Dermatitis herpetiformis
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Amber Ehsan Faquih, MD[2] Kiran Singh, M.D. [3]
Synonyms and keywords: Duhring disease; Duhring-Brocq disease
Overview
Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief: Vishnu Vardhan Serla M.B.B.S. [2]
Overview
Dermatitis Herpetiformis (also called Duhring’s disease), is a chronic itchy rash which is frequently associated with Celiac Disease. The rash is made of papules and vesicles which are present on different parts of the body mostly commonly on neck, trunk, buttocks and knees. It is an autoimmune mediated skin condition, which is IgA mediated reaction and is associated with gluten sensitivity of small bowel. There is presence of antibodies which leads to positive serology test results.
Dermatitis herpetiformis is associated with high prevalence of other autoimmune diseases.
Historical Perspective
Classification
Pathophysiology
Causes
Differentiating Dermatitis Herpetiformis from Other Diseases
Epidemiology and Demographics
Risk Factors
Dermatitis herpetiformis usually begins in people age 20 and older, although children may sometimes be affected. It is seen in both men and women.
Natural History, Complications and Prognosis
Diagnosis
History and Symptoms
Dermatitis herpetiformis breakouts are usually extremely itchy. In many people the vesicles or papules appear on the elbows, knees, back, and buttocks (pressure points). It may also present as a patch of red skin with little water blisters scattered about. It is a systemic condition; the unpredictable skin rash may appear or be exacerbated by any irritation such as dry skin, scratching or clothing that is rough or scratchy. The fact that the rash is most prevalent at pressure points (where clothing rubs the most) may be why the symptoms sometimes appear to be symmetrical.
Physical Examination
Laboratory Findings
A skin biopsy and direct immunofluorescence test of the skin are performed in most cases; doctors may additionally recommend a biopsy of the intestines.
CT Findings
MRI Findings
Other Imaging Findings
Treatment
Medical Therapy
Dapsone, an antibiotic, may help the majority of patients. A strict gluten-free diet will also be recommended to help control the disease. Adherence to this diet may eliminate the need for medications and prevent later complications.
Surgery
Prevention
References
Historical Perspective
Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]
Overview
Dermatitis Herpetiformis (also called Duhring’s disease), is a chronic itchy rash which is frequently associated with Celiac Disease. The rash is made of papules and vesicles which are present on different parts of the body mostly commonly on neck, trunk, buttocks and knees. It is an autoimmune mediated skin condition, which is IgA mediated reaction and is associated with gluten sensitivity of small bowel. There is presence of antibodies which leads to positive serology test results.
Dermatitis herpetiformis is associated with high prevalence of other autoimmune diseases.
Historical Perspective
Dermatitis Herpetiformis also called Duhring’s disease after the name of a physician who first described it, Louis Adolphus Duhring in 1884. Initially the disease was put in the category of bullous disease such as pemphigus vulgaris. Later in 1888. another physician named Brocq, described a similar lesion and named it Polymorphic pruritic dermatitis which showed same pathology as dermatitis herpetiformis. Thus, Duhring- Brocq’s disease is the synonym for dermatitis herpetiformis. The difference in pathology of bullous diseases from dermatitis herpetiformis was found in 1943 by Civatte. In 1967, a link between dermatitis herpetiformis was established[1][2].
References
Classification
Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]
Overview
Dermatitis Herpetiformis (also called Duhring’s disease), is a chronic itchy rash which is frequently associated with Celiac Disease. The rash is made of papules and vesicles which are present on different parts of the body mostly commonly on neck, trunk, buttocks and knees. It is an autoimmune mediated skin condition, which is IgA mediated reaction and is associated with gluten sensitivity of small bowel. There is presence of antibodies which leads to positive serology test results.
Dermatitis herpetiformis is associated with high prevalence of other autoimmune diseases.
Classification
Dermatitis herpetiformis is classified as an autoimmune disease which causes inflammation of the skin resulting in rash, there is no further classification into subtypes.
References
Pathophysiology
Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]
Overview
Dermatitis Herpetiformis (also called Duhring’s disease), is a chronic itchy rash which is frequently associated with Celiac Disease. The rash is made of papules and vesicles which are present on different parts of the body mostly commonly on neck, trunk, buttocks and knees. It is an autoimmune mediated skin condition, which is IgA mediated reaction and is associated with gluten sensitivity of small bowel. There is presence of antibodies which leads to positive serology test results.
