S2k guidelines on the management of paraneoplastic pemphigus/paraneoplastic autoimmune multiorgan syndrome initiated by the European Academy of Dermatology and Venereology (EADV).

BACKGROUND Paraneoplastic pemphigus (PNP), also called paraneoplastic autoimmune multiorgan syndrome (PAMS), is a rare autoimmune disease with mucocutaneous and multi-organ involvement. PNP/PAMS is typically associated with lymphoproliferative or haematological malignancies, and less frequently with solid malignancies. The mortality rate of PNP/PAMS is elevated owing to the increased risk of severe infections and disease-associated complications, such as bronchiolitis obliterans. OBJECTIVES These guidelines summarize evidence-based and expert-based recommendations (S2k level) for the clinical characterization, diagnosis and management of PNP/PAMS. They have been initiated by the Task Force Autoimmune Blistering Diseases of the European Academy of Dermatology and Venereology with the contribution of physicians from all relevant disciplines. The degree of consent among all task force members was included. RESULTS Chronic severe mucositis and polymorphic skin lesions are clue clinical characteristics of PNP/PAMS. A complete assessment of the patient with suspected PNP/PAMS, requiring histopathological study and immunopathological investigations, including direct and indirect immunofluorescence, ELISA and, where available, immunoblotting/immunoprecipitation, is recommended to achieve a diagnosis of PNP/PAMS. Detection of anti-envoplakin antibodies and/or circulating antibodies binding to the rat bladder epithelium at indirect immunofluorescence is the most specific tool for the diagnosis of PNP/PAMS in a patient with compatible clinical and anamnestic features. Treatment of PNP/PAMS is highly challenging. Systemic steroids up to 1.5 mg/kg/day are recommended as first-line option. Rituximab is also recommended in patients with PNP/PAMS secondary to lymphoproliferative conditions but might also be considered in cases of PNP/PAMS associated with solid tumours. A multidisciplinary approach involving pneumologists, ophthalmologists and onco-haematologists is recommended for optimal management of the patients. CONCLUSIONS These are the first European guidelines for the diagnosis and management of PNP/PAMS. Diagnostic criteria and therapeutic recommendations will require further validation by prospective studies

A Glance at the Practice of Pediatric Teledermatology Pre- and Post–COVID-19: Narrative Review

BackgroundThe COVID-19 pandemic has accelerated the use of pediatric teledermatology, with centers showing increased uptake of teledermatology. Pediatric patients possess unique characteristics that pose different challenges with teledermatology compared to adults, in turn affecting the feasibility and uptake of pediatric teledermatology in the community. ObjectiveThis narrative review summarizes the evolution of pediatric teledermatology from pre–COVID-19 pandemic times to the post–COVID-19 period. MethodsA search of MEDLINE, PubMed, and Embase was performed for original articles written in English and published from December 1, 2019, to April 1, 2022. ResultsA total of 22 publications were included. ConclusionsTeledermatology will continue to play an increasing role in the management of skin diseases. A mindset shift in the types of conditions deemed suitable for pediatric teledermatology is needed

Two Cases of Dyskeratosis Congenita with Clinically Distinct Presentations, Seen in National University Hospital, Singapore

10.1159/000518299Skin Appendage Disorder

Teledermatology in an emergency department: benefits and gaps

10.1186/s12873-023-00854-2BMC EMERGENCY MEDICINE23

Ulcerated yellow-brown nodules on the chest

10.1016/j.jdcr.2021.05.040JAAD Case Reports1427-2

Cutaneous angiosarcoma of the scalp mimicking a keratoacanthoma

Cutaneous angiosarcoma (CA) has a wide range of clinical presentations. In this case report, we discuss a 78-year-old gentleman, who presented with a keratoacanthoma-like scalp lesion that turned out histologically to be a cutaneous angiosarcoma. A brief overview of CA, including its etiology, prognostic factors, clinical manifestations, and treatment options will also be discussed