Toxic epidermal necrolysis (TEN) is a rare but potentially life-threatening dermatological condition. It is characterised by diffuse exfoliation of >30% of the skin and mucous membranes due to immune-mediated destruction of the epidermis. It is thought to be due to a hypersensitivity reaction to a variety of medications. The mainstay of treatment involves withdrawal of the potential offending agent, supportive care and use of non-adherent dressings. Pre-packaged dressings are expensive or not readily available in our locality; hence, the need for improvisation, to manage the condition and improve outcomes. We report a case of TEN in a 2-year old female who presented with a 6-day history of fever and a rash on the trunk, for which mother applied topical calamine lotion, gave an oral herbal concoction for 3 days, with no improvement, after which she commenced amoxicillin + clavulanic acid. Twenty-four hours later, the rash progressed to involve the whole body. On presentation, she had extensive exfoliation of >70% of the body surface area, with multiple bullae, intraoral ulcers and excoriation of the eyelids and genitals. She was admitted into the Paediatric Intensive Care Unit, where she received intravenous hydration and antibiotics, micronutrients, nasogastric feeds and eye care. The skin was cleaned daily with 0.9% saline and dressed with petroleum jelly-impregnated gauze prepared at the Central Sterilising Services Department of the hospital. Skin ulcers resolved, and she was discharged after 6 weeks. In resource-poor settings such as ours, cheaper, sterile locally available materials could be used for dressing to improve outcomes.

We report a rare long-term case in which systemic polyarteritis nodosa (PAN) was ultimately diagnosed in adulthood after childhood-onset recurrent inflammatory disease with atypical Still-like features. A Japanese man had recurrent febrile episodes from infancy and was diagnosed with juvenile idiopathic arthritis at 9 years of age. In early adulthood, he developed recurrent fever, rash, polyarthralgia, splenomegaly, neutrophilia, and elevated inflammatory markers, and was considered to have Still-spectrum disease. However, ferritin elevation remained modest despite marked systemic inflammation, suggesting that the early phenotype may not have represented typical Still's disease and may instead have reflected a broader autoinflammatory process. At 25 years of age, he developed digital ischaemia with necrosis, palpable nodules, and erectile dysfunction. Angiography demonstrated narrowing of the ulnar and palmar arteries, and skin biopsy showed necrotising vasculitis involving medium-sized vessels, leading to the diagnosis of systemic PAN. He subsequently developed fever, rash, inner-ear involvement, hypertrophic pachymeningitis, and tortuosity/dilatation of a right intercostal artery. A subtle suspected lesion of the right testicular artery later became a definite enlarging aneurysmal lesion on serial imaging. Systemic inflammatory manifestations were subsequently controlled during treatment that included glucocorticoids, azathioprine, and tocilizumab. However, serial imaging demonstrated gradual enlargement of the right testicular artery aneurysmal lesion despite the absence of fever, rash, or C-reactive protein elevation for 5 years. This clinical course suggests that vascular progression may not always be adequately reflected by C-reactive protein levels during interleukin-6 blockade. This case illustrates the diagnostic difficulty of childhood-onset recurrent inflammatory disease with atypical Still-like features that was ultimately recognized as systemic PAN in adulthood. Occult or under-recognized medium-vessel vasculitis may have been present earlier in the disease course. Long-term diagnostic reappraisal, vascular imaging when indicated, and continued follow-up across paediatric and adult rheumatology care may therefore be important in similar patients, particularly in the current biologic era.

Acute nontraumatic mediastinitis is a rare and potentially life-threatening condition. In children, management strategies remain poorly standardized. Here, we describe four pediatric cases: two originating from cervical infections (descending necrotizing mediastinitis [DNM]) and two with indeterminate source. The first two patients presented with febrile otorhinolaryngologic symptoms, one complicated by secondary respiratory failure and the other by possibly toxin-related symptoms. The third presented with varicella and scarlet fever-like rash, complicated by multiorgan involvement secondary to a probable toxic shock syndrome. The fourth case presented with fever without focus and shoulder pain. Three patients developed secondary pleural effusions. In three cases, diagnosis was confirmed by computed tomography (CT) scan and in one case by MRI. None had necessitated surgical drainage. All patients were managed conservatively with prolonged antibiotic therapy and achieved complete recovery without sequelae. These cases suggest that, in selected pediatric patients, a conservative, antibiotic-based approach can be both safe and effective.

Adult-onset Still's disease (AOSD) is a rare systemic autoinflammatory disorder characterized by fever, rash, arthritis, and hyperferritinemia, often presenting as a diagnostic challenge due to its nonspecific manifestations. We report the case of a 35-year-old previously healthy male who presented with a 20-day history of pruritic erythematous skin lesions, high-grade fever, severe odynophagia, and symmetric inflammatory polyarthritis. Laboratory evaluation revealed leukocytosis with neutrophilia, elevated acute-phase reactants, liver enzyme abnormalities, and hyperferritinemia (1,050 ng/mL). Infectious, autoimmune, and neoplastic causes were excluded. During hospitalization, the patient developed chest pain with electrocardiographic changes and elevated troponin levels, consistent with acute pericarditis. Skin biopsy demonstrated nonspecific inflammatory dermatitis. A diagnosis of AOSD was established based on clinical findings and Yamaguchi criteria. Initial treatment with systemic corticosteroids resulted in partial improvement; however, early relapse required escalation to tocilizumab, achieving complete clinical remission at two months. This case highlights the importance of considering AOSD in patients with fever of unknown origin and elevated inflammatory markers, as well as the role of early targeted therapy in improving outcomes.

Objective: Metagenomic sequencing was employed to analyze the pathogen detection profile in pediatric severe acute non-A-E hepatitis. Methods: Based on the platform of the China Childhood Severe Acute Hepatitis Collaborative Group, a case series study was conducted. This study enrolled 36 children with severe acute non-A-E hepatitis, who were admitted to 17 hospitals between April and July 2022. Clinical data, including etiological test results and liver function tests, were collected, and peripheral blood and nasopharyngeal swab specimens were obtained. Metagenomic next-generation sequencing (mNGS) was performed to detect potential infectious pathogens. Results: Among 36 children, there were 24 males and 12 females, with an onset age of 3.5 (1.1, 9.0) years. Common clinical symptoms were fever in 22 cases (61%), jaundice in 13 cases (36%), vomiting in 12 cases (33%), abdominal pain in 10 cases (28%), rash in 10 cases (28%), and diarrhea in 3 cases (8%). Serum alanine aminotransferase and aspartate aminotransferase levels were 950 (826, 1 404) and 811 (498, 1 295) U/L, respectively. Using PCR, plasma Epstein-Barr virus (EBV)-DNA was tested in 31 cases (86%) and plasma cytomegalovirus (CMV)-DNA in 25 cases (69%), and all results were below 5×10[5] copies/L. Plasma mNGS was performed on all 36 patients, detecting 11 viruses. These included EBV in 14 cases, CMV in 12 cases, human adenovirus in 2 cases, herpes simplex virus type 1 in 2 cases, adeno-associated virus type 2 (AAV2) in 1 case, and 6 other viruses. No patient tested positive for both human adenovirus and AAV2 simultaneously. Plasma mNGS results showed a viral read count of 4 (2, 10) per 1×10[8] reads. Nasopharyngeal swab mNGS was performed on 8 cases (22%), detecting 7 viruses. These included human herpesvirus 7 in 4 cases, EBV in 3 cases, and CMV in 3 cases, as well as 4 other viruses. Human adenovirus and AAV2 were not detected. Among the 4 children with human herpesvirus 7, 2 cases were also positive for human herpesvirus 6B, but neither virus was detected in their plasma mNGS. Conclusions: The detection rates of human adenovirus and AAV2 are both low among children with severe acute non-A-E hepatitis, and there are no cases of co-infection with both viruses.