In 1963, Rowellet al. described a syndrome combining lupus erythematosus (LE) and erythema multiforme (EM)-like lesions. In this report, we present a 15-year-old female who presented with both systemic LE and EM-like skin lesions meeting all of the major and one of the minor criteria for a diagnosis of Rowell syndrome. Her condition improved with administration of systemic and topical corticosteroids, and hydroxychloroquine. Rowell Syndrome, a rare entity, is often debated as a coincidental overlap of other conditions according to Bonciolini,et al.[1]In light of Rowell Syndrome's infrequency and the paucity of available literature, we emphasize the clinical significance of recognizing this challenging condition.

Toxic epidermal necrolysis (TEN) is a rare, life-threatening dermatological emergency characterized by extensive epidermal necrosis and detachment, along with significant mucous membrane erosions and systemic symptoms. While conventional medications such as sulfonamides and anticonvulsants are known triggers, recent reports suggest that herbal medications can also induce TEN. This case report highlights the development of TEN in a patient with invasive cervical cancer following the use of an herbal supplement.

A 48-year-old Filipino woman with newly diagnosed invasive cervical cancer developed TEN after taking an herbal supplement containing 23 botanical extracts. Despite discontinuing the supplement, her condition rapidly worsened, with vesicles and bullae progressing to epidermal detachment over 90% of her body surface area. A histopathological examination confirmed the diagnosis of TEN. The patient was treated with systemic corticosteroids and supportive care, achieving full recovery within 14 days.

This case underscores the potential risks associated with herbal medications, particularly in vulnerable patients such as those with cancer. Although conventional pharmaceuticals are the primary culprits for TEN, this report highlights the need for greater awareness of herbal medicine-induced adverse drug reactions. Underreporting of such events, due to lack of awareness among patients and healthcare providers, further complicates the issue. This case emphasizes the need for strengthened regulatory policies and enhanced education to safeguard public health amidst the increasing use of herbal remedies worldwide.

Acute generalized exanthematous pustulosis (AGEP) and Stevens-Johnson Syndrome (SJS) are uncommon, severe cutaneous drug eruptions with distinct clinical and histopathological features. AGEP-SJS overlap is a rare and complicated cutaneous drug eruption. Neutrophilia, leukocytosis, and elevated liver enzymes can be seen in these patients. Currently, there are no available dermoscopic studies on AGEP overlapping SJS. The pathophysiology of overlapping drug reaction are mediated by T cells and delayed-type hypersensitivity. Management includes removal of offending drug and giving supportive measures like pain management, moist dressing and fluids.

Stevens-Johnson syndrome (SJS), also known as the multifactorial erythematous drug eruption, is a class of adverse reactions of the skin and mucous membranes primarily caused by drug allergy often involving the oral cavity, eyes, and external genital mucosa, generally accompanied by fever, and can be life-threatening in severe cases. In February 2022, the Department of Stomatology, the First Affiliated Hospital of Zhengzhou University admitted a patient with huge inflammatory hyperplasia of bilateral lingual margins secondary to SJS. Upon admission, no other obvious symptoms were observed except for tongue hyperplasia. The patient suffered from a severe adverse drug reaction caused by acetaminophen 2 months ago and was complicated by liver dysfunction and pulmonary infection. After 1 month of treatment and rehabilitation, he developed a secondary tongue mass and was subsequently admitted to Dept. of Oral and Maxillofacial Surgery Ward 2, the First Affiliated Hospital of Zhengzhou University. After completing the examination, the tongue mass was surgically removed. After a follow-up of 11 months, the patient's condition was satisfactory and no temporary discomfort was observed. The case of tongue mass secondary to SJS is extremely rare. If a stomatologist encounters a similar case, we should carefully inquire about the drug allergy history and recent medication history, and be alert to whether or not they had adverse drug reactions recently.
Male ; Humans ; Stevens-Johnson Syndrome/drug therapy* ; Hyperplasia/pathology* ; Skin ; Drug Hypersensitivity/pathology* ; Tongue

Child ; Family ; Humans ; Necrosis ; Skin Diseases ; Stevens-Johnson Syndrome/etiology*

Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are rare but severe diseases. This study aimed to validate the predictive ability of risk models in patients with SJS/TEN and propose possible refinement in China. Patients in the Department of Dermatology of Huashan Hospital from January 2008 to January 2019 were included. Results showed that the severity-of-illness score for TEN (SCORTEN) had a good discrimination (area under the receiver operating characteristic curve (AUC), 0.78), and it was superior to auxiliary score (AS) and ABCD-10, which indicates age, bicarbonate level, cancer, dialysis, and 10% involved body surface area (AUC, 0.69 and 0.68, respectively). The calibration of SCORTEN (Hosmer-Lemeshow goodness-of-fit test, P = 0.69) was also better than that of AS (P = 0.25) and ABCD-10 (P = 0.55). SCORTEN and ABCD-10 were similar (Brier score (BS), 0.04 and 0.04) in terms of accuracy of predictions. In addition, the imaging appearance of pulmonary consolidation on computed tomography was associated with high mortality. Refined models were formed using the variables and this imaging appearance. The refined AS and ABCD-10 models were similar in discrimination compared with the original SCORTEN (0.74 vs. 0.78, P = 0.23; 0.74 vs. 0.78, P = 0.30, respectively). Therefore, SCORTEN showed good discrimination performance, calibration, and accuracy, and refined AS or ABCD-10 model may be an option when SCORTEN variables are not available.
Cohort Studies ; Humans ; Retrospective Studies ; Severity of Illness Index ; Stevens-Johnson Syndrome/diagnostic imaging* ; Tomography

Stevens-Johnson syndrome/toxic epidermal necrolysis (SJS/TEN) is a rare adverse cutaneous reaction with a low incidence and high mortality. Despite posing a serious threat to patients' health and lives, there is no high-quality evidence for a standard treatment regimen. Here we report the case of a 62-year-old man with stage IV pancreatic cancer who experienced immunotherapy-induced SJS/TEN. After consensus-based regular treatments at a local hospital, his symptoms became worse. Thus, he consented to receive Chinese herbal medicine (CHM) therapy. The affected parts of the patient were treated with the CHM Pi-Yan-Ning which was applied externally for 20 min twice a day. After 7 days of treatment, the dead skin began peeling away from the former lesions that had covered his hands, feet, and lips, indicating that skin had regenerated. After 12 days of treatment, the patient's skin was completely recovered. In this case, SJS/TEN was successfully treated with Pi-Yan-Ning, suggesting that there might be tremendous potential for the use of Pi-Yan-Ning in the treatment of severe skin reactions to drug treatments. Further basic investigations and clinical trials to explore the mechanism and efficacy are needed.
Drugs, Chinese Herbal/therapeutic use* ; Humans ; Immunologic Factors ; Incidence ; Male ; Middle Aged ; Skin ; Stevens-Johnson Syndrome/etiology*

Introduction: Erythema multiforme has been known as an infection or drug-associated mucocutaneous eruption characterized by target lesions. A clinical entity, known as Mycoplasma-induced rash and mucositis seen mostly in the pediatric population is emerging and may be associated with atypical pneumonia caused by Mycoplasma pneumoniae. This presents with features overlapping with erythema multiforme and SJS-TEN spectrum but with a different trigger, prognosis, and recurrence rate. Case summary: Target lesions in the clinical setting are usually characteristically associated with erythema multiforme, a mucocutaneous condition associated with an underlying infectious trigger. We present a case of a 10-year-old Filipino boy who was initially diagnosed with erythema multiforme major. Eventual testing for the etiology of the underlying infection, Mycoplasma pneumoniae, proved to be a useful diagnostic that gave a better grasp on the case’s mechanism, sequela, and prognosis. The patient was admitted for pneumonia and his presenting mucositis was severe. Cutaneously, he had atypical target and few target lesions on the trunk and extremities. He was diagnosed as a case of Mycoplasma-induced rash and mucositis (MIRM) and treated with antibiotics and systemic steroids for which he recovered fully in three weeks. MIRM should be separated from erythema multiforme, Stevens Johnsons syndrome and toxic epidermal necrolysis as it follows a different disease course. Conclusion: Mycoplasma-induced rash and mucositis is now considered a distinct entity despite it having overlapping features with erythema multiforme and SJS-TEN spectrum. It presents usually in the younger age group with absent to sparse atypical vesiculobullous or targetoid lesions, significant mucosal involvement, and confluent necrosis on histology. It is important to identify it as a trigger because of its more frequent and severe mucosal sequelae. Management includes symptomatic relief, antibiotic therapy with a macrolide in the presence of pneumonia and systemic steroids when mucositis is severe. Majority of patients achieve full recovery.
Erythema Multiforme ; Mycoplasma pneumoniae ; Mucositis ; Exanthema

