Hospital Medicine

Dermatitis herpetiformis

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Dermatitis herpetiformis

I. What every physician needs to know.

Dermatitis herpetiformis is an autoimmune blistering (papules and vesicles) skin condition associated with a gluten-sensitive enteropathy (celiac disease).

II. Diagnostic Confirmation: Are you sure your patient has dermatitis herpetiformis?

Dermatitis herpetiformis is a skin condition characterized by extremely itchy papules and vesicles usually on the extremities and trunk. It is often associated with a gluten-sensitive enteropathy (celiac disease). The enteropathy is asymptomatic in most patients. It is characterized by papules with vesicles and erythematous plaques, both of which are pruritic. Because of the extreme pruritic nature of the condition, one also often sees excoriation superimposed on the primary lesions. In fact, the primary lesions of dermatitis herpetiformis (papules, vesicles) are often replaced by excoriations by the time a patient presents for medical attention. The usual location is on the extensor surfaces of the extremities and on the trunk. Some patients can also have lesions on their scalp. Mucosal involvement is rare. Diagnosis can be confirmed by skin biopsy. It is usually a life-long condition, however, the clinical course of skin disease is variable with presentations ranging from periodic eruptions to continuous symptoms that wax and wane.

A. History Part I: Pattern Recognition:

Exquisitely pruritic, grouped skin papules and vesicles located on extensor surfaces of the extremities, the trunk, scalp, and occasionally buttocks. It is usually seen in adults aged in their 20-40s. It rarely involves the mucosa. Often, the primary lesions are replaced by excoriations from patient scratching.

B. History Part 2: Prevalence:

The prevalence has not been studied extensively but is estimated at 11.2 cases per 100,000 population. It occurs most frequently in those of northern European ancestry with the incidence in this population estimated at rates between 0.4-3.5 per 100,000 per year. It is typically occurs in adults aged 20-40s and is rare in children. However, it can occur in individuals of any age. It is slightly more common in men than women.

It is most common among patients with celiac disease (thought to be related to the shared human leukocyte antigen (HLA) associations).

C. History Part 3: Competing diagnoses that can mimic dermatitis herpetiformis.

Conditions that mimic dermatitis herpetiformis are linear immunoglobulin A (IgA) bullous dermatosis, bullous pemphigoid, bullous lupus, urticaria, atopic dermatitis, contact dermatitis, arthropods bites, and scabies.

Dermatitis herpetiformis can be distinguished from these conditions by its clinical appearance and skin biopsy findings.

D. Physical Examination Findings.

Papules and vesicles located on extensor skin surfaces of the extremities, trunk, scalp and/or buttocks. These can have overlying excoriations.

E. What diagnostic tests should be performed?

Physical examination and skin biopsy should be done. Routine histopathology of a skin biopsy are often suggestive of the diagnosis. Direct immunofluorescence examination of a skin biopsy of a lesion usually confirms the diagnosis.

1. What laboratory studies (if any) should be ordered to help establish the diagnosis? How should the results be interpreted?

Skin biopsy is the primary diagnostic test. Serologies including IgA anti-tissue transglutaminase (anti-tTG IgA) antibodies and antiendomysial antibodies are often elevated in patients with this condition (similar to celiac disease) although these are not necessary to make the diagnosis. They can be useful in monitoring adherence to a gluten-free diet.

2. What imaging studies (if any) should be ordered to help establish the diagnosis? How should the results be interpreted?

None, although one may want to photograph the lesions to help with diagnostic evaluation.

F. Over-utilized or “wasted” diagnostic tests associated with this diagnosis.

NA

III. Default Management.

A gluten-free diet should be initiated and continued indefinitely. This diet is often recommended based on the results of the skin biopsy alone. Testing for celiac is not required but can be considered.

Many patients have a good clinical response to oral dapsone therapy which first helps with the itching and then with the lesions themselves. However, complete resolution of the condition usually does not occur without initiating a gluten-free diet.

A. Immediate management.

Start gluten-free diet.

B. Physical Examination Tips to Guide Management.

Follow-up for resolution of the skin lesions once a patient starts a gluten-free diet.

