What is Dermatitis Herpetiformis ?
Dermatitis herpetiformis (DH) is a chronic, intensely itchy,
blistering skin manifestation of gluten sensitive enteropathy,
commonly known as celiac disease. DH affects 15 to 25 percent of
people with celiac disease. Most people with DH have no other
symptoms of celiac disease. DH is found mainly in adults and is
more common in men and people of northern European descent.
Symptoms
DH is characterized by small, clustered papules and vesicles that
erupt symmetrically on the elbows, knees, buttocks, back, or
scalp. Men may also have oral or genital lesions. A burning
sensation may precede lesion formation. Lesions are usually
scratched off by the time a patient comes in for a physical exam.
Causes
DH is caused by the deposit of immunoglobulin A in the skin,
which triggers further immunologic reactions resulting in lesion
formation. DH is an external manifestation of an abnormal immune
response to gluten, in which antibodies form against the
skin antigen epidermal transglutaminase.
Diagnosis
A skin biopsy is the first step in diagnosing DH. Direct
immunofluorescence of clinically normal skin adjacent to a lesion
shows granular deposits in the upper dermis. Histology of
lesional skin may show microabscesses containing neutrophils and
eosinophils but may reveal only excoriation due to the intense
itching patients experience.
Skin biopsies performed on the affected skin are nearly always
positive for deposition. Blood tests for antiendomysial or
anti-tissue transglutaminase antibodies may also suggest celiac
disease.
A positive biopsy and serology confirm celiac disease. In the
absence of these results, patients should be referred to a
gastroenterologist for a definitive diagnosis via intestinal
biopsy.
Treatment
Dapsone, a sulfone, provides immediate relief of symptoms. For
patients who cannot tolerate dapsone, sulfapyridine or
sulfamethoxypyridazine may be used, although these drugs are less
effective than dapsone. A strict gluten-free diet is the only
treatment for the underlying disease. Even with a gluten-free
diet, drug therapy may need to be continued for 1 to 2 years.
Healthy skin
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