Society of Dermatology Hospitalists supportive care guidelines for the management of Stevens-Johnson syndrome/toxic epidermal necrolysis in adults

Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are life-threatening conditions with high morbidity and mortality. Supportive care management of SJS/TEN is highly variable. A systematic review of the literature was performed by dermatologists, ophthalmologists, intensivists, and gynecologists with expertise in SJS/TEN to generate statements for supportive care guideline development. Members of the Society of Dermatology Hospitalists with expertise in SJS/TEN were invited to participate in a modified, online Delphi-consensus. Participants were administered 9-point Likert scale questionnaires regarding 135 statements. The RAND/UCLA Appropriateness Method was used to evaluate and select proposed statements for guideline inclusion; statements with median ratings of 6.5 to 9 and a disagreement index of ≤1 were included in the guideline. For the final round, the guidelines were appraised by all of the participants. Included are an evidence-based discussion and recommendations for hospital setting and care team, wound care, ocular care, oral care, urogenital care, pain management, infection surveillance, fluid and electrolyte management, nutrition and stress ulcer prophylaxis, airway management, and anticoagulation in adult patients with SJS/TEN

Cell-free DNA from nail clippings as source of normal control for genomic studies in hematologic malignancies

Comprehensive genomic sequencing is becoming a critical component in the assessment of hematologic malignancies, with broad implications for patient management. In this context, unequivocally discriminating somatic from germline events is challenging but greatly facilitated by matched analysis of tumor:normal pairs. In contrast to solid tumors, conventional sources of normal control (peripheral blood, buccal swabs, saliva) could be highly involved by the neoplastic process, rendering them unsuitable. In this work we describe our real-world experience using cell free DNA (cfDNA) isolated from nail clippings as an alternate source of normal control, through the dedicated review of 2,610 tumor:nail pairs comprehensively sequenced by MSK-IMPACT-heme. Overall, we find nail cfDNA is a robust source of germline control for paired genomic studies. In a subset of patients, nail DNA may have tumor DNA contamination, reflecting unique attributes of the hematologic disease and transplant history. Contamination is generally low level, but significantly more common among patients with myeloid neoplasms (20.5%; 304/1482) compared to lymphoid diseases (5.4%; 61/1128) and particularly enriched in myeloproliferative neoplasms with marked myelofibrosis. When identified in patients with lymphoid and plasma-cell neoplasms, mutations commonly reflected a myeloid profile and correlated with a concurrent/evolving clonal myeloid neoplasm. For nails collected after allogeneic stem-cell transplantation, donor DNA was identified in 22% (11/50). In this cohort, an association with recent history of graft-vs-host disease was identified. These findings should be considered as a potential limitation for the use of nail as normal control but could also provide important diagnostic information regarding the disease process

Updates in SJS/TEN: collaboration, innovation, and community

Stevens-Johnson Syndrome/Toxic Epidermal Necrolysis (SJS/TEN) is a predominantly drug-induced disease, with a mortality rate of 15–20%, that engages the expertise of multiple disciplines: dermatology, allergy, immunology, clinical pharmacology, burn surgery, ophthalmology, urogynecology, and psychiatry. SJS/TEN has an incidence of 1–5/million persons per year in the United States, with even higher rates globally. One of the challenges of SJS/TEN has been developing the research infrastructure and coordination to answer questions capable of transforming clinical care and leading to improved patient outcomes. SJS/TEN 2021, the third research meeting of its kind, was held as a virtual meeting on August 28–29, 2021. The meeting brought together 428 international scientists, in addition to a community of 140 SJS/TEN survivors and family members. The goal of the meeting was to brainstorm strategies to support the continued growth of an international SJS/TEN research network, bridging science and the community. The community workshop section of the meeting focused on eight primary themes: mental health, eye care, SJS/TEN in children, non-drug induced SJS/TEN, long-term health complications, new advances in mechanisms and basic science, managing long-term scarring, considerations for skin of color, and COVID-19 vaccines. The meeting featured several important updates and identified areas of unmet research and clinical need that will be highlighted in this white paper

Apartment House Heating with Renewable Energy

Představená bakalářská práce se zabývá vytápěním bytového domu. Závěrečná práce se skládá ze dvou částí — projektové části a rozšiřující části. Cílem projektové části je návrh optimálního řešení vytápění zadaného bytového domu. Součástí projektové části jsou výpočty potřebné k návrhu otopného systému, technické listy od výrobců jednotlivých zařízení, technická zpráva a výkresy v podrobnosti dokumentace pro provedení stavby. Rozšiřující část se zabývá zdroji energie, popisem obnovitelných a neobnovitelných zdrojů energie, které lze využit při návrhu systémů vytápění. Dále v rozšiřující části je představen návrh vytápění pomocí alternativního zdroje energie.The presented bachelor thesis deals with the heating of an apartment building. The final work consists of two parts — the project part and the extension part. The aim of the project part is to design an optimal solution for heating a given apartment building. The project part includes calculations needed to design the heating system, technical sheets from the manufacturers of individual devices, technical report and drawings in the details of the documentation for the construction. The extension part deals with energy sources, description of renewable and non-renewable energy sources that can be used in the design of heating systems. Furthermore, in the extension part is presented the design of heating using an alternative energy source

