Background: Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are rare and potentially life-threatening mucocutaneous blistering diseases that clinically can resemble autoimmune bullous diseases. Moreover, it has been shown that autoantibodies against epidermal proteins are present in SJS/TEN.
Objectives: To establish the presence of antibodies against desmosomal and hemidesmosomal proteins in confirmed SJS/TEN patients.
J Eur Acad Dermatol Venereol
January 2023
Background: Paraneoplastic pemphigus (PNP) is an extremely rare life-threatening blistering autoimmune disease that is associated with an underlying neoplasm. There is a set diagnostic criterion for PNP, which is primarily based on a severe stomatitis and the detection of specific antibodies against envoplakin, periplakin and alpha-2-macroglobulin-like protein 1. However, it has become increasingly evident that there are patients with PNP that do not meet all the diagnostic criteria requirements.
View Article and Find Full Text PDFBackground: The variable clinical severity of mucous membrane pemphigoid (MMP) often leads to diagnostic and therapeutic delays.
Objective: To describe the characteristics of a large cohort of patients with MMP.
Methods: A retrospective review of clinical and diagnostic characteristics as well as treatment responses in 145 patients with MMP.
The u-serrated immunodeposition pattern in direct immunofluorescence (DIF) microscopy is a recognizable feature and confirmative for the diagnosis of epidermolysis bullosa acquisita (EBA). Due to unfamiliarity with serrated patterns, serration pattern recognition is still of limited use in routine DIF microscopy. The objective of this study was to investigate the feasibility of using convolutional neural networks (CNNs) for the recognition of u-serrated patterns that can assist in the diagnosis of EBA.
View Article and Find Full Text PDFImportance: An accurate diagnosis of mucous membrane pemphigoid (MMP) is essential to reduce diagnostic and therapeutic delay.
Objective: To assess the diagnostic accuracy of direct immunofluorescence microscopy on mucosal biopsy specimens and immunoserology in a large cohort of patients with suspected MMP.
Design, Setting, And Participants: This retrospective cohort study was carried out in a single tertiary care center for blistering diseases between January 2002 and March 2019.
This cross-sectional study of 125 residents of 7 nursing homes in the Netherlands evaluates the prevalence of pemphigoid as a potentially unrecognized cause of pruritus.
View Article and Find Full Text PDFAutoimmune bullous diseases are a group of chronic inflammatory disorders caused by autoantibodies targeted against structural proteins of the desmosomal and hemidesmosomal plaques in the skin and mucosa, leading to intra-epithelial or subepithelial blistering. The oral mucosa is frequently affected in these diseases, in particular, in mucous membrane pemphigoid, pemphigus vulgaris, and paraneoplastic pemphigus. The clinical symptoms are heterogeneous and may present with erythema, blisters, erosions, and ulcers localized anywhere on the oral mucosa, and lead to severe complaints for the patients including pain, dysphagia, and foetor.
View Article and Find Full Text PDFBackground: Nonbullous pemphigoid is an under-recognized phenotype of the autoimmune bullous disease pemphigoid, characterized by the absence of blisters. Several disease aspects have not been studied previously.
Objective: To describe the characteristics of nonbullous pemphigoid.
Importance: A substantial number of patients with bullous pemphigoid do not develop skin blisters and may not have received the correct diagnosis. Diagnostic criteria and an optimal diagnostic strategy are needed for early recognition and trials.
Objectives: To assess the minimal requirements for diagnosis of bullous and nonbullous forms of pemphigoid and to evaluate the optimal diagnostic strategy.
Direct immunofluorescence (DIF) microscopy of a skin biopsy is used by physicians and pathologists to diagnose autoimmune bullous dermatoses (AIBD). This technique is the reference standard for diagnosis of AIBD, which is used worldwide in medical laboratories. For diagnosis of subepidermal AIBD (sAIBD), two different types of serrated pattern of immunodepositions can be recognized from DIF images, namely n- and u-serrated patterns.
View Article and Find Full Text PDFPruritus is the most common dermatological complaint in elderly people and may have a significant negative influence on quality of life. In elderly, the identification of the underlying cause of pruritus can be difficult, due to the broad differential diagnosis and the frequent occurence of comorbidities and polypharmacy. In daily practice, a classification can be used of 'pruritus with primary skin lesions' and 'pruritus without primary skin lesions' for a more specific search to the underlying cause.
View Article and Find Full Text PDFBackground: Direct immunofluorescence (DIF) microscopy of a skin biopsy specimen is the reference standard for the diagnosis of pemphigoid diseases (PDs). Serration pattern analysis enables the differentiation of epidermolysis bullosa acquisita (EBA) from other PDs using DIF microscopy alone. However, practice gaps need to be addressed in order to implement this technique in the routine diagnostic procedure.
View Article and Find Full Text PDFBackground: Bullous pemphigoid is an autoimmune disease that typically presents with tense bullae and severe pruritus. However, bullae can be lacking, a subtype termed nonbullous pemphigoid.
Objective: To summarize the reported characteristics of nonbullous pemphigoid.
Background: In 2015 and 2016, female patients in Flanders consulted a dermatologist because they developed skin lesions after wearing a specific brand of canvas shoes.
Objectives: To identify the culprit allergen in the shoes.
Methods: Eighteen young females aged 14-22 years presented with itching and erythematous to purple-coloured eczematous lesions on both feet.
Background: During rubber vulcanization, new compounds can be formed.
Objectives: To report a case of allergic shoe dermatitis in which the search for the allergen ultimately led to the identification of dimethylthiocarbamylbenzothiazole sulfide (DMTBS).
Methods: A female presented with eczema on her feet after wearing Sperry Top Sider® canvas sneakers.
Importance: Anti-p200 pemphigoid is a rare subepidermal autoimmune blistering disease characterized by autoantibodies against a 200-kDa protein in the basement membrane zone. Anti-p200 pemphigoid is probably often misdiagnosed because of low availability of diagnostic assays and expertise and classified as bullous pemphigoid or epidermolysis bullosa acquisita.
Objective: To clinically characterize patients with anti-p200 pemphigoid, identified by using indirect immunofluorescence microscopy on skin substrates deficient in type VII collagen and laminin-332 (knockout analysis), to validate this technique by immunoblot with dermal extract, and to incorporate direct immunofluorescence serration pattern analysis in the diagnostic algorithm.
Purpose: To present a case in which a covered stent fractured 7 months after implantation in the internal carotid artery (ICA).
Case Report: A 59-year-old man presented with a large traumatic pseudoaneurysm of the left ICA. Seven months after successful treatment with a covered Symbiot stent, clinical complaints recurred because the pseudoaneurysm recanalized due to fracture of the stent at its midsection.