Explore chapters and articles related to this topic
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.
Inflammatory dermatoses affecting the nail
Published in Eckart Haneke, Histopathology of the NailOnychopathology, 2017
Pemphigus vegetans is a pemphigus vulgaris variant usually occurring in intertriginous folds. When occurring on the nails, it may clinically mimic acrodermatitis continua suppurativa of Hallopeau.253 Histologically, there are spongiform pustules with neutrophilic cell infiltration in a hyperplastic epithelium. The dermal inflammatory cell infiltration is dense and mainly consists of neutrophil granulocytes and an increase in eosinophils. Acantholysis may not be obvious and further sections or biopsies may be necessary.254 Immunofluorescence shows intercellular antibodies.
Pemphigus
Published in Lionel Fry, Atlas of Bullous Diseases, 2020
Pemphigus vegetans is a variant of PV in which healing of the lesions is associated with vegetating proliferation of the epidermis. The common sites for this type of the disease are the intertriginous areas, i.e. the groins (Figure 3.17), axillae and submammary regions. Secondary infection is common and crusting may occur.
Skin diseases of the vulva: inflammatory, erosive-ulcerating and apocrine gland diseases, zinc and vitamin deficiency, vulvodynia and vestibulodynia
Published in Journal of Obstetrics and Gynaecology, 2018
Freja Lærke Sand, Simon Francis Thomsen
Pemphigus is a rare chronic bullous disease in which autoantibodies react with desmosomes in the cell–cell adhesion structure. Pemphigus vulgaris commonly affects the mucous membranes in the oral cavity, conjunctiva and the vulvar and intertriginous areas as the initial manifestation (Lever 1979). A localised benign variant of pemphigus is termed pemphigus vegetans which heals through formation of verrucous hyperkeratotic vegetations. The clinical symptoms are painful bullae and erosions on the vulva. The diagnosis depends on histopathology showing suprabasal acantholysis, formation of bullae and separated basal keratinocytes (tombstones). Immunofluorescence microscopy shows deposits of IgG antibodies in the intercellular spaces of the epidermis. Treatment with prednisolone is needed in the majority of women, which may be combined with other systemic immunosuppressants.