The Theoretical Aspects of the Security of Human Development

The article is aimed at substantiating the basic categories of the conception of security of human development and formation of a definition of the concept of «security of human development» that would provide the most comprehensive understanding of the nature of this process. The main approaches to researching the phenomenon of «security» were analyzed. Characteristics of the formation of human security as part of a holistic paradigm of human development were considered together with rethinking it as a new theory of global security. A cross-categorical analysis of the concepts of «human development» and of «human security» was carried out by the main criteria: characteristics, time frames, main purpose and objectives. As a result, the human security is complementary to the concept of human development in the part of safe variants of choice (freedom from needs); it further facilitated the security provision at the level of prosperity and progress, not at the level of survival. It has been determined that the conception of human security is at the same time an element of the conception of human development as well as of human security

Diagnosis of Common Dermopathies in Dialysis Patients: A Review and Update

Cutaneous abnormalities in patients with end-stage renal disease (ESRD) receiving hemodialysis or peritoneal dialysis may demonstrate signs of their underlying condition or reveal associated disease entities. While a thorough examination of the scalp, skin, mucosa, and nails is integral to establishing a diagnosis, certain conditions will resolve only with dialysis or improvement of their renal disease and others may not require or respond to treatment. Half and half nails, pruritus, xerosis, and cutaneous hyperpigmentation are common manifestations in ESRD. With hemodialysis, uremic frost is no longer prevalent in ESRD patients and ecchymoses have decreased in incidence. Acquired perforating dermatoses are seen in over one-tenth of hemodialysis patients. Metastatic calcinosis cutis and calciphylaxis are both rarely reported, although the latter is seen almost exclusively in the setting of hemodialysis. Diagnosis of nephrogenic systemic fibrosis has historically been challenging; as such, new diagnostic criteria have been proposed. Blood porphyrin profiles are needed to differentiate between porphyria cutanea tarda and pseudoporphyria. We will review and provide an update on the aforementioned common cutaneous manifestations of ESRD in patients receiving dialysis

Disseminated punctate keratoderma: a rare case report and review of the literature

We report a rare case of a 53-year-old womanpresenting with diffuse, late-onset disseminatedhyperkeratotic papules. Biopsy showed massivehyperkeratosis overlying a crateriform epidermaldepression and hypergranulosis with mild epidermalhyperplasia. There was no parakeratosis, cornoidlamella, or dyskeratosis. Based on the clinical findingsand histopathological features, a diagnosis ofdisseminated punctate keratoderma was made. Thisis a rare subtype of palmoplantar keratoderma, whichhas a putative increased risk of malignancy. This casereport emphasizes the importance of identifyingthe clinical and histological presentation of this rarecondition; referral of the patient for age-appropriatemalignancy screening is appropriate. We also presenta concise review of treatment options

Disseminated punctate keratoderma: a rare case report and review of the literature

We report a rare case of a 53-year-old womanpresenting with diffuse, late-onset disseminatedhyperkeratotic papules. Biopsy showed massivehyperkeratosis overlying a crateriform epidermaldepression and hypergranulosis with mild epidermalhyperplasia. There was no parakeratosis, cornoidlamella, or dyskeratosis. Based on the clinical findingsand histopathological features, a diagnosis ofdisseminated punctate keratoderma was made. Thisis a rare subtype of palmoplantar keratoderma, whichhas a putative increased risk of malignancy. This casereport emphasizes the importance of identifyingthe clinical and histological presentation of this rarecondition; referral of the patient for age-appropriatemalignancy screening is appropriate. We also presenta concise review of treatment options

Ponatinib-induced ichthyosiform drug eruption: insights into acquired ichthyosis

Cutaneous adverse events are commonly experienced with use of tyrosine kinase inhibitors in the treatment of leukemia and typically include nonspecific cutaneous eruptions and xerosis. We report the case of a man who experienced an ichthyosiform drug eruption while taking ponatinib, a third-generation tyrosine kinase inhibitor. Disruption of epidermal growth pathways through inhibition of various receptor tyrosine kinases by ponatinib may offer insights into the pathophysiologic mechanisms behind acquired ichthyosis

Ustekinumab for treatment of cutaneous Crohn’s disease

Cutaneous Crohn disease (CD) affecting the vulva, perineum, and perianal skin, is a rare entity, which may accompany or precede gastrointestinal CD. Vulvar involvement, if untreated, may ultimately require extensive surgery including vulvectomy to gain control of the disease. Both gastrointestinal and cutaneous CD respond to biologics, which block TNF. In addition, ustekinumab, which targets both IL-12 and IL-23 cytokines, is effective in patients with gastrointestinal CD who fail TNF blockade. However, it is unclear if ustekinumab is effective for cutaneous CD. Herein we present a patient with cutaneous CD affecting the vulva and perianal skin, which, at seven months, had a marked response to ustekinumab administered at higher doses than typically used for psoriasis