The importance of acetylcholine (ACh) in keratinocyte adhesion and acantholysis has been investigated over the last three decades, particularly in the pathophysiology of autoimmune blistering dermatoses. Pemphigus vulgaris (PV) is an autoimmune blistering skin disease where autoantibody-mediated suprabasilar intraepidermal splitting causes flaccid blisters and non-healing erosions of the oral mucosa and sometimes also of the skin. Historically, acantholysis in PV was thought to be driven by anti-desmoglein (Dsg) antibodies. Herein, we describe the role of autoantibodies against keratinocyte muscarinic and nicotinic acetylcholine receptors, as well as the annexin-like molecule pemphaxin that also binds ACh, in the immunopathogenesis of PV. The identification of targets in this disease is important, as they may lead to novel diagnostic and therapeutic options in the future for this potentially deadly disease.
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http://dx.doi.org/10.3390/biology12030354 | DOI Listing |
Cureus
November 2024
Dermatology, Amiri Hospital, Ministry of Health, Kuwait City, KWT.
Pemphigus foliaceus (PF) is an autoimmune blistering disease characterized by the disruption of the epidermal cell adhesion protein desmoglein 1 (DsG1). PF classically presents with superficial erosions or blisters, but can rarely mimic other dermatological conditions, which makes diagnosis challenging. We report the case of a 57-year-old Sri Lankan man with a one-month history of widespread ichthyosis-like plaques and scales which started on his scalp and progressed in a cranio-caudal fashion and were associated with pruritus and few blisters.
View Article and Find Full Text PDFSAGE Open Med Case Rep
December 2024
Division of Dermatology, Department of Medicine, University of Alberta, Edmonton, AB, Canada.
Childhood linear immunoglobulin A bullous dermatosis is a well-recognized autoimmune blistering dermatosis that can be idiopathic, drug-induced, secondary to autoimmune diseases, malignancies, infections, or gastrointestinal diseases including inflammatory bowel disease. However, it has not been previously associated with a history of organ transplantation. Here, we report two cases of childhood linear immunoglobulin A bullous dermatosis in young infants following multivisceral organ transplant, including one with a particularly recalcitrant course.
View Article and Find Full Text PDFAntibodies (Basel)
December 2024
Autoimmune Blistering Dermatoses Section, Department of Dermatology, Poznan University of Medical Sciences, 61701 Poznan, Poland.
Background/objectives: Pemphigus comprises a diverse group of disorders within the autoimmune bullous dermatoses (AIBDs) spectrum. Among these, pemphigus vulgaris (PV) and pemphigus foliaceus (PF) are the most commonly encountered variants. Despite its rarity, this condition can pose a life-threatening risk.
View Article and Find Full Text PDFCureus
November 2024
Internal Medicine, Larkin Community Hospital Palm Springs Campus, Hialeah, USA.
Pemphigus vulgaris (PV) is a rare autoimmune disorder characterized by mucocutaneous blistering due to autoantibodies targeting desmoglein proteins, leading to acantholysis. This case report presents a 60-year-old Hispanic male patient with a history of hypertension who developed PV, initially presenting with pruritic scalp lesions that progressively spread to the nares, mouth, chest, neck, and inguinal region. Despite initial management with topical treatments, the lesions persisted, prompting hospitalization.
View Article and Find Full Text PDFCureus
November 2024
Rheumatology, Cooper University Health Care, Camden, USA.
Drug-induced lupus erythematosus (DILE) is an autoimmune reaction that results in symptoms of polyarthralgia, fever, and cutaneous lesions and other manifestations. Several drugs have been documented to cause this disease, including procainamide, isoniazid, methyldopa, penicillamine, and hydralazine. Systemic lupus erythematosus (SLE) manifestations often occur after the patient has been taking the drug without complications for months to years.
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