Dermatitis herpetiformis

Dermatitis herpetiformis Duhring is a disease described by L. A. Duhring in 1884. Recently, it has become the most common manifestation of gluten enteropathy (GE) or celiac disease.

General characteristics

 * It is not an associated disease or complication of celiac disease, but an equivalent form of GE manifestation, with the same pathogenesis, treatment, prognosis and possibilities of complications.Dermatitis-herpetiformis.jpg
 * as considered a purely skin disease for a long time, until 1966, when it was found that the mucosa of the small intestine in up to ¾ of the patients shows the same functional and morphological changes as in celiac disease.
 * after the proof of the beneficial effect of a gluten-free diet not only on intestinal but also on skin changes, a certain connection began to be assumed and the names of pre-celiac disease, cutaneous celiac disease etc. were used.
 * both forms, celiac sprue (CS) and dermatitis herpetiformis (DH), are completely independent of each other, can occur simultaneously and have qualitatively identical laboratory, histological and functional deviations.
 * a significant and still unexplained fact is that the incidence of DH has clearly increased in recent years and is shifting to increasingly younger age groups.Micrograph of dermatitis herpetiformis.jpg

Clinical picture

 * Characteristic is the skin finding, which corresponds to the name - it is an eruption of typical strongly itchy blisters of herpetiform appearance:
 * individually and in groups,
 * different sizes and stages of development,
 * with predilection localization on limb extensors, on the torso, buttocks and in the buttocks,
 * most patients have no internal problems (about 20% report intermittent "diarrhea"),
 * physical findings usually remain within normal limits, without significant asthenia, muscle hypotrophy or other signs malabsorption,
 * in laboratory findings it is possible to find:
 * mild degree anemia,
 * reduction in beta-carotene and serum iron,
 * positivity antibodies against gliadin, endomysia and tissue transglutaminase,
 * in direct contrast, however, there are clear '''changes in the' 'intestinal mucosa' ', detectable in 70–80% of patients and identical to changes in CS:
 * atrophy of villi,
 * inflammatory lymphoplasmocytic infiltration,
 * edema of lamina propria,
 * reduction of enterocyte enzymatic equipment.

Diagnosis

 * It is determined by a dermatologist according to the clinical findings, possibly with confirmation of a skin biopsy,
 * all patients must be followed by a gastroenterological examination with an enterobiopsy and a degree of disability,
 * there is no relationship between the extent of skin changes, mucosal changes and subjective problems,
 * monitoring of antibody levels is suitable for monitoring the course of the disease → values ​​decrease after the introduction of a gluten-free diet and increase again when it is broken or when the disease relapses,
 * laboratory signs of possible malabsorption are insignificant, as mucosal changes in DH (as opposed to CS) may be focal in nature.

Therapy

 * in the long term, patients with DH are at risk of the same serious complications, especially the increased risk of developing malignant tumors,
 * the skin finding (not the intestinal!) subsides after the administration of DDS-Sulfones → but after their discontinuation it quickly recovers
 * due to a number of possible side effects (methemoglobinemia) it should be given at the lowest effective dose,
 * the only causal treatment is a permanent and complete gluten-free diet,
 * adherence to it improves (after a number of months) mucosal and skin changes, adjusts biochemical parameters and minimizes the risk of complications,
 * the introduction of a gluten-free diet in our conditions represents not only a significant impact on personal and professional life, but also requires increased financial demands and in the usual absence of problems, the motivation to follow it in patients with DH is clearly lower than in CS,
 * However, the course, risks and possible complications are the same and demonstrate the need for continuous monitoring by a gastroenterologist.

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