To the Editor:
A 32-year-old single woman was referred to our gastroenterology clinic, complaining of intermittent bloody diarrhea for the past 8 years. She had several visits and underwent sigmoidoscopy twice, but no definite diagnosis was made. Our gastroenterologist advised a total colonoscopy because of her complaints of abdominal pain, bloody diarrhea, duration of the chief complaint, and her lack of symptoms of infectious disease.
After primary preparation, a total colonoscopy was performed in October 2007 and several biopsies were taken from visible lesions. It was reported that her rectum had good vascularity, descending and sigmoid colon were easily bruised, ascending and transverse colon were easy bruised with fine erosions, and the cecum had erosions. Mascroscopic examination revealed inflammatory bowel disease with mild edema and increased inflammatory cells in the lamina propria composed of mononuclear cells and eosinophils, and few neutrophils in the lamina and muscular layer of mucosa, compatible with inflammatory bowel disease-indeterminate. The patient was treated with six tablets of mesalazin 500 mg a day and she showed a good response to the medicine with recovery in her symptoms.
In February 2008, the patient reported oral aphthous lesions, which were treated conservatively. She showed ophthalmic pruritic lesions in April 2008, which started to spread in the form of fine vesicles converting to bullous lesions all over the body surface (see Figure 1). She was referred to dermatology clinic, and Tzanck smear revealed many neutrophils, consistent with pus, that were negative for multinucleated cells or malignancy. Biopsies were taken from lesions of the posterior of the chest and pubis. Microscopic examination sections show skin tissue with subepidermal bulla. In the dermis and the cavity of the bulla, there was an inflammatory infiltrate containing many neutrophils. Foci of surface ulceration and fibrin deposition could be seen. Final histopathology examination was compatible with dermatitis herpetiformis.
The patient was admitted to the hospital because of painful and fulminated ulcers, which prevented her from swallowing and eating food. One-fourth of a dapson tablet was given for 2 days. Prednisolone 50 mg/day, mesalazine 1000 mg tid, and one hydroxyzine tablet q12h were continued for 10 days postadmission. Prednisolone was tapered and the lesions disappeared after the first 2 days of treatment. She was advised to consider a celiac regimen without any wheat. She was symptom-free when followed up and the only complaint was weight gain due to edema.
Autoimmune bullous diseases were the first differential diagnoses considered for her lesions, including pemphigus and its variants, bullous pemphigoid, and dermatitis herpetiformis. Among these diagnoses, dermatitis herpetiformis (DH) was the most probable diagnosis due to the underlying disease.
On the one hand, it is estimated that established celiac disease (CD) is present in 12.6% of patients with DH, and on the other hand, DH had been diagnosed in 3.7%-11.1% of patients with known CD (Cholongitas, Pipili, & Ioannidou, 2008).
Dermatitis herpetiformis is a recurrent chronic and rare papulovesiclar disease, which is considered a cutaneous manifestation of gluten sensitivity (Dieterich et al., 1999). As she did not accept to undergo endoscopy, we evaluated the serologic tests for CD such as antiendomysial antibody and anti-tissue transglutaminase (Ttg) antibody with ELISA method, which revealed normal serology. The patient was assessed with Direct Immuno Fluorescent method to rule out the other possible differential diagnosis, linear IgA bullous lesion, which was negative.
The serologic level of Ttg antibody and endomysial antibody are considered useful in screening of CD in patients with dermatitis herpetiform with a specificity and sensitivity of 97.6% and 89.1% (Dieterich et al., 1999). We evaluated the level of anti-Ttg antibody at the first step, which was negative, and can therefore rule out the possibility of CD in this patient, although she refused to undergo a screening upper gastroscopy.
The association between ulcerative colitis and dermatitis herpetiform is rare and about 10 cases were reported up to now in the literatures. DH is among the miscellaneous skin manifestations of ulcerative colitis versus specific or reactive manifestations. Both DH and ulcerative are more common in men, but there are some women described with this coexistence (Chiba et al., 1996; Malmusi, Manca, & Girolomoni, 1994).
ACKNOWLEDGMENT
The authors appreciate Dr. Shahryar Semnani for his kind cooperation in providing references and editing the letter.
Sima Besharat, MD
Mohammad Ebrahimirad, MD
Taghi Amiriani, MD
Vahideh Kazeminejad, MD
Golestan University of Medical Sciences, Golestan Research Center of Gastroenterology and Hepatology, Gorgan City, Golestan Province, Iran
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