Bullous dermatoses are commonly encountered in childhood practice. Much confusion exists among clinicians because of the similarity of clinical lesions, paucity of relevant literature, and varied nomenclature used for these diseases. For a better understanding of the proposed classification, this second part is a review of diseases with nonhereditary characteristics of subepidermal cleavage and hereditary characteristics with intraepidermal and subepidermal cleavage.
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http://dx.doi.org/10.1111/j.1540-9740.2007.05958.x | DOI Listing |
Arch Dermatol Res
January 2025
Department of Dermatology, State Key Laboratory of Complex Severe and Rare Diseases, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, National Clinical Research Center for Dermatologic and Immunologic Diseases, 9 Dongdan 3rd Alley, Beijing, 100730, China.
Bullous pemphigoid (BP) is a debilitating autoimmune skin blistering disease, characterized by the deposition of specific autoantibodies at the dermal-epidermal junction. This leads to an inflammatory cascade involving the activation of complement proteins, mast cell degranulation, immune cell recruitment, and the release of proteases by granulocytes. While several cytokines and signaling pathways have been implicated in the pathogenesis of BP, the precise mechanism behind autoantibody production remains unclear.
View Article and Find Full Text PDFAm J Dermatopathol
February 2025
Department of Dermatology and Venereology, Faculty of Medicine, Medical University of Plovdiv, Plovdiv, Bulgaria.
Pemphigus is a group of autoimmune bullous diseases mediated by autoantibodies most often of the immunoglobulin G class, subclasses immunoglobulin G1, and immunoglobulin G4 (IgG4), directed against desmosomal adhesion proteins of keratinocytes. This study aimed to evaluate IgG4 immunoreactivity on paraffin sections using immunohistochemistry in patients with pemphigus as a diagnostic test. Fifty formalin-fixed paraffin-embedded specimens from patients with pemphigus were selected.
View Article and Find Full Text PDFJ Dermatolog Treat
December 2025
Hospital for Skin Diseases, Chinese Academy of Medical Sciences and Peking Union Medical College, Nanjing, Jiangsu, China.
Background: Hailey-Hailey disease (HHD), a genetic blistering disease, is caused by a mutation in a calcium transporter protein in the Golgi apparatus encoded by the gene. Clinically, HHD is characterized by flaccid vesicles, blisters, erosions, fissures, and maceration mainly in intertriginous regions. Some patients remain refractory to conventional treatments.
View Article and Find Full Text PDFJ Cell Sci
January 2025
Institute of Anatomy and Experimental Morphology, Center for Experimental Medicine, University Cancer Center Hamburg, University Medical Center Hamburg-Eppendorf, 20246 Hamburg, Germany.
Desmosomes are adhesive cell contacts abundant in tissues exposed to mechanical strain, such as the stratified and simple epithelia of the epidermis and mucous membranes, as well as the myocardium. Besides their role in mechanical cell cohesion, desmosomes also modulate pathways important for tissue differentiation, wound healing and immune responses. Dysfunctional desmosomes, resulting from pathogenic variants in genes encoding desmosomal components, autoantibodies targeting desmosomal adhesion molecules or inflammation, cause the life-threatening diseases arrhythmogenic cardiomyopathy and pemphigus and contribute to the pathogenesis of inflammatory bowel diseases.
View Article and Find Full Text PDFMedicine (Baltimore)
January 2025
Department of Rehabilitation Medicine, Hebei General Hospital, Shijiazhuang, Hebei, China.
Rationale: Steven-Johnson syndrome (SJS) is characterized by severe illness, rapid progression, and high mortality rates, with the vast majority of cases induced by medications. Botulinum toxin, a neurotoxin produced by Clostridium botulinum, has not been reported in the literature as a causative agent of SJS.
Patient Concerns: A 56-year-old male patient, who underwent surgery for cerebral hemorrhage, developed widespread patchy annular papules following the injection of botulinum toxin into the masseter muscle.
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