Necrobiosis lipoidica in childhood: a review of literature with emphasis on therapy.

Necrobiosis lipoidica (NL) is a rare chronic granulomatous disease that manifests as sharply demarcated, telangiectatic, brownish-red plaques with atrophic yellowish centers prone to ulceration and occurs predominantly on the shins. In children, NL is extremely rare, but resistance to therapy, troublesome cosmetic appearance, painful ulcerations, and possible development of squamous cell carcinoma in long-persisting lesions are challenges during treatment. Our review includes 29 reports of NL in patients aged <18 years published from 1990 on PubMed, EMBASE, and Medline. The mean age of patients was 14.3 years, with a female predominance of 2 : 1 and a high prevalence of diabetes mellitus (80%). Data showed that potent topical steroids up to twice daily is the first-line treatment. For refractory cases, therapy can be switched to tacrolimus. Ulcerations benefit from phase-adapted wound care and anti-inflammatory medical dressings such as medical honey. Adding hyperbaric oxygenation to local or systemic therapy in difficult-to-treat ulcerated lesions can be considered. Refractory cases may be switched to topical photochemotherapy or systemic treatment with TNF-α inhibitors, systemic steroids (preferably in non-diabetic patients), pentoxifylline, or hydroxychloroquine. Necrobiosis lipoidica in childhood is difficult to treat, with a treatment failure rate of 40%. Therefore, further research through patient registries is recommended.

[Treatment of chronic hard-to-heal wounds with hyaloronic acid ester: a case series of six patients]. / Therapie von schwerbehandelbaren chronischen Wunden mit Hyaluronsäureester: Eine Fallbeschreibung über sechs Patient*innen.

The treatment of chronic wounds is usually a major challenge for the involved medical staff. Various different treatment options have to be tested achieving a satisfactory result. In this retrospective case report, we describe successful treatments with hyaluronan acid ester fleece in six patients with chronic wounds of different origins. Furthermore, all patients received compression bandages or compression stockings, two patients were treated additionally with pressure relief devices and all were debrided if necessary. The chronic wounds of five out of the six patients healed completely over a period of 1.5-11 months or only with a minimal residual defect. However, one patient was still resistant to therapy over five months. In summary, the treatment with hyaluronan esters achieved good results especially in patients with complex medical history that makes a successful therapy particularly difficult.

More than keratitis, ichthyosis, and deafness: Multisystem effects of lethal GJB2 mutations.

BACKGROUND: Infant death in keratitis-ichthyosis-deafness (KID) syndrome is recognized; its association with specific genotypes and pathophysiology is inadequately understood. OBJECTIVE: We sought to discover characteristics that account for poor outcomes in lethal KID syndrome. METHODS: We collected 4 new cases and 9 previously reported, genotyped cases of lethal KID syndrome. We performed new molecular modeling of the lethal mutants GJB2 p.A88V and GJB2 p.G45E. RESULTS: Infant death occurred in all patients with GJB2 p.G45E and p.A88V; it is unusual with other GJB2 mutations. Early death with those 2 "lethal" mutations is likely multifactorial: during life all had ≥1 serious infection; most had poor weight gain and severe respiratory difficulties; many had additional anatomic abnormalities. Structural modeling of GJB2 p.G45E identified no impact on the salt bridge previously predicted to account for abnormal central carbon dioxide sensing of GJB2 p.A88V. LIMITATIONS: This clinical review was retrospective. CONCLUSION: GJB2 p.G45E and p.A88V are the only KID syndrome mutations associated with uniform early lethality. Those electrophysiologically severe mutations in GJB2 reveal abnormalities in many organs in lethal KID syndrome. All patients with KID syndrome may have subtle abnormalities beyond the eyes, ears, and skin. Early genotyping of KID syndrome births will inform prognostic discussion.

The Development of Indicator Cotton Swabs for the Detection of pH in Wounds.

Indicator cotton swabs have been developed in order to enable faster, less expensive, and simpler information gathering of a wound status. Swabs are normally used for cleaning the wound, but here, they were covalently functionalized with indicator chemistry. Thus, they in principle enable simultaneous wound cleaning and wound pH detection. Using an indicator dye with a color change from yellow to red, combined with an inert dye of blue color, a traffic light color change from green to red is induced when pH increases. The indicator cotton swabs (ICSs) show a color change from green (appropriate wound pH) to red (elevated wound pH). This color change can be interpreted by the naked eye as well as by an optical color measurement device in order to obtain quantitative data based on the CIE L*a*b* color space. Two types of swabs have been developed-indicator cotton swabs ICS1 with a sensitive range from pH 5 to 7 and swabs ICS2 with a sensitive range from 6.5 to 8.5. The swabs are gamma-sterilized and the effect of sterilization on performance was found to be negligible. Furthermore, cytotoxicity testing shows cell viability and endotoxin levels to be within the allowable range.

