Epidemiological Data

We collected 14 cases of DH among 11,456 patients who had been seen in consultation or admitted to the hospital. The prevalence of DH at the dermatology department was 0.12%. The mean age of the patients was 8 years (range: 4 to 27 years). Eight patients were male and six were female (sex ratio: 1.33). Most patients were in the 6–10 year age group (7 cases) (Table 1) and were schoolchildren (8 cases) living in a rural environment.

Table 1: Age and sex distribution of the fourteen patients.

Clinical Data

The duration of the disease ranged from five days to one year, with a mean duration of 59.35 days. A history of gastrointestinal disturbances, such as abdominal pain and diarrhea, was observed in eight patients. Pruritis was the main functional sign observed and was present in twelve patients. The general condition remained good in ten patients. All patients presented with generalized polymorphous lesions consisting of vesicular bullae, papules, and erosive and blackish excoriated lesions in clusters in some areas (Fig. 1). Mucosal involvement was present as vesicular stomatitis (2 cases) and conjunctivitis (1 case).

Figure 1: Multiple vesicular bullae on the trunk in clusters in the lumbar area.

Paraclinical Data

A biopsy of the bullous lesion was performed in six patients and the findings were consistent with DH in 5 cases. Four patients had anemia, as shown by a full blood count. G6PD was measured in two patients yet showed no deficiency.

Treatment

A gluten-free diet (GFD) and dapsone (2 mg/kg/day) were proposed in all patients. Systemic corticotherapy was administered in association with dapsone in one patient who did not respond to dapsone alone after one month.

Course of the Disease

After one month of treatment, six patients were clinically healed and the episode of vesicular-bullous lesions had cleared. The episode persisted in seven patients and one patient was lost to follow-up. After three months of follow-up, five patients had no recurrence of bullous lesions. A new outbreak occurred in two cases, and six patients who lived in rural areas were lost to follow-up.

Epidemiology

We recorded 14 cases (0.12%) of DH at the dermatology department of our institution during the five-year period. Kaloga et al. in the Ivory Coast and Akakpo et al. in Togo found a lower prevalence at their departments, with 7 patients in 5 years and 11 patients in 8 years, respectively [6,7]. Zaraa et al. in Tunisia observed 9 cases in 11 years [8]. Our findings confirmed the rarity of DH.

The disease predominantly affected males. Ouattara and Sidigg et al. also found a male predominance, with sex ratios of 1.56 and 1.03, respectively [4,5]. On the other hand, Akakpo et al. and Zaara et al. found a female predominance, with sex ratios of 0.8 [7] and 0.5 [8], respectively. A female predominance was also reported by Smith et al. in the U.S. [9].

The age of our patients ranged from 4 to 27 years, with a mean of 8 years. The disease was most frequent in the 6–10 year age group. This was similar to reports in the literature indicating that DH most often develops between the second and seventh year of life, as found by Ermacora et al. [10]. Other studies, however, have shown a predominance of DH in adults aged between 25.6 and 66 years [2,5,6,11].

Clinical Aspects

The duration of the disease before the specialist consultation ranged from five days to one year, with a mean of 59.35 days. This delay was long and is explained in our context by initial neglect by the patients of their skin problems, the use of phytotherapy before seeking medical advice, and the lack of access to dermatologists. This long time to diagnosis has also been reported in some developed countries, sometimes being as long as two years [12].

Pruritis was the principal functional sign, reported by the majority of the patients. In the literature, DH is classically accompanied by intense pruritis [1,4,7,13,14].

Eight patients presented with gastrointestinal signs. However, confirmatory gastrointestinal endoscopy was not performed. Akakpo et al. observed no signs of gluten enteropathy, nor did they conduct supplementary investigations to detect one [7]. Ouattara reported a case of associated coeliac disease [4]. Zaraa et al. reported two cases of coeliac disease among four patients who had undergone supplementary tests [8]. Dermatitis herpetiformis is considered as the cutaneous manifestation of gluten intolerance, invariably accompanied by coeliac disease [10,14].

Generalized polymorphous lesions were present in all our patients. The same observation was made by Akakpo et al. [7] and by Ouattara, who reported polymorphous and symmetrical lesions in 73.9% of cases [4].

Three patients (21.4%) had oral and ocular mucosal involvement. Akakpo et al. also reported such involvement in 36.4% of cases [7] and Ouattara in 17.4% [4]. Zaara et al. found no mucosal involvement [8]. It is rare in DH [1].

Paraclinical Aspects

A biopsy of the lesions was performed in six patients, and the histology was compatible with GH in five cases. Ouattara confirmed this observation in 52.2% of biopsies [4]. Histology makes little contribution to the diagnosis. The reference diagnostic test for DH is direct immunofluorescence (DIF) [1,14], which is unavailable in our context.

Treatment

A gluten-free diet was initiated in all patients. It is indispensable for the management of DH [1,14], yet is difficult to follow. Treatment with dapsone (2 mg/kg/day) was offered to the fourteen patients. This molecule is the mainstay of DH treatment [1,14,15] and was effective in obtaining rapid regression of the cutaneous signs, generally within several days.

Evolution

After three months of follow-up, seven patients who came from a rural environment were lost to follow-up. This could be explained by financial difficulties and the use of phytotherapy. Better therapeutic education could considerably reduce the number of patients lost to follow-up.

Statement of Human and Animal Rights

All the procedures followed were in accordance with the ethical standards of the responsible committee on human experimentation (institutional and national) and with the 2008 revision of the Declaration of Helsinki of 1975.

Statement of Informed Consent

Informed consent for participation in this study was obtained from all patients.