寻常型天疱疮伴胸腺瘤1例—附尸解报告

报告１例严重的寻常型天疱疮病例。患者合并有多个器官的严重感染，最后因感染死于中毒性休克。尸解发现死者并有胸腺瘤。复习文献、讨论了天疱疮与胸腺瘤的关系。     

Pemphigus vulgaris

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Pemphigus vulgaris

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Pemphigus vulgaris and disseminated nocardiosis.

Infectious diseases, in particular septicaemia from Staphylococcus aureus, Proteus vulgaris and Pseudomonas aeruginosa, are the most severe and frequent complications for the immunosuppressive therapy of pemphigus. Infection by Nocardia asteroides in subjects with pemphigus vulgaris is rare. We report the sixth case found of such an association; the subject died of disseminated nocardiosis while receiving steroids and immunosuppressive drugs, 4 years after being diagnosed with chronic pemphigus vulgaris.     

Pemphigus vulgaris

Pemphigus vulgaris is an autoimmune blistering disorder. The diagnosis is based on clinical presentation, histology, and direct and indirect immunofluorescence. Optimal treatment remains the greatest challenge. Treatment options include combinations of systemic steroids with azathioprine, mycophenolate, and cyclophosphamide. However, there is little solid data regarding the drug of choice. In our patient, the best tolerated and effective treatment was found after several therapeutic attempts.     

Pemphigus vulgaris associated with a syndrome of immunodeficiency and thymoma: a case report

A 44-year-old patient with immunodeficiency and thymoma syndrome who had received treatment for myasthenia gravis presented with pemphigus vulgaris localized to the oral cavity. Direct immunofluorescence staining demonstrated the presence of IgG antibody against the intercellular substance of stratified epithelium. No circulating intercellular antibodies, anti-nuclear antibody, rheumatoid factors, anti-thyroid antibody or anti-smooth muscle antibody were present. This association of pemphigus vulgaris with primary immunodeficiency suggests that altered immunity may be important in the pathogenesis of pemphigus.     

Pemphigus vulgaris

Pemphigus vulgaris is a chronic autoimmune bullous dermatosis that results from the production of autoantibodies against desmogleins 1 and 3. It is the most frequent and most severe form of pemphigus, occurring universally, usually between 40 and 60 years of age. It usually begins with blisters and erosions on the oral mucosa, followed by lesions on other mucous membranes and flaccid blisters on the skin, which can be disseminated. There is a clinical variant, pemphigus vegetans, which is characterized by the presence of vegetating lesions in the large folds of the skin. Clinical suspicion can be confirmed by cytological examination, histopathological examination, and direct and indirect immunofluorescence tests. The treatment is performed with systemic corticosteroids, and immunosuppressive drugs may be associated, among them azathioprine and mycophenolate mofetil. More severe cases may benefit from corticosteroids in the form of intravenous pulse therapy, and recent studies have shown a beneficial effect of rituximab, an anti-CD20 immunobiological drug. It is a chronic disease with mortality around 10%, and septicemia is the main cause of death. Patients need long-term and multidisciplinary follow-up.     

Pemphigus vulgaris malignus

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Therapieresistenter Pemphigus vulgaris

Zusammenfassung Der Pemphigus vulgaris stellt eine potenziell letale, bullöse Autoimmundermatose der Haut und der Schleimhäute dar, deren Standardtherapie bei schweren Verlaufsformen derzeit aus dem kombinierten Einsatz von hoch dosierten Glukokortikoiden und Azathioprin besteht. Wir präsentieren eine 55-jährige Patientin mit rezidivierendem Pemphigus vulgaris, die unter dieser Behandlung therapierefraktär und progredient war. Unter einer Pulstherapie mit hoch dosiert intravenösem Methylprednisolon wurde die Exazerbation der Erkrankung aufgehalten. Eine komplette Abheilung sowie Langzeitremission wurde unter oraler Kombinationstherapie aus Methylprednisolon und Doxycyclin erzielt.

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Recalcitrant pemphigus vulgarisCombined treatment with methylprednisolone and doxycycline

Pemphigus vulgaris is a potentially lethal autoimmune bullous disease of the skin and the mucous membranes. High-dose systemic glucocorticoids and azothioprine represent the current standard treatment in severe cases. A 55-year-old woman with recalcitrant pemphigus vulgaris failed to respond to this standard approach. Short-term pulse therapy with intravenous methylprednisolone stopped the disease progression. Subsequently oral methylprednisolone combined with oral doxycycline led to complete healing and long-term remission.

     

Pemphigus vulgaris and myasthenia gravis

This paper describes the occurrence of pemphigus vulgaris in a 46-year-old female with a thymic tumour. The myasthenia gravis went into remission within a few months of thymectomy, but the patient continued to need steroids for pemphigus vulgaris for a further 5 years. The histological appearances of the thymic tumour and skin and the serological findings are described.     

Fall 61. Steroidnebenwirkungen bei Pemphigus vulgaris

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Fall 61. Steroidnebenwirkungen bei Pemphigus vulgaris

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