Dühring's Disease

DÜHRING'S DISEASE, dermatitis herpetiformis Duhring's (synonyms: polymorphic dermatitis, polymorphic pemphigus, hydroa herpetiforme, herpes gestationis, herpes circinatus bullosus, herpes phlyctaenodes, dermatitis polymorpha dolorosa, dermatitis multiformis, hydroa pruriginosum, pemphigus pruriginosus, pemphigus circinatus), first described in 1885 by Dühring as a skin disease characterized by 1) chronic recurrent course, 2) polymorphism of skin manifestations (presence of vesicles, blisters, spots, pustules, etc.), 3) almost constant itching sensation, and 4) minimal effect on general health. There are some disagreements regarding the nature of this disease and its relationship to pemphigus, chronic urticaria, erythemas, etc. For many years, a number of authors [especially the Vienna school (Kaposi) and to some extent the German school] doubted whether D. d. should be distinguished as a separate nosological entity. Kaposi identifies D. d. with chronic pemphigus. Representatives of the French school (Brocq), as well as the Russian school (Nikolsky), on the contrary, argued for the independence of this disease, with Brocq expanding the concept of D. d., noting that in it the rash does not always have a herpetiform character. Brocq recommends including D. d. in a broad syndrome which he calls dermatitis polymorpha. The etiology and pathogenesis have not yet been clarified. Lipschutz, Bogolepov and others adhere to the infectious theory, based on results of positive bacterial findings in the examination of blister contents. However, the microorganisms found by different authors are very diverse and are not specifically characteristic of this disease. Experiments on animals have also been inconclusive to date. Others, considering that Dühring's disease is most often observed in hysterical and neuropathic individuals and that changes in various parts of the central and peripheral nervous systems were found in this disease, tend to consider D. d. as an angiotrophoneurosis. Others seek to explain the various manifestations of the disease by endogenous intoxication, mainly from the gastrointestinal tract, believing that nervous symptoms are secondary. Many authors emphasize the etiological significance of pregnancy and the special sensitivity of these patients to certain medicinal substances, for example to KI. This fact was even attempted to be used for differential diagnosis between D. d. and pemphigus (Darier). In addition, many authors have found eosinophilia in the blood (and in blisters) in D. d. This gave Lered the basis to express the idea that D. d. is a blood disease and eosinophilia is the result of irritation of the bone marrow, which can be caused by various toxic substances. Finally, some authors put forward endocrine disorders, especially of the thyroid gland, as an etiological factor. Pathological anatomy. Histopathological changes in the skin manifestations of D. d. do not represent anything characteristic and generally correspond to the morphological nature of those elements (blisters, spots, wheals, pustules, etc.) from which the rash of each individual attack is composed. The most common element of the rash in D. d. is a blister, lying either under the horny layer or deeper, sometimes at the border of the epidermis and dermis; while in addition to diffuse infiltration of the epidermis with leukocytes, moderate lymphocytic and leukocytic infiltration of the papillary and subpapillary layers of the skin, fibrinous exudate, ballooning degeneration and usually significant accumulation of eosinophils are found in the center and on the bottom of the blister. Skin appendages are usually unchanged. Course of the disease and symptomatology. The course of D. d. is usually chronically recurrent; the disease with known interruptions lasts for many years (sometimes until death). The intervals between individual attacks vary greatly - from 6 weeks to 3 months and even up to 1 year. The disease most often begins in young and middle age, but also often in childhood: thus, according to Petrov's data, D. d. was observed up to 7 years in 35 cases, and between 7 and 14 years in 40 cases, with boys getting sick in 57% and girls in 43%. In some cases, the disease can proceed more acutely, limited to one attack. The rash in D. d. usually appears suddenly, without any prodromal symptoms; often it is preceded by general malaise, slight increases in temperature and sensations of tingling and itching in the skin, especially in the upper and lower extremities. The rash is located symmetrically, first in the area of the forearms, then spreading to the extremities and trunk. Palms and soles, as well as the skin of the head, are usually free from rashes. Sometimes the rash is abundant, immediately acquiring a diffuse character, in other cases it appears gradually in the form of a series of successive outbreaks. The rash begins with the appearance of an itchy, indistinctly shaped diffuse spot, which however, either through the stage of a wheal or directly, passes into a blister. In other cases or in other places, a blister, usually surrounded by a reddish border, can also arise directly on unchanged skin. The size of the blisters varies greatly. Sometimes small vesicles appear, tending to group in the manner of simple herpes (hence the name d. herpetiformis), sometimes larger elements, as in pemphigus [see separate table (pp. 107-108), Fig. 3]. Usually the size of a blister is equal to a cherry stone or a pea. The blisters, initially yellowish-transparent, quickly become cloudy, partially turning into pustules, in other places the elements of the rash immediately appear in the form of pustules. Under the influence of itching, friction or spontaneously, the blisters and vesicles rupture, causing slight weeping and turning into superficial erosions, which in turn are very quickly covered with yellowish-gray or blood crusts. All the listed elements or various combinations of them usually exist simultaneously, and therefore the rash in D. d. takes on a clearly polymorphic character. In other cases, during a given attack, the rash may be monomorphic, but each subsequent attack is characterized by elements of a different morphological type. The mucous membranes are also often affected, especially the oral cavity (according to Darier, in ^1/2 of all cases). In the area of the genital and adjacent parts, vegetations are occasionally observed at the sites of former blisters. These vegetations are indistinguishable in appearance from the phenomena of pemphigus vegetans. The rash in D. d. causes painful sensations and paresthesias in the form of itching, burning, tingling, etc. Itching is particularly strong, which almost never disappears and sometimes becomes unbearable, sometimes persisting long after the rash disappears. In general, however, despite the chronic course and subjective disorders, the disease has very little effect on general health. Temperature increases to 38-39° are far from always, more often during outbreaks of the rash. Usually at this time a decrease in urea in the urine (Nikolsky) and retention of chlorides (Poppe) are also observed. D. d. as a rule has a favorable course. Diagnosis and prognosis. The diagnosis of the disease in pronounced cases is not difficult. In mild and abortive forms, a group of bullous and polymorphic erythemas should be considered, in severe (especially with vegetations) - pemphigus, to which D. d. is very close. The distinctive signs in favor of D. d. should be: polymorphism of the rash, especially the presence along with blisters of urticarial elements, herpetic grouping of vesicles, constant presence of severe itching and mainly the favorable nature of the course process. Eosinophilia in the blood and blisters, noted in D. disease by a number of authors (Pautrier, Bogrov, etc.) as a differential diagnostic sign, is not reliable. - The prognosis for D. d. in terms of duration of life and disorders of general health is favorable. Fatal cases are observed as the greatest rarity.--Treatment. No reliable therapeutic means exists. Symptomatically, arsenic in usual prescriptions or in the form of Novarsan sometimes works well, especially with the simultaneous administration of 2-2.5 g of quinine per day (Darier