Dermatitis herpetiformis is associated with high prevalence of other autoimmune diseases.
Pathophysiology
Dermatitis herpetiformis is rare autoimmune skin condition which is strongly associated with celiac disease. The development of the rash is explained by both genetic and environmental factors which initiate the immune response.
Genetics
Dermatitis herpetiformis is found to be associated with the HLA DQ2 or HLA DQ8 alleles. The same alleles are found in patients with celiac disease, this explains the strong associated between celiac disease and dermatitis herpetiformis. The presence of these alleles in an individual presenting with symptoms of celiac disease or dermatitis herpetiformis has 100% negative predictive value.[1]
Environment
Gluten in diet is the most significant in the development of the skin rash. Gluten consists of Gliadin and Gutenin, bother are peptides. Gliadin is the one which is responsible for the pathogenesis of the disease.
Immune response
The rash is developed due to the body’s immune response which causes inflammatory reaction on the skin. The antibodies formed against the antigen ‘ Gliadin’ and the presence of other autoantibodies like transglutaminases, the later are the most important in the pathogenesis of the disease.
References
- ↑ Clarindo MV, Possebon AT, Soligo EM, Uyeda H, Ruaro RT, Empinotti JC. Dermatitis herpetiformis: pathophysiology, clinical presentation, diagnosis and treatment. An Bras Dermatol. 2014;89(6):865-877. doi:10.1590/abd1806-4841.20142966
Causes
Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]
Overview
Dermatitis Herpetiformis (also called Duhring’s disease), is a chronic itchy rash which is frequently associated with Celiac Disease. The rash is made of papules and vesicles which are present on different parts of the body mostly commonly on neck, trunk, buttocks and knees. It is an autoimmune mediated skin condition, which is IgA mediated reaction and is associated with gluten sensitivity of small bowel. There is presence of antibodies which leads to positive serology test results.
Dermatitis herpetiformis is associated with high prevalence of other autoimmune diseases.
Causes
Dermatitis herpetiformis is an autoimmune skin manifestation of celiac disease. Approximately 10% patients suffering from celiac disease are affected by this rash, while more than 90% patients with the dermatitis herpetiformis eruptions are found to have gluten sensitive enteropathy which resembles to celiac disease. It is found that both genetic and environmental factors are the cause of development of the rash[1].
References
- ↑ Pekka Collin, Teea T. Salmi, Kaisa Hervonen, Katri Kaukinen & Timo Reunala (2017) Dermatitis herpetiformis: a cutaneous manifestation of coeliac disease, Annals of Medicine, 49:1, 23-31, DOI: 10.1080/07853890.2016.1222450
Differentiating Dermatitis herpetiformis from other Diseases
| Disease Name | Appearance | Causes | Symptoms | Distribution | Triggers |
|---|---|---|---|---|---|
| Atopic dermatitis | Eczematous Scalinge, Xerosis and Lichenification.
|
The cause can be genetic, personal or infectious.
(see Atopic dermatitis causes) |
Intense itching, scaling and thickening of the skin. | The distribution might vary with the age of patient.
In adults it involves the areas where skin flexes like elbows, behind the knees and in front of neck. |
Family or personal history of Atopy. |
| Scabies | Papules which can progress to Vesicles and Bullae. | Sarcoptes scabiei | Intense itching, redness and rash. | Webs of the fingers and toes.
Wrists. |
infested Fomite, usually beddings. |
| Bullous pemphigoid | Blisters all over the skin. | Autoimmune reaction to the underlying skin
structures called Hemidesmosome. |
Intense itching which precedes formation of blisters. | All over skin sparing mucous
membranes. |
|
| Pemphigus vulgaris | Flaccid Bullae all over the skin. | Autoimmune disease in which antibodies are
formed against Desmoglein. |
Intense itching and formation of flaccid blisters which might rupture and cause secondary infection. | All over the skin and mucous
membrane. |
|
| Linear IgA bullous dermatosis | Clinical pattern of bullae formation. | Autoimmune disease in which antibodies are
formed against basement membrane proteins like Lamina lucida and Sublamina densa. |
Intense itching , formation of papules, vesicles and bullae which might rupture. | All over the skin and mucous
membrane. |
Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]
Overview
Dermatitis Herpetiformis (also called Duhring’s disease), is a chronic itchy rash which is frequently associated with Celiac Disease. The rash is made of papules and vesicles which are present on different parts of the body mostly commonly on neck, trunk, buttocks and knees. It is an autoimmune mediated skin condition, which is IgA mediated reaction and is associated with gluten sensitivity of small bowel. There is presence of antibodies which leads to positive serology test results.