[Differential diagnosis of skin changes on the lower extremities in chronic venous insufficiency]. / Differentialdiagnose der Hautveränderungen an den Beinen bei CVI.

Varicous veins and postthrombotic syndrome can make typical reversible or irreversible skin changes on the lower extremities if no treatment is initiated. The typical clinical signs should be recognised in an early stage and possible differential diagnoses have to be excluded.

A Standardized Treatment Pathway for Telehealth-Based Care of Chronic Wounds in Austria.

Chronic wounds present a significant healthcare challenge in Austria as well as in other countries. The interdisciplinary approach to wound treatment involving various caregivers, doctors, and relatives, poses challenges in documentation and information exchange. To overcome these barriers and promote patient-centered care, a new telehealth-supported treatment pathway for chronic wounds has been developed. The primary focus was to regularly update the status of the chronic wound by responding to predefined questions and transmitted images of the chronic wound. This was achieved by an interdisciplinary team of experts in chronic wound care, providing a new perspective for digital implementation in the healthcare system.

Development of a cellular assay as a personalized model for testing chronic wound therapeutics.

Exudates of non-healing wounds contain drivers of pathogenicity. We utilized >800 exudates from non-healing and healing wounds of diverse etiologies, collected by three different methods, to develop a wound-specific, cell-based functional biomarker assay. Human dermal fibroblast proliferation served as readout to a) to differentiate between healing and non-healing wounds, b) follow the healing process of individual patients, and c) assess the effects of therapeutics for chronic wounds ex vivo. We observed a strong correlation between wound chronicity and inhibitory effects of individual exudates on fibroblast proliferation, with good diagnostic sensitivity (76-90%, depending on the sample collection method). Transition of a clinically non-healing to a healing phenotype restored fibroblast proliferation and extracellular matrix formation while reducing inflammatory cytokine production. Transcriptional analysis of fibroblasts exposed to ex vivo non-healing wound exudates revealed an induction of inflammatory cytokine- and chemokine pathways and the unfolded protein response, indicating that these changes may contribute to the pathology of non-healing wounds. Testing the wound therapeutics platelet derived growth factor and silver sulfadiazine yielded responses in line with clinical experience and indicate the usefulness of the assay to search for and profile new therapeutics.

Novel protease-based diagnostic devices for detection of wound infection.

A gelatinase-based device for fast detection of wound infection was developed. Collective gelatinolytic activity in infected wounds was 23 times higher (p ≤ 0.001) than in noninfected wounds and blisters according to the clinical and microbiological description of the wounds. Enzyme activities of critical wounds showed 12-fold elevated enzyme activities compared with noninfected wounds and blisters. Upon incubation of gelatin-based devices with infected wound fluids, an incubation time of 30 minutes led to a clearly visible dye release. A 32-fold color increase was measured after 60 minutes. Both matrix metalloproteinases and elastases contributed to collective gelatinolytic enzyme activity as shown by zymography and inhibition experiments. The metalloproteinase inhibitor 1,10-phenanthroline (targeting matrix metalloproteinases) and the serine protease inhibitor phenylmethlysulfonyl fluoride (targeting human neutrophil elastase) inhibited gelatinolytic activity in infected wound fluid samples by 11-37% and 60-95%, respectively. Staphylococcus aureus and Pseudomonas aeruginosa, both known for gelatinase production, were isolated in infected wound samples.

[Treatment of chronic wounds]. / Behandlung chronischer Wunden.

The treatment of chronic wounds often poses a therapeutic challenge. Assessment of the pathogenesis of the wound, causative and symptomatic interventions are key. Concerning treatment-concepts the evaluation of comorbidities, pain management, the nutrition status of as well as the mobility of the patient are recommended.

Neonatal lupus erythematosus and its clinical variability.

Neonatal lupus erythematosus (NLE) is a rare disease affecting newborns that is caused by maternal autoantibodies transmitted across the placenta. The disease may affect the skin, the heart, and rarely the hepatobiliary or hematological systems. A serious complication affecting some patients with NLE is atrioventricular heart block (AV block). The clinical picture of cutaneous NLE varies considerably. NLE presents with confluent, scaly, periorbital erythema, or erythematous infiltrated plaques with central vesicles and lesions resembling seborrheic eczema or fungal infection. In any newborn with such skin lesions, NLE should be included in the differential diagnosis. Dermatologists play an important role in the diagnosis. We review different skin lesions occurring in neonatal lupus erythematosus based on five patients from our own clinic.

[Uncommon presentation of psoriasis vulgaris in an 11-year-old patient]. / Ungewöhnliche Präsentation einer Psoriasis vulgaris bei einem 11-jährigen Patienten.

Psoriasis vulgaris occurs with a prevalence of up to 2% in childhood and adolescence. In most cases, diagnosis can be made clinically. We report about a pediatric patient with psoriasis vulgaris who posed a diagnostic challenge due to uncharacteristic manifestation. The most important differential diagnoses of various forms of psoriasis vulgaris as well as actual treatment recommendations in childhood and adolescence are discussed.

Gadofosveset-Trinatrium-Enhanced MR Angiography and MR Venography in the Diagnosis of Venous Thromboembolic Disease: A Single-Center Cohort Study.

BACKGROUND: The aim of this single-center combined prospective/retrospective cohort study was to analyze Gadolinium (Gd)-enhanced MRA (magnetic resonance angiography) and MRV (MR venography) for the diagnosis of pulmonary artery embolism and deep venous thrombosis. The gold standard methods result in major exposure to radiation and a high amount of nephrotoxic iodinated contrast media. This is the first larger contrast-enhanced MR imaging study of acute and chronic venous thromboembolic disease of various stages. METHODS: We prospectively examined 88 patients presenting clinical signs of deep vein thrombosis and/or pulmonary artery embolism. A single-session, one-stop shop Gd-enhanced MRA/MRV at 1.5 Tesla, using gradient echo sequences with very short repetition and echo times as well as low flip angles with subtraction and three-dimensional reconstruction, was performed. A diagnosis was made with the consensus of two experienced radiologists. RESULTS: We observed excellent MRA image quality in 87% and even higher diagnostic image quality of MRV in 90% of our examinations. Pulmonary artery embolism occurred with deep vein thrombosis in 22%. CONCLUSIONS: Gd-enhanced MRA/MRV provided excellent image quality for the diagnosis of venous thromboembolic disease in the majority of cases. It may be particularly useful to plan and follow-up filter implantation and retrieval in the inferior caval vein.

Sensor materials for the detection of human neutrophil elastase and cathepsin G activity in wound fluid.

Human neutrophil elastase (HNE) and cathepsin G (CatG) are involved in the pathogenesis of a number of inflammatory disorders. These serine proteinases are released by neutrophils and monocytes in case of infection. Wound infection is a severe complication regarding wound healing causing diagnostic and therapeutic problems. In this study we have shown the potential of HNE and CatG to be used as markers for early detection of infection. Significant differences in HNE and CatG levels in infected and non-infected wound fluids were observed. Peptide substrates for these two enzymes were successfully immobilised on different surfaces, including collagen, modified collagen, polyamide polyesters and silica gel. HNE and CatG activities were monitored directly in wound fluid via hydrolysis of the chromogenic substrates. Infected wound fluids led to significant higher substrate hydrolysis compared with non-infected ones. These different approaches could be used for the development of devices which are able to detect elevated enzyme activities before manifestation of infection directly on bandages. This would allow a timely intervention by medical doctors thus preventing severe infections.

Prevalence of tinea capitis in Southeastern Austria between 1985 and 2008: up-to-date picture of the current situation.

Tinea capitis is the most common dermatophyte infection in childhood, but may rarely occur in adults and the elderly. Causative agents vary within different geographical areas as well as during decades. The aim of this study was to evaluate the prevalence and causative agents of tinea capitis in Southeastern Austria. Retrospective analysis of 714 patients diagnosed with tinea capitis seen at the outpatient Department of Dermatology/Medical University of Graz during the time period 1985-2008 was carried out. A total of 517 of the 714 patients were children, 21 adults and in the case of 176 patients age was not available. Microsporum canis was found in 84.4%. Trichophyton soudanense tinea capitis is seen since 1998, Trichophyton tonsurans for the first time in 2008. Tinea capitis has become very important for the public health. Besides an increasing incidence, there is a change in age of the patients affected and with the pattern of causative agents as a result of immigration movements and lifestyle habits mainly influenced by domestic pets. Our situation reflects nearly the epidemiology of the bordering countries of Austria mainly in the Southeastern surroundings. These epidemiological changes are a challenge for general practitioners, dermatologists and veterinarians to work close together for advice on control, early diagnosing and adequate treatment.

Post-severe Acute Respiratory Syndrome Coronavirus 2 Monoclonal Antibody Treatment Hospitalizations as a Sentinel for Emergence of Viral Variants in New York City.

We partnered with the US Department of Health and Human Services to treat high-risk, nonadmitted coronavirus disease 2019 (COVID-19) patients with bamlanivimab in the Bronx, New York per Emergency Use Authorization criteria. Increasing posttreatment hospitalizations were observed monthly between December 2020 and March 2021 in parallel to the emergence of severe acute respiratory syndrome coronavirus 2 variants in New York City.