriasis lichenoides acuta, and skin affected by UV-rays) were associated with "eosinophilic necrosis" in the epithelial cells which morphologically corresponded to the fibrinoid necrosis of the connective tissue and of the liver cells described previously. Besides toxic, infectious and septic conditions, circulatory disturbances (hypoxia, anoxia) appear to have a special significance. Observations by way of the light and fluorescence microscope revealed the characteristics of keratin variants and precursors in some necrobiotic cells which influence the peculiar properties of fibrinoid necrosis of the skin epithelia. The role of the mixed paraproteins is emphasized and reference is made to the role of a mixed paraprotein ("keratofibrinoid") which is formed in the course of the regressive process and to which the morphological changes may be attributed.

Drug Eruptions↗