Dermatitis herpetiformis is associated with high prevalence of other autoimmune diseases.
Differentiating Dermatitis Herpetiformis from Other Diseases
Dermatitis herpetiformis is an intensely itching, blistering rash which might present with excoriations (see Dermatitis herpetiformis physical examination). It should be differentiated from all those skin conditions which presents with itching, blisters and excoriations.
References
Epidemiology and Demographics
Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]
Overview
Dermatitis Herpetiformis (also called Duhring’s disease), is a chronic itchy rash which is frequently associated with Celiac Disease. The rash is made of papules and vesicles which are present on different parts of the body mostly commonly on neck, trunk, buttocks and knees. It is an autoimmune mediated skin condition, which is IgA mediated reaction and is associated with gluten sensitivity of small bowel. There is presence of antibodies which leads to positive serology test results.
Dermatitis herpetiformis is associated with high prevalence of other autoimmune diseases.
Epidemiology and Demographics[1]
Incidence
The incidence ranges from ranges from 0.4 to 2.6 per 100.000 people per year, which is reported to be decreased while the incidence of celiac disease is increasing. Dermatitis herpetiformis is a rare complication pf celiac disease.
Prevalence
The highest prevalence is reported in Finland, while on the US and Europe it ranges from 11.2 to 75.3 per 100.000.
Age
Dermatitis herpetiformis occurs in adulthood in ages 30-40s. It does not occur commonly in children but few cases are reported.
Gender
Men are most commonly presented with dermatitis herpetiformis as compared to women with a ratio of 2:1.
Race
More prevalent in Caucasian population, it rarely occurs in African American and Asian populations.
References
- ↑ Antiga E, Maglie R, Quintarelli L, Verdelli A, Bonciani D, Bonciolini V and Caproni M (2019) Dermatitis Herpetiformis: Novel Perspectives. Front. Immunol. 10:1290. doi: 10.3389/fimmu.2019.01290
Risk Factors
Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]
Overview
Dermatitis Herpetiformis (also called Duhring’s disease), is a chronic itchy rash which is frequently associated with Celiac Disease. The rash is made of papules and vesicles which are present on different parts of the body mostly commonly on neck, trunk, buttocks and knees. It is an autoimmune mediated skin condition, which is IgA mediated reaction and is associated with gluten sensitivity of small bowel. There is presence of antibodies which leads to positive serology test results.
Dermatitis herpetiformis is associated with high prevalence of other autoimmune diseases.
Risk Factors
The most important risk factor in the development of this rash of dermatitis herpetiformis is presence of Gluten in the diet of genetically susceptible individuals. see Dermatitis herpetiformis pathophysiology
References
Natural History, Complications and Prognosis
Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]
Overview
Dermatitis Herpetiformis (also called Duhring’s disease), is a chronic itchy rash which is frequently associated with Celiac Disease. The rash is made of papules and vesicles which are present on different parts of the body mostly commonly on neck, trunk, buttocks and knees. It is an autoimmune mediated skin condition, which is IgA mediated reaction and is associated with gluten sensitivity of small bowel. There is presence of antibodies which leads to positive serology test results.
Dermatitis herpetiformis is associated with high prevalence of other autoimmune diseases.
Natural History
This chronic rash is rarely widely distributed. The vesicles on the rash are rare to be noticed because of intense itching which is relieved with scratching and rupture of the vesicles. The rash can be controlled with the use of antibiotic and gluten free diet.
Complications
Thyroid disease may be found in many patients with dermatitis herpetiformis. Patients are also more likely to develop certain cancers of the intestines.
Prognosis
The disease may be well controlled with treatment. Without treatment, there may be a significant risk of intestinal cancer.[1]
References
Diagnosis
Diagnosis
History and Symptoms | Physical Examination | Laboratory Findings | CT | MRI | Other Imaging Findings | Other Diagnostic Studies
Treatment
Treatment
Medical Therapy | Primary Prevention | Secondary Prevention | Cost-Effectiveness of Therapy | Future or Investigational Therapies
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