Schleimhautbeteiligung bei blasenbildenden Autoimmunerkrankungen

Günther, Claudia

doi:10.1007/s00105-016-3871-6

Cited by 5 publications

(3 citation statements)

References 31 publications

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“…Patients present with vesicles that rupture easily leading to erosions and rarely ulcers. Moreover, the erosions and ulcers represent routes of entry for a variety of pathogenes and determine secondary infections and even sepsis [17]. The pain is the main complaint and impairs the quality of life drastically, which is assessed with the use of The Dermatology Life Quality Index (DLQI) [21].…”

Section: Clinical Manifestationsmentioning

confidence: 99%

Untitled

2022

FARMACIA

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Pemphigus belongs to autoimmune blistering diseases and affects the skin and/or mucous membranes with a potentially fatal outcome in untreated patients. The main type is represented by pemphigus vulgaris (PV) which accounts for 70% of all cases in Europe. The diagnosis and management of PV in Romania are challenging due to lack of official treatment guidelines, the treatment being based on different protocols of each medical unit. This paper aims to adjust the diagnosis and management of PV in Romania according to the existing and accepted international guidelines. RezumatPemfigusul aparține grupului de boli buloase autoimune și afectează pielea și/sau mucoasele, putând avea o evoluție fatală în cazul pacienților netratați. Principala formă de boală este pemfigusul vulgar, care alcătuiește 70% din totalul cazurilor din Europa. Diagnosticul și managementul pemfigusului vulgar în România reprezintă o provocare din cauza lipsei unui protocol terapeutic oficial; tratamentul fiind bazat pe indicații diferite specifice fiecărei unități medicale. Scopul acestui manuscris este de a adapta diagnosticul și managementul pemfigusului vulgar în România în conformitate cu ghidurile existente și acceptate la nivel internațional.

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Section: Clinical Manifestationsmentioning

confidence: 99%

Untitled

2022

FARMACIA

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“…Mucous membranes are most frequently affected in MMP and paraneoplastic pemphigus, followed by pemphigus vulgaris and linear IgA bullous dermatosis. 57,58 Except in dermatitis herpetiformis associated with coeliac disease, GI involvement is generally rare in AIBD, and the true incidences are unknown. Oesophagus seems most commonly affected, in particular by pemphigus vulgaris, MMP and EBA.…”

Section: Gi Involvement In Autoimmune Bullous Dermatosesmentioning

confidence: 99%

“…Subepidermal AIBD may include bullous pemphigoid, pemphigoid gestationis, mucous membrane pemphigoid (MMP), dermatitis herpetiformis, linear IgA bullous dermatosis, epidermolysis bullosa acquisita (EBA) and anti‐p200 pemphigoid, while intraepidermal AIBD comprise different subtypes of pemphigus like pemphigus vulgaris, pemphigus foliaceus, pemphigus herpetiformis, IgA pemphigus, IgA/IgG pemphigus and paraneoplastic pemphigus. Mucous membranes are most frequently affected in MMP and paraneoplastic pemphigus, followed by pemphigus vulgaris and linear IgA bullous dermatosis 57,58 . Except in dermatitis herpetiformis associated with coeliac disease, GI involvement is generally rare in AIBD, and the true incidences are unknown.…”

Section: Gi Involvement In Autoimmune Bullous Dermatosesmentioning

confidence: 99%

Gastrointestinal involvement of primary skin diseases

Hsieh

Wei

et al. 2020

Acad Dermatol Venereol

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Less is known about gastrointestinal (GI) involvement of primary skin diseases due to the difference in embryology, histology, microbiology and physiology between integument and alimentary tract. Oesophagus, following the oropharyngeal mucosa, is the most common GI segment affected by primary skin diseases, especially by eosinophilic oesophagitis, lichen planus and autoimmune bullous dermatoses like pemphigus vulgaris, mucosal membrane pemphigoid and epidermolysis bullosa acquisita. Eosinophilic oesophagitis is an emerging chronic atopic disease with oesophageal dysfunction as the typical presentation, and oesophageal narrowing, rings and stricture as late complications. Oesophageal lichen planus mainly involves the proximal to mid-oesophagus in elderly aged women with long-term oral mucosal lesions. In acute attack of pemphigus vulgaris, oesophageal involvement is not uncommon but often neglected and may cause sloughing oesophagitis (oesophagitis dissecans superficialis) with acute GI bleeding in rare cases. GI manifestation of hereditary bradykininergic angio-oedema with colicky acute abdomen mostly affects small intestine, usually in the absence of pruritus or urticaria, and is more severe and long-lasting than the acquired histaminergic form. Strong evidence supports association between inflammatory bowel disease, especially Crohn disease, and hidradenitis suppurativa/acne inversa. Patients with vitiligo need surveillance of autoimmune liver disease, autoimmune atrophic gastritis or coeliac disease when corresponding symptoms become suspect. Melanoma is the most common primary tumour metastatic to the GI tract, with small intestine predominantly targeted. Gastrointestinal involvement is not uncommon in disseminated mycosis fungoides. Extramammary Paget's disease is an intraepidermal adenocarcinoma of controversial origin, and a high association between the anogenital occurrence and colorectal adenocarcinoma has been reported. As GI tract is the largest organ system with multidimensional functions, dermatologists in daily practice should be aware of the gastrointestinal morbidities related to primary skin diseases for an early diagnosis and treatment.

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Erfolgreiche Therapie eines sehr schweren Pemphigus vulgaris mit Rituximab

et al. 2019

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ZusammenfassungDer Pemphigus vulgaris ist eine blasenbildende Autoimmunerkrankung, die aufgrund der Hautbarrierestörung im Bereich der Blasen ein erhöhtes Irritations- und Infektionsrisiko bedingt. Wir berichten über einen 62-jährigen Patienten mit ausgeprägten Mundschleimhaut- und Hautmanifestationen eines Pemphigus vulgaris, der sich auf initiale Therapie mit hochdosierter Gabe von Prednisolon und steroidsparenden Immunsuppressiva nicht regredient zeigte. Der schwere Krankheitsverlauf wurde durch eine intensivpflichtige Sepsis zusätzlich kompliziert. Durch Gabe von Rituximab und einer begleitenden Immunapheresebehandlung konnte nach 8 Monaten eine partielle Remission und nach 1,5 Jahren eine komplette Remission erreicht werden. Der Pemphigus vulgaris ist auch heute eine schwere und komplikationsreiche Hauterkrankung, die eine therapeutische Herausforderung darstellen kann.

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Schleimhautbeteiligung bei blasenbildenden Autoimmunerkrankungen

Cited by 5 publications

References 31 publications

Untitled

Untitled

Gastrointestinal involvement of primary skin diseases

Erfolgreiche Therapie eines sehr schweren Pemphigus vulgaris mit Rituximab

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