Dermatitis Herpetiformis

    Dermatitis herpetiformis [DH] is associated with gluten-sensitive enteropathy [celiac disease] and is characterized by a chronic, intensely pruritic papulovesicular rash symmetrically distributed over exterior surfaces of the body, the trunk, buttocks, scalp, and neck.  There is strong evidence that the changes in the intestinal mucosa and the immunologic findings in the majority of patients are identical to those found in celiac disease.  Second, gluten has been found to be closely related to the skin rash that occurs with dermatitis herpetiformis.   Informally, gluten-sensitive enteropathy is often referred to as celiac disease of the gut and dermatitis herpetiformis is referred to as celiac disease of the skin.  

    In the literature, dermatitis herpetiformis may be referred to as Duhrings' disease; a herpes circinatur bullosus; or as a hydroa herpetifome.  DH is a chronic disease of the skin marked by a severe, extensive itching eruption of vesicles and papules which occur in groups.  A spontaneous healing of the skin rarely occurs except in children; relapses are common--especially in adults.  It was in 1960 that physicians caring for disorders of the skin discovered that a particular type of itchy rash called dermatitis herpetiformis may also be associated with the atrophy of the villi and usually responds to the gluten-free diet.  

    The histology of the intestinal lesion is virtually identical to that seen in celiac disease, although villous atrophy and inflammatory infiltrate are generally milder and often clinically insignificant in DH.  Administration of dapsone or other sulfones often relieves the skin itching associated with the skin lesions of dermatitis herpetiformis.  

    Diagnosis of dermatitis herpetiformis depends on the presence of the characteristic skin lesions and demonstration of IgA deposits in the skin.  Although many patients have minimal or no gastrointestinal complaints, an intestinal biopsy generally reveals intestinal involvement.  

    Elimination of all gluten from the diet generally leads to resolution of skin symptoms as well as a normalization of intestinal findings, but typically over an extended period of several months.  Administration of sulfones, the mainstay therapy for dermatitis herpetiformis, leads to a rapid resolution of the skin symptoms;  sulfones, however,  have virtually no effect on the intestinal symptoms.  For the intestinal symptoms, the DH patient must follow a strictly regimented prescription gluten-free diet carefully adapted to their needs and to their level of the illness.  Authors McNeish, et al, stated that because there are no means to predict future responses to gluten for the DH patient, the safest advice to persons with diagnosed conditions of celiac disease and dermatitis herpetiformis must be to adhere to a strict gluten-free diet indefinitely.

    The unusual interrelationship of dermatitis herpetiformis and celiac sprue is particularly interesting.  Many DH patients have mild intestinal lesions but have no intestinal symptoms.  In some few DH patients with normal intestinal biopsies, a mucosal lesion can be induced with high intake of gluten.  Less that 10 percent of patients with celiac disease have evidence of dermatitis herpetiformis.  Thus, dermatitis herpetiformis and celiac disease appear to be distinct diseases with the curious relationship that over 80 percent of patients with the skin disease [DH] also have at least latent celiac disease, whereas only relatively few patients with celiac disease have dermatitis herpetiformis.

    Strict gluten withdrawal eventually reverses both the intestinal lesions of celiac disease and the skin lesions of dermatitis herpetiformis.  But, the treatment of the skin lesions with sulfones fails to reverse the lesions in the gut representing celiac disease.  It is important to note that in dermatitis herpetiformis, the prevalence of HLA-B8, HLA-DR3, and HLA-DQw2 and circulating antibodies to gliadin peptides is the same as those observed in patients with celiac disease without the skin disease, dermatitis herpetiformis.

    The use of pharmaceuticals and nutritional products often represents an important area of self-care for both the celiac and DH patient.  While the percentage of pharmaceutical products containing  gluten is small, gluten is used as an inert excipient in tablets, capsules, and suspensions for both oral and rectal products and as a wheat starch sealer.  The central message is that both over-the-counter and prescription medications may contain gluten or by-products of gluten and may then have the potential to trigger a  toxic reaction for the DH patient.  Some patients may need to make use of selected non-allergenic products [available at most pharmacies] such as soaps and shampoos for the shower and detergents for the laundry. Before using a hand lotion [with a grain derivative], a face powder [with a wheat starch extender], a lipstick [with oat gum], a shampoo [with wheat germ oil], a mouthwash [with a grain derivative], a toothpaste [with gluten]; or products with any array of colorings based on grains, dyes based on lakes and other glutens such as wheat germ oil, wheat flour or barley water, the patient must check with their pharmacist or physician for information and direction.  Careful label reading and interpretation of terms on labels along with obtaining information regarding sealers, extenders, and non-identified ingredients will help the DH patient to develop a listing of allowed pharmaceuticals and nutritional products.

Source:  Sleisenger and Fortran, Gastroenintestinal Disease: Pathophysiology/Diagnosis/Management, W.B. Saunders Co., Fifth Edition.  

 

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Updated on 02.08.02   webmaster@e-celiacs.org