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Blistering skin disorders
Published in Rashmi Sarkar, Anupam Das, Sumit Sethi, Concise Dermatology, 2021
Pemphigus is a group of blistering disorders, which is characterized by acantholysis, i.e. loss of keratinocyte to keratinocyte adhesion, which results in the formation of epidermal blisters in mucosae and skin. Acantholysis is induced by autoantibodies against intercellular adhesion molecules. There are several types (Table 6.5), out of which pemphigus vulgaris (PV) is the most common. The average age of onset is 40 to 60 years. The lesions are thin-walled, delicate blisters that usually rapidly rupture and erode (Figure 6.3). They occur anywhere on the skin surface and very frequently occur within the mouth and throat, where they cause much discomfort and disability. The disorder is persistent, although fluctuating in intensity. Before adequate treatment became available, it was usually fatal. Pemphigus foliaceus is another variant where blistering occurs in the superficial layers of the epidermis. Clinically, blisters are seen rarely and erosions with crusting are seen mainly over the scalp, face, and seborrheic areas. It can sometimes become generalized and the patient may go into erythroderma. Though a chronic disease, its course is more benign than pemphigus vulgaris.
Skin diseases of the elderly
Published in Robert A. Norman, Geriatric Dermatology, 2020
Pemphigus vulgaris is a non-scarring, blistering disorder of the skin and mucous membranes, characterized by flaccid blisters on erythematous skin which easily ruptures and leaves denuded areas. Mucous membranes, especially the mouth, are frequently involved. Pemphigus usually affects middle-aged people of all races and both sexes, although there is an increased susceptibility in Jewish people, especially Ashkenazi Jews. Patients appear to be genetically predisposed to other autoimmune diseases. Clinically it may present as a localized disease, especially in the mouth, scalp or nose, months before the skin is involved. The skin areas of predilection are scalp, face, axillae, groin and flexural sites. Lesions on the scalp and flexural areas may become vegetative (pemphigus vegetans). Nail involvement can cause dystrophy and paronychia. Histo-pathology of skin lesions reveals supra-basal cell clefting with acantholytic cells in a blister cavity. The immunopathology reveals IgG autoantibodies, which bind demoglein 3 (target antigen) in the desmosomes and the cell membrane of keratinocytes.
Answers
Published in Andrew Schofield, Paul Schofield, The Complete SAQ Study Guide, 2019
Andrew Schofield, Paul Schofield
Pemphigus vulgaris can be a life-threatening condition that occurs most commonly in Ashkenazi Jews. It is usually mediated by an autoimmune process, whereby there are antibodies generated against the desmosomal protein which causes kératinocytes to separate from one another, causing widespread bulla formation. It can also be caused by drug reactions. Bulla usually form on the mucosal surfaces, and this is helpful in distinguishing from bullous pemphigoid (in which mucosal involvement is rare). Biopsy is usually diagnostic. Until immunosuppressive agents were readily available, pemphigus had a high mortality rate; however, even with immunosuppression it still carries a risk of death due to the disease process or side effects of the treatment.
Cardiac function in pemphigus vulgaris patients before and after steroid pulse therapy
Published in Journal of Dermatological Treatment, 2021
Mohammad Shahidi-Dadras, Mehdi Pishgahi, Mohammadreza Tabary, Zohreh Kheradmand, Farnaz Araghi, Sahar Dadkhahfar, Reza M. Robati
Heart and skin share similar structural features such as the existence of adherence junctions and intercellular connecting structures (1,2). Therefore, it is not surprising that skin disease may affect the heart and vice versa. Pemphigus vulgaris which is an autoimmune dermatological disorder characterized by vesiculobullous lesions over the skin and mucosae may also give rise to cardiac disease (3). Intravenous administration of high doses of corticosteroids, popularly called steroid pulse therapy, has been an effective treatment in severe forms of pemphigus disease with several advantages over the conventional therapies (4,5). However, it has been established that the administration of high doses of intravenous corticosteroids is associated with arrhythmias, such as sinus bradycardia, supraventricular tachycardia, atrial fibrillation, ventricular tachycardia, myocardial infarction, and cardiac arrest (6–10). In light of these concerns, several studies have recommended inpatient administration of high-dose corticosteroid pulse, as well as continuous cardiac monitoring and metabolic blood tests (10).
Treatment of pemphigus vulgaris: part 2 – emerging therapies
Published in Expert Review of Clinical Immunology, 2019
Rebecca L. Yanovsky, Michael McLeod, A.Razzaque Ahmed
Pemphigus vulgaris (PV) is an autoimmune disease characterized by IgG autoantibodies against stratified squamous epithelia resulting in flaccid vesicles bullae and erosions, that can progress to become a life-threatening disease [1]. PV has a substantial effect on the patient’s quality of life, in large part due to its unpredictable clinical course and often relapsing nature. Although recent advances in therapy for PV have decreased morbidity and mortality. Pemphigus patients still face an average 2–3 times increase in mortality compared to the general population [2]. Conventional therapy involves corticosteroids (CS) and immunosuppressive agents (ISA). A 2011 systematic review of 11 randomized controlled trials, concluded that there was insufficient power and evidence in the literature, to recommend a single steroid-sparing agent as being the optimal choice [3]. Recent studies suggest biologic agents such as IVIg and rituximab are promising options [4–8]. Anti-CD20 antibodies with short-term CS for severe PV have recently been recommended as first-line treatments [9]. In addition, more biological agents are being tested for the treatment of PV, with the intent of offering more options to patients and their physicians. In this manuscript, information is presented on a number of clinical trials currently underway for the treatment of pemphigus vulgaris.
Ultra-low dose rituximab for refractory pemghigus vulgaris: a pilot study
Published in Expert Opinion on Biological Therapy, 2020
Irene Russo, Serena Miotto, Andrea Saponeri, Mauro Alaibac
Pemphigus vulgaris is an autoimmune blistering disease affecting the skin and mucous membranes, characterized by the presence of autoantibodies targeting the desmosomal structural proteins (desmoglein 1 and desmoglein 3), which mediate the adhesion between keratinocytes, resulting in intraepidermal acantholysis and blister formation. Early recognition and treatment are crucial for remission of this autoimmune disease. Current treatments for pemphigus vulgaris include anti-inflammatory and immunosuppressive agents [1]. Rituximab, a monoclonal antibody directed against the CD20 antigen of B-lymphocytes, has been shown to be effective in severe forms of pemphigus vulgaris [2]. However, the optimal dosage of rituximab for the treatment of pemphigus has not been clearly defined [3].