C. Laboratory Tests to Monitor Response To, and Adjustments in, Management.

No laboratory tests are typically followed although serologic testing (anti-tTG, anti-endomysial IgA, immunoglobulin G (IgG)) can be used to monitor adherence to the gluten-free diet. The only exception is if the patient is started on dapsone for symptomatic relief. If on dapsone, regular laboratory testing (complete blood count, renal panel, liver function tests) should be undertaken to monitor for signs of dapsone toxicity.

Consider testing for thyroid function abnormalities as hypothyroidism is the most commonly associated autoimmune disease after celiac disease.

D. Long-term management.

Lifelong gluten-free diet.

E. Common Pitfalls and Side-Effects of Management

The major risk of treatment relates to any use of dapsone for the short-term management of symptoms. The dosing of this drug should be reduced to as low as possible and removed as soon as symptoms improve. Regular lab monitoring should be undertaken for dapsone toxicity. If lesions are not resolving, consider lack of adherence to gluten-free diet.

IV. Management with Co-Morbidities

A. Renal Insufficiency.

Only an issue if patient given dapsone.

B. Liver Insufficiency.

No change in standard management.

C. Systolic and Diastolic Heart Failure

No change in standard management.

D. Coronary Artery Disease or Peripheral Vascular Disease

No change in standard management.

E. Diabetes or other Endocrine issues

No change in standard management.

F. Malignancy

Patients with dermatitis herpetiformis may be at higher risk of non-Hodgkin lymphoma (specifically enteropathy associated T cell lymphoma).

G. Immunosuppression (HIV, chronic steroids, etc).

No change in standard management.

H. Primary Lung Disease (COPD, Asthma, ILD)

No change in standard management.

I. Gastrointestinal or Nutrition Issues

Patients may also have underling celiac disease or gluten enteropathy. Consider testing for celiac disease if patient also has any suggestive malabsorption symptoms, unexplained anemia, liver function abnormalities, or bone density issues at a young age.

J. Hematologic or Coagulation Issues

No change in standard management.

K. Dementia or Psychiatric Illness/Treatment

No change in standard management.

V. Transitions of Care

A. Sign-out considerations While Hospitalized.

Not applicable, unlikely to be hospitalized for this condition.

B. Anticipated Length of Stay.

NA

C. When is the Patient Ready for Discharge.

NA

D. Arranging for Clinic Follow-up

Patient should be followed in dermatology and/or primary care to ensure appropriate diagnosis, adherence to gluten-free diet, and resolution of skin lesions. Nutrition referral could be considered if the patient is having difficulty with a gluten-free diet. Consider screening for celiac disease and/or hypothyroidism if relevant symptoms are present.

1. When should clinic follow up be arranged and with whom.

Arrange follow-up with dermatology and with a nutritionist on an as-needed basis.

2. What tests should be conducted prior to discharge to enable best clinic first visit.

NA

3. What tests should be ordered as an outpatient prior to, or on the day of, the clinic visit.

NA

E. Placement Considerations.

NA

F. Prognosis and Patient Counseling.

Adherence to a gluten-free diet is critical for resolution of lesions. The gluten-free diet must be adhered to for life.

VI. Patient Safety and Quality Measures

A. Core Indicator Standards and Documentation.

NA

B. Appropriate Prophylaxis and Other Measures to Prevent Readmission.

NA

VII. What's the evidence?

Bolotin, D, Petronic-Rosic, V. "Dermatitis herpetiformis: Part I. Epidemiology, pathogenesis, and clinical presentation". J Am Acad Dermatol.. vol. 64. 2011. pp. 1017-1024.

Bolotin, D, Petronic-Rosic, V. "Dermatitis herpetiformis: Part II. Diagnosis, management, and prognosis". J Am Acad Dermatol.. vol. 64. 2011. pp. 1027-1033.

Alsonso-LLamazares, J, Gibson, LE, Rogers, RS. "Clinical, pathologic, and immunopathologic features of dermatitis herpetiformis: review of the Mayo Clinic experience". Int J Dermatol. vol. 46. 2007. pp. 910.

Burrows, D.. "The prevalence of dermatitis herpetiformis". Br J Dermatol. vol. 86. 1972. pp. 437.

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