Drug reaction with eosinophilia and systemic symptoms (DRESS) syndrome and toxic epidermal necrolysis (TEN) are severe cutaneous adverse reactions. Although viral reactivation is associated with DRESS syndrome, its role in TEN remains unclear. An 80-year-old woman visited our hospital because of fever and skin eruption. DRESS syndrome was diagnosed and was thought to caused by the use of the drug allopurinol. She was treated by discontinuation of the drug and administration of systemic steroids. She recovered from DRESS syndrome and was discharged from the hospital with tapering doses of steroids prescribed. One week after discharge, she visited our hospital again as the skin rash recurred and oral pain as well as oral and ocular mucosal lesions developed. In addition to the skin rash, blisters and Nikolsky's sign that were different from the skin lesions present in the previous DRESS syndrome were observed. Unlike those in DRESS syndrome, the viral serological test results were positive for anti-cytomegalovirus (CMV) IgM and CMV polymerase chain reaction. Therefore, it was thought that TEN was due to reactivation of CMV and she was treated this with ganciclovir and intravenous immunoglobulin. Here, we report a case of TEN caused by viral reactivation after DRESS syndrome developed after use of allopurinol which recovered after steroid treatment.
Aged, 80 and over ; Allopurinol ; Blister ; Cytomegalovirus Infections ; Cytomegalovirus ; Drug Hypersensitivity Syndrome ; Eosinophilia ; Exanthema ; Female ; Fever ; Ganciclovir ; Humans ; Immunoglobulin M ; Immunoglobulins ; Polymerase Chain Reaction ; Serologic Tests ; Skin ; Steroids ; Stevens-Johnson Syndrome

Stevens-Johnson syndrome (SJS), and toxic epidermal necrolysis (TEN). One class of medications that has been highly associated with such drug eruptions is antiepileptic drugs (AEDs). We attempt to investigate drug eruptions associated with AEDs as a class, as well as with individual AEDs, in Korea.METHODS: We used the Korea Institute of Drug Safety and Risk Management - Korea Adverse Event Reporting System (KIDS-KAERS) database, a nationwide database of adverse events reports, between January 2008 and December 2017 to investigate the reporting count of all drug eruptions and calculated the ratio of DRESS/SJS/TEN reports for each AED.RESULTS: Among a total of 2,942 reports, most were of rash/urticaria (2,702, 91.8%), followed by those of DRESS (109, 3.7%), SJS (106, 3.6%), and TEN (25, 0.85%). The common causative AEDs were lamotrigine (699, 23.8%), valproic acid (677, 23%), carbamazepine (512, 17.4%), oxcarbazepine (320, 10.9%), levetiracetam (181, 6.2%), and phenytoin (158, 5.4%). In limited to severe drug eruptions (DRESS, SJS, and TEN; total 241 reports), the causative AEDs were carbamazepine (117, 48.8%), lamotrigine (57, 23.8%), valproic acid (20, 8.3%), phenytoin (15, 6.3%), and oxcarbazepine (10, 4.2%). When comparing aromatic AED with non-aromatic AED, aromatic AEDs were more likely to be associated with severe drug eruption (aromatic AEDs: 204/1,793 versus non-aromatic AEDs: 37/1,149; OR, 3.86; 95% CI, 2.7–5.5). Death was reported in 7 cases; DRESS was the most commonly reported adverse event (n = 5), and lamotrigine was the most common causative AED (n = 5).CONCLUSION: Although most cutaneous drug eruptions in this study were rash or urticaria, approximately 8% of reports were of severe or life-threatening adverse drug reactions, such as SJS, TEN, or DRESS. When hypersensitivity skin reactions occurred, aromatic AEDs were associated with 4 fold the risk of SJS/TEN/DRESS compared with non-aromatic AEDs. Our findings further emphasize that high risk AEDs should be prescribed under careful monitoring, and early detection and prompt interventions are needed to prevent severe complications.]]>
Anticonvulsants ; Carbamazepine ; Drug Eruptions ; Drug Hypersensitivity Syndrome ; Drug-Related Side Effects and Adverse Reactions ; Exanthema ; Hypersensitivity ; Korea ; Pharmacovigilance ; Phenytoin ; Risk Management ; Skin ; Stevens-Johnson Syndrome ; Urticaria ; Valproic Acid