Rare illness, a case of Bullous Pemphigus: the benefits of apheresis.
نویسندگان
چکیده
Bullous Pemphigus and Pemphigus Vulgaris are autoimmune dermatological illnesses that manifest particularly in adults. They are characterized by the presence of auto-antibodies against specific structures of the skin and mucous membranes marked by the appearance of epithelial beads. While Bullous Pemphigus is of limited duration and is generally benign, in contrast Pemphigus Vulgaris is an illness that begins with lesions of the oral mucosa, where it can remain localized for many weeks then progressively extends to other parts of the body. A cutaneous biopsy is the best diagnostic procedure, followed by immunofluorescence which allows detection of IgG antibodies specific to the intracellular components of the epidermis and mucosa in the serum of patients with Pemphigus. Clinically, from the start, the lesions appear as flabby boils which often break easily. Wide areas of abrasions represent a serious problem for control of secondary infections and maintenance of fluid equilibrium (more or less the same problems that are seen in the treatment of serious burns). Bullous Pemphigus and Vulgaris are among 284 illnesses and 47 groups of rare illnesses for which a Ministerial decree was published in the 18th May, 2001. This is number 279 and number 160 of July 12th 2001 which founded a national network for rare illnesses and indications for exemption from cost. This rare illness that has serious pathology and results in disability for patients deprived of therapy has a low prevalence in Europe with less than 5 cases/10,000 patients. It represents a social problem since these patients often are difficult to diagnose and have trouble finding suitable care. The case which prompted our comment: A recent case of Pemphigus Vulgaris is presented which helps to emphasize this condition. These patients have a at best, a precarious social life, so much so that it was brought to the attention of the office of Social Services of our area in order to consider ways to improve the life of the patient. Mrs CG, age 58, was hospitalized in the department of Medicine of the Greater Hospital of Moderate on 06.1.02 because of serious organ deterioration. The diagnosis of Pemphigus Vulgaris had been verified histologically following cutaneous biopsy. The patient had been hospitalized in September of the preceding year in the department of infectious illnesses of the same hospital and, in succession, was transferred to the institute of Dermatology and Dermatological Diseases of the Corresponding author. Tel.: +1-613-748-9613; fax: +1-613748-6392. E-mail address: [email protected] (G. Rock).
منابع مشابه
A Case Report of Neonatal Pemphigus vulgaris
Pemphigus vulgaris (PV) is a chronic, rare mucocutaneous autoimmune bullous diseases characterized by flaccid blisters and/or pustules, with secondary erosions of the mucous membranes / skin. Pemphigus vulgaris is threatening patient life by the formation of splits within the epidermis, accompanied by acantholysis (separation of keratinocytes from each other). In this article, a term female neo...
متن کاملImmunofluorescence Pattern of Autoimmune Bullous Diseases in Iranian Patients
Background & Objective: Autoimmune bullous diseases are associated with autoimmunity against structural components in the skin and mucous membranes. Autoantibodies are against the intercellular junctions in pemphigus disease and hemidesmosomal unchoring complex in pemphigiod diseases and epidermolysis bullosa aquisita. The tissue-bound and circulating serum autoantibodies can be detect...
متن کاملIs there any association between a vitamin D receptor gene polymorphism (FokI) and pemphigus vulgaris?
Background: Pemphigus vulgaris (PV) is an autoimmune bullous disease of the skin and mucous membranes caused by activation and proliferation of T cells, production of Th2 cytokine profile and pathogenic antibodies. Vitamin D is a probable immunodeviator to Th2, which its actions are mediated through the vitamin D receptor (VDR). FokI is the only single nucleot...
متن کاملTherapeutic Apheresis in Dermatological Diseases
Since the mid-1970s, membrane modules became available and plasma separation techniques have gained importance. Therapeutic apheresis (TA) has been successfully introduced in a variety of autoantibody-mediated diseases. In dermatology, TA is increasingly applied as a support treatment for severe and/or refractory autoimmune bullous diseases. All autoimmune disease are characterized by autoantib...
متن کاملSerum Levels of Interleukin 17 in Patients with Pemphigus and Healthy Subjects
Introduction: Pemphigus is a relatively rare autoimmune bullous disease involving skin and mucous epithelia. The disease occurs as a result of production of auto-antibodies against inter-cellular epithelia especially cadherin and desmoglein. Interleukin 17 (IL-17) is one of the specific cytokines of T helper. IL-17 plays an important role in delayed type reaction and recruits neutrophils and mo...
متن کاملذخیره در منابع من
با ذخیره ی این منبع در منابع من، دسترسی به آن را برای استفاده های بعدی آسان تر کنید
برای دانلود متن کامل این مقاله و بیش از 32 میلیون مقاله دیگر ابتدا ثبت نام کنید
ثبت ناماگر عضو سایت هستید لطفا وارد حساب کاربری خود شوید
ورودعنوان ژورنال:
- Transfusion and apheresis science : official journal of the World Apheresis Association : official journal of the European Society for Haemapheresis
دوره 30 1 شماره
صفحات -
تاریخ انتشار 2004