Dermatitis Herpetiformis
Dermatitis herpetiformis is a chronic, autoimmune skin disease characterized by intense itching and the appearance of small, blistering lesions. It is often associated with celiac disease, as patients with dermatitis herpetiformis typically have a gluten sensitivity.
Epidemiology. Dermatitis herpetiformis is more common among Caucasians and is rare among people of African and Asian descent. The disease’s incidence ranges from approximately 11 to 75 cases per 100.000 individuals. It is more common in men than women, with a male-to-female ratio of about 1.5:1. The disease typically manifests in young adults, although it can occur at any age.
The pathophysiology of dermatitis herpetiformis is closely linked to celiac disease. Ingestion of gluten by genetically predisposed individuals triggers an immune response, leading to the formation of antibodies against transglutaminase. These antibodies attack the skin, causing characteristic inflammation and lesions.
The causes of dermatitis herpetiformis include genetic predisposition and gluten exposure. Patients often carry the HLA-DQ2 or HLA-DQ8 genes associated with celiac disease. Gluten consumption triggers the immune response that leads to the development of the disease.
Symptoms of dermatitis herpetiformis include intense itching and the appearance of small, blistering lesions. Lesions typically appear symmetrically on the elbows, knees, buttocks, and back. The blisters can break easily, forming crusts.
Differential Diagnosis of dermatitis herpetiformis includes other skin conditions that may cause similar symptoms, such as contact dermatitis, atopic dermatitis, psoriasis, and blistering disorders like pemphigoid.
Dermatitis herpetiformis complications include persistent itching and skin lesions, which can lead to secondary infections due to blister breakdown. Additionally, the disease is associated with an increased risk of developing other autoimmune disorders, such as Hashimoto’s thyroiditis and type 1 diabetes.
The treatment of dermatitis herpetiformis involves avoiding gluten. Adopting a strict gluten-free diet can reduce or eliminate symptoms. Additionally, dapsone is an effective medication for managing symptoms, although it can have side effects like hemolytic anemia and methemoglobinemia. For patients who cannot tolerate dapsone, alternative treatments include sulfapyridine or other immunosuppressive agents.
Laboratory Tests and Monitoring Laboratory tests for diagnosing dermatitis herpetiformis include direct immunofluorescence (DIF) of the skin, which detects granular deposits of IgA in the dermal papillae. Additionally, detecting antibodies against tissue transglutaminase and epidermal transglutaminase in the blood can indicate the presence of celiac disease.
- Tissue Transglutaminase (tTG), IgA Antibodies
- Tissue Transglutaminase (tTG), IgG Antibodies
- Endomysial Antibodies IgA
- Endomysial Antibodies IgG
- Deamidated Gliadin Peptides (DGP), IgA Antibodies
- Deamidated Gliadin Peptides (DGP), IgG Antibodies
- Immunoglobulin A
- F-actin Antibodies
- Coeliac Disease, Basic Profile
- Coeliac Disease, Extended Profile
- Coeliac Disease, Genetic Testing
Several blood tests may also be necessary to differentiate the disease from other skin diseases with similar clinical features.
- Pemphigoid Antibodies (Antibodies against Epidermal Basement Membrane)
- Pemphigus Antibodies
- Collagen VII, Antibodies
- BP180, IgG antibodies
- BP180, IgA Antibodies
- BP230, Antibodies
- Desmoglein-1, Antibodies
- Desmoglein-3, Antibodies
- Dapsone
Monitoring patients after diagnosing and treating dermatitis herpetiformis involves regular clinical examinations to assess the response to a gluten-free diet and medication. Patients should be monitored for the development of other autoimmune disorders and undergo regular blood tests to evaluate antibody levels against transglutaminase. Additionally, monitoring dapsone levels in the blood may be necessary to avoid toxicity.
This article belongs to a new series on our blog that covers all fields of health! We present information on the most frequent pathological conditions in a comprehensive, clear, understandable, but always scientifically documented way so you can know and protect the most crucial good: your health!
Ioannis Sideris, Medical Doctor
