A case of esophageal complication in epidermolysis bulhosa simplex.
نویسندگان
چکیده
UNLABELLED It is well known the involvement of the gastrointestinal tract in Epidermolysis Bulhosa (EB) and its consequences on both nutrition and growth. We present a case of a young girl with EB diagnosed soon after birth, admitted to our emergency room with food impactation. The endoscopy revealed a double barrel esophagus. No medical or surgical therapy was started and after 2 months of enteric feeding, spontaneous resolution was observed at endoscopy. Skin biopsy was compatible with EB Simplex. According to the literature EB Simplex is the subtype the least associated with GI tract involvement, presenting with dysphagia in only 2% of the patients, and with the lowest cumulative risk of esophageal stricturing. CONCLUSION We present an unusual case of EB simplex involvement of the esophagus with spontaneous resolution of a false lumen, with clinical improvement of the patient.
منابع مشابه
Epidermolysis Bullosa associated with pyloric stenosis: A case report
Epidermolysis bullosa are a group of hereditary skin diseases manifested as blisters on the sites of trauma to the skin. According to the level of blister development, epidermolysis bullosa are divided into the following groups: epidermolysis bullosa simplex, junctional epidermolysis bullosa and dystrophic epidermolysis bullosa. The latter two groups are associated with poorer prognosis and hig...
متن کاملFREE JEJUNAL GRAFT FOR TREATMENT OF ESOPHAGEAL STRICTURE DUE TO FAMILIAL EPIDERMOLYSIS BULLOSA
In this paper, two patients with severe dysphagia and esophageal stricture secondary to epidermolysis bullosa are presented and discussed. They are siblings of an affected family. Primary resection and anastomosis had been performed previously in both patients, but dysphagia had recurred. We treated these patients with free jejunal graft to the esophagus with excellent results. Both patient...
متن کاملA newborn presenting with epidermolysis bullosa with duodenal atresia: A very rare case report and review of the literature
Background: Epidermolysis bullosa (EB) comprises a group of genetically determined skin fragility disorders, which are characterized by blistering of the skin and mucosa, in response to little or no apparent trauma. These disorders represent heterogeneous phenotypes and are associated with various complications ranging from localized skin fragility to neonatal death. Nevertheless, the term "Epi...
متن کاملOtorhinolaryngological and esophageal manifestations of epidermolysis bullosa.
UNLABELLED Epidermolysis bullosa (EB) is a group of skin diseases with different clinical manifestations and varied inheritance patterns. Blisters may appear spontaneously or following minimal trauma to the skin or mucosa. AIM this paper aims to describe the otorhinolaryngological manifestations and esophageal complications related to EB, and the experience in treating patients with esophagea...
متن کاملEpidermolysis bullosa simplex with muscular dystrophy. Review of the literature and a case report.
BACKGROUND Epidermolysis bullosa simplex associated with muscular dystrophy is a genetic skin disease caused by plectin deficiency. A case of a 19-year-old Czech patient affected with this disease and a review all previously published clinical cases are presented. MAIN OBSERVATIONS In our patient, skin signs of the disease developed after birth. Bilateral ptosis at the age of 8 years was cons...
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:
- Gastroenterologia y hepatologia
دوره 35 9 شماره
صفحات -
تاریخ انتشار 2012