La dermatitis herpetiforme constituye una enfermedad crónica, hereditaria, de base inmune, que afecta preferentemente a la población del norte de Europa. La dermatitis herpetiforme es una enfermedad ampollosa autoinmune que aparece como expresión cutánea de la intolerancia al gluten. Forma parte de un . Dermatitis herpetiformis in Brazilan male celiac disease patients: a case series. Dermatitis herpetiforme en hombres brasileños con enfermedad celiaca: una.
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Dermatitis herpetiformis in Brazilan male celiac disease patients: Celiac disease CD is a chronic autoimmune systemic disease triggered and maintained by gluten proteins.
CD can begin at any age with a higher prevalence among the female gender ratio 2: Due to minor prevalence in male gender, CD has been less studied in this group 2. Dermatitis herpetiformis DH is an autoimmune blistering cutaneous disease considered as the specific phenotypic expression of a gluten-sensitive enteropathy on the skin, immunohistochemicaly indistinguishable from CD 3,4.
Gender can influence the onset, clinical presentation, progression, and outcome of some autoimmune diseases 6.
Dermatitis herpetiforme en hombres brasileños con enfermedad celiaca: una serie de casos
Although controversial, clinical presentation of several autoimmune diseases in males can be more severe 7. The present study aims dermztitis report the clinical aspects of male Brazilian patients with both CD and DH, also showing their laboratory and histological findings, treatment and follow-up information. This study with an observational, descriptive and retrospective design analyzed a series of consecutively diagnosed celiac patients; it was approved by the Institutional Ethic Committee.
Of a total of eighty-seven men, all with European ascendancy, diagnosed with CD and attended by a single physician in a private office between andfourteen All data were collected from patients’ charts. CD was diagnosed based on clinical, serological, and histological findings Marsh’s classification 1. Diagnoses of DH was based on clinical and histological findings dermatiis skin biopsies.
The follow-up of the patients consisted in interviews, clinical examination and serological tests. All the evaluations were conducted by the same physician at the moment of diagnosis, once a month up to the 6 th month after diagnosis, and then every six months. Fourteen male patients, with a median age of 34 rank dermatitus to 55 years were diagnosed with both CD and DH.
Their general and gastrointestinal complaints are shown in table I. Demographical data of patients are shown in table II. All patients were diagnosed as having DH due to a typical skin lesion presentation. Despite the presence of classical features of DH, a delay in the diagnosis, ranging from 0 to months median: Duodenal biopsies confirmed CD in all the patients, and the histological evaluation showed severe enteropathy in 11 cases One patient presented jaundice after being treated with dapsone and this drug was discontinued.
Nine patients were investigated for thyroid diseases, and 4 Five patients had another first-degree relative with a gluten-related disorder: DH was reported in one father and in one sister, CD was reported in one sister and one daughter, and one patient had twin daughters with both CD and DH. Our study documented that DH is commonly associated to CD in Brazilian male patents in a similar way that reported from European and North American studies, suggesting that geographic differences are herpetiorme influenced in these diseases 2,4.
The same was observed regarding the mean age of occurrence third or fourth decadeas well as with diagnostic delay 4,8 Table II. The mechanisms that trigger overt symptoms are not well understood. In Brazil, the only report on this subject is credited to Kotze, who referred DH in Although all DH patients presented gluten sensitivity, the great majority were asymptomatic regarding the digestive point of view. In our study, bloating, chronic diarrhea and herpetifoorme pain were the main digestive symptoms referred.
General symptoms of patients mainly included weight loss, malnutrition, and peripheral edema Table I. Physical examination by itself may provide a suspicion of DH; however, additional testing is usually required. Some authors related that small bowel biopsies are usually not necessary in a DH workup 3.
Additionally, we recommend obtaining duodenal biopsies because this first evaluation of the intestinal mucosa is useful for the future, especially if a suspicion of malignancy lymphoma exists 1,3.
Screening of patients’ relatives must be recommended at the time of diagnosis, drrmatitis, in dermatiits present study, the discovery of first-degree relatives having CD or DH in 5 patients dsrmatitis our assertion, as the majority of them were asymptomatic or undiagnosed.
This malignancy can occur both in and outside the gastrointestinal tract as a nodal or extranodal disease. On the other hand, survival rates in patients with DH do not appear to differ from those of the general population. Gastroenterologists treating patients with CD must be alert for skin manifestations of gluten sensitivity and other dfrmatitis diseases. On the other hand, dermatologists may be aware of digestive implications of DH. Celiac disease in Brazilian patients: Forty-years of clinical experience.
Gender- and age-related differences in symptoms. Scad J Gastroenterol ; The Italian Group for Cutaneous Immunopathology.
Guidelines for the diagnosis and treatment of dermatitis herpetiformis. J Eur Acad Dermatol Venereol ; Skin manifestations of celiac disease. Celiac disease and dermatitis herpetiformis: The spectrum of gluten sensitive enteropathy.
Int J Dermatol ; Redox state, cell death and autoimmune diseases: Nussinovitch U, Shoenfeld Y. The role of gender and organ specific autoimmunity.
Clinical, pathologic, and immunopathological features of dermatitis herpetiformis: Review of the Mayo Clinic experience. Lymphoma in patients with dermatitis herpetiformis and their first-degree relatives. Dermafitis J Dermatol ; Bolontin D, Petronic-Rosic V.
Epidemiology, pathogenesis, and clinical presentation. J Am Acad Dermatol ; Dear EditorCeliac disease CD is a chronic autoimmune systemic disease triggered and maintained by gluten proteins.
Patients and methods This study with an observational, descriptive and retrospective design analyzed a series of consecutively diagnosed celiac patients; it was approved by the Institutional Ethic Committee. Results Fourteen male patients, with a median age of 34 rank 17 to 55 years were diagnosed with both CD and DH. Discussion Dermatittis study documented that DH is commonly associated to CD in Brazilian male patents in a similar way that reported from European and North American studies, suggesting that geographic differences are not influenced in these diseases 2,4.