Dermatitis herpetiformis is a long-lasting autoimmune condition that causes a very itchy rash, usually on the outer surface of the body. The small blisters can often be hard to see because they get damaged from scratching. This condition is closely related to GSE (gluten-sensitive enteropathy) as both take into account the creation of IgA autoantibodies resistant to transglutaminases, which then deposit in the top layers of the skin. To manage it, patients need to avoid gluten completely and often use medications such as dapsone. 

This condition is relatively common among people of Northern European countries with an incidence ranging from 1.2-39.2 for every one lakh individuals and an incidence of 0.4- 2.6 for 100,000 persons every year. It is more prevalent in men than in women with a male/female proportion of 1.5 to 2:1. The condition is less common in African and Asian populations but has been observed in both older adults and children. The extent of GSE can vary widely among patients, and dermatitis herpetiformis disease is often associated to autoimmune diseases like autoimmune thyroid disease. Additionally, individuals with this condition face a higher risk of vitiligo, small bowel lymphoma, vitiligo, and Addison’s disease. Genetic factors play a crucial role in susceptibility to the disease. 

Gluten-sensitive enteropathy (GSE) and dermatitis herpetiformis are a result of an autoimmune response directed by IgA against transglutaminase proteins. Tissue transglutaminase is the main target in GSE. There is some evidence which shows that TG3 – epidermal transglutaminase is the main antigen in  . These 2 proteins have many common enzyme domains and present in epidermis. TG3 is necessary in cross linking and to maintain a barriers which can protect the skin. 

Although the exact mechanisms involved in the process of itching and inflammation in the dermatitis herpetiformis are still not completely understood, yet they are not irreducible. One suggestion is that TG3 is released from skin cells and goes into the upper dermis. It binds to the circulating IgA antibody. There is a possibility that IgA and TG3 make a complex and deposit in the skin. 

This condition manifests as a genetic, environmental, and dietary gluten issue. Genetics are closely associated with certain HLA types, while environmental factors such as dietary gluten and gliadin, a constituent of gliadin, are involved in its pathogenesis. Screening may be required for the first-degree relatives of GSE or dermatitis herpetiformis patients. Environmental factors which include exposure to iodine and tobacco smoking may also be a contributing factor of the disease. Monozygotic twins are a good example of people who share a high rate of disease concordance.                                                            

Dermatitis herpetiformis diseases is a longstanding sickness that requires being on gluten-free diet for life. People who successfully adhere to this diet are likely to have good long-term outcomes and may even be able to reduce or discontinue their dapsone medication. Newer study findings show that patients with non-responsive gluten-sensitive enteropathy tend to have poor outcomes. The disease is chronic, but a remission can happen often seen in adults above 40 years of age. 

The main symptom of this disease is itching on the skin. The most of the patients experience rash on the surface of knees, elbows, scalp, or buttocks. They also have symptoms which are related to GSE like diarrhea, bloating, or other GI complications or other autoimmune disease like hypothyroidism. 

Dermatitis herpetiformis diseases is the diagnosis when a typical rash of grouped red vesicles and papules appears mainly on extensor surfaces. Owing to the severe scratching, the vesicles are not seen properly, and the scratch marks are the ones that present patients instead. The lesions are usually symmetrically distributed, and they are the ones that recover without creating scar. A few of the patients may notice the occurrence of small purple spots on the soles and palms. The mucosa may be affected, and dental problems, such as enamel pits, have been noted. The first-degree family members of patient who have GSE may also have defects in enamel. 

1. Small bowel lymphoma 
2. Anemia due to malabsorption 
3. Osteoporosis 
4. Loss of weight 
5. Neuropathy 
6. Ataxia 
7. Short stature in children 

Medical care: 

This disease is treatable by different approaches. A gluten-free diet coupled with dapsone tapering is a typical treatment, which leads to about 12% of patients achieving remission. Sulfasalazine was used to be prescribed in the US, and it can still be given as a monotherapy or in combination with dapsone. The efficacy of the treatments that might be less efficacious are colchicine, cyclosporine, azathioprine, heparin, and prednisone. Ultraviolet light therapy may provide some symptomatic relief, and a combination of tetracycline and niacinamide has shown some success in this treatment. The use of cyclosporine should be limited because of its propensity to cause intestinal lymphomas. Anti-body targeting strategies such as rituximab and intravenous immunoglobulin may also be effective. It is said that the Atkins diet has cured dermatitis herpetiformis, and topical anti-inflammatory medications, iodides, and NSAIDs, may help in relieving from the disease. 

Infectious Disease

Dietary modifications: The AGA Institute states that the main therapy for dermatitis herpetiformis, a skin disorder brought on by gluten ingestion, is a lifetime gluten-free diet. Adherence is greatly aided by patient support, encouragement, and education. A gluten-free diet can help most people manage their symptoms, and some may even decide to discontinue using dapsone. It can be difficult to stick to this diet, though, and it takes a lot of patience. Diets low in gluten may alleviate symptoms, however this is not always the case. 

Infectious Disease

Dapsone: The exact mechanism of action of dapsone in treating dermatitis herpetiformis disease is not known. It is known to prevent the use of PABA by bacteria for synthesizing folic acid which may prevent the growth of bacteria.  

Sulfasalazine: This possesses anti-inflammatory activity and has higher affinity in the connective tissues. 

Colchicine: This causes activation, migration of neutrophils and degranulation which might reduce inflammation. 

Rituximab: It is a monoclonal antibody (humanized). This is known to bind to the CD20 antigen and induce antibody or complement -mediated cytolysis. 

Infectious Disease

Prednisone: It is a glucocorticosteroid that possesses moderate anti-inflammatory and mild mineralocorticoid effects. It is known to control inflammation by stopping the rate of synthesis of proteins and suppressing the migration of PMNs (polymorphonuclear leukocytes). 

Infectious Disease

Dermatitis herpetiformis is a disease the treatment of which needs to be pursued from several points. These include a diagnosis with an assessment through clinical evaluation and laboratory testing and the prescription of a gluten-free diet. Treatment based on these three parts involves the following components: a strict gluten-free diet, taken throughout life; medication; and other treatments, including sulfasalazine or colchicine. The third component is that patients need education regarding dietary prescriptions and motivation and regular follow-through. Other important modalities of treatments incorporate symptom assessment and medication adjustments, dietary compliance, complication and co-condition management. Long-term management includes lifelong adherence to the gluten-free diet with periodic reviews and ongoing education. If in case the conventional methods are not enough, then experimental treatments such as elemental diets or antibody-based treatments might be tried. Each step is of importance in the line of management; however, it has to be modified according to the needs and therapeutic responses in each patient. To conclude, dermatitis herpetiformis calls for an integrated approach in management.