CLINICAL REPORT
195 Advances in dermatology and venereology ActaDV Acta Dermato-Venereologica ActaDV
Diagnostic Delay in Dermatitis Herpetiformis in a High-prevalence Area
Eriika MANSIKKA 1, 2, Teea T. SALMI 1, 2, Katri KAUKINEN 2, 3, Pekka COLLIN 4, Heini HUHTALA 5, Timo REUNALA 1, 2 and Kaisa
HERVONEN 1, 2 1
Department of Dermatology, 3 Department of Internal Medicine, 4 Department of Gastroenterology and Alimentary Tract Surgery, Tampere University Hospital, 2 Faculty of Medicine and Life Sciences, and 5 Faculty of Social Sciences, University of Tampere, Tampere, Finland
Dermatitis herpetiformis( DH) is an extra-intestinal manifestation of coeliac disease. The highest currently reported prevalence of DH is in Finland, but knowledge of diagnostic delay is limited. This study investigated the duration of rash prior to diagnosis in 446 patients with DH, analysing the results in 3 periods of 15 years. The diagnosis was considered delayed when the duration of rash before diagnosis was 2 years or longer. Factors associated with delayed diagnosis were analysed. Within the 45 years, the median duration of rash before diagnosis decreased significantly, from 12.0 to 8.0 months( p = 0.002) and the occurrence of a delayed diagnosis decreased from 47 % to 25 %( p = 0.002). Female sex, the presence of villous atrophy, and a diagnosis of DH before the year 2000 were significantly associated with delayed diagnosis. In conclusion, the present study showed that one-quarter of patients currently have a diagnostic delay of 2 years or more, which is far from ideal.
Key words: dermatitis herpetiformis; coeliac disease; diagnostic delay; gluten-free diet; dapsone.
Accepted Oct 16, 2017; Epub ahead of print Oct 19, 2017 Acta Derm Venereol 2018; 98: 195 – 199.
Corr: Kaisa Hervonen, Department of Dermatology, Tampere University Hospital, PO Box 2000, FIN-33521 Tampere, Finland. E-mail: kaisa. hervonen @ staff. uta. fi
Dermatitis herpetiformis( DH) is a cutaneous manifestation of coeliac disease presenting as an itchy polymorphic blistering rash on the elbows, knees, buttocks and scalp( 1). Diagnosis of DH is based on the presence of typical skin symptoms and the demonstration of IgA in the papillary dermis during direct immunofluorescence examination( 2). Although 75 % of patients with DH have small bowel mucosal villous atrophy at diagnosis, only a minority have marked gastro intestinal symptoms( 3, 4). The treatment of DH is a lifelong gluten-free diet( GFD), similar to treatment of coeliac disease( 4, 5). A GFD results in healing of the enteropathy and the rash, but the rash alleviates slowly and additional treatment with dapsone( 4,4’-diaminodiphenylsulfone) is frequently needed at the start of dietary treatment( 6, 7).
DH is considered relatively uncommon, having the highest reported prevalence of 75.3 per 100,000 people in
Finland and a lower prevalence in UK and the USA( 8 – 10). In contrast to the established increase in the incidence of coeliac disease, the incidence of DH decreased in both Finland and UK during the 1990s( 8, 9). DH constitutes a diagnostic challenge to general practitioners and other non-dermatologists, and can easily be misdiagnosed as other itchy or blistering skin diseases( 11, 12). Early diagnosis is warranted in DH, since ongoing symptoms reduce quality of life, and undiagnosed DH predisposes to complications, such as lymphoma and low bone mineral density( 13 – 15).
For coeliac disease, the median time from onset of gastro intestinal symptoms to diagnosis in Finland is currently 3 years( 16). Fortunately, diagnostic delay has decreased over the past decades in Finland, other European countries, and the USA( 17 – 20). However, up-to-date knowledge about diagnostic delay in DH is lacking. The aim of this study was to investigate the changes in the diagnostic delay in DH and to analyse possible factors associated with delayed diagnosis. Our prospectively collected large DH cohort enabled us to perform delay analyses for 3 periods of 15 years.
PATIENTS AND METHODS
All patients suspected of having DH in the Tampere region, Finland, are referred by private dermatologists and general practitioners working in healthcare centres to the Department of Dermatology, Tampere University Hospital, for confirmation of DH diagnosis. Clinical suspicion of DH is an adequate reason for referral regardless of coeliac autoantibody result, and the referral policy for DH has remained the same for the years of the present study. In Tampere University Hospital, diagnosis of DH is based on the detection of typical clinical symptoms and the presence of granular IgA deposits in the papillary dermis( 2), and this diagnostic procedure has remained unchanged during the study period. All patients are treated at a special DH clinic, where they are advised to adhere to a lifelong GFD.
Our DH cohort consists of 512 patients who had been diagnosed and treated at the DH clinic between 1970 and 2014. A total of 446 patients were included for further analysis. Of the 66 patients excluded, 10 did not have IgA deposits in the skin, 21 had died over 20 years previously and their data was not available, and 35 had a previous diagnosis of coeliac disease. Data on the duration of the rash before diagnosis were collected from medical records held at Tampere University Hospital. The diagnosis was considered delayed when the duration of the rash before diagnosis was 2 years or more. The definition for delayed diagnosis was based on a previous study performed in our hospital district( 21) and
This is an open access article under the CC BY-NC license. www. medicaljournals. se / acta Journal Compilation © 2018 Acta Dermato-Venereologica. doi: 10.2340 / 00015555-2818 Acta Derm Venereol 2018; 98: 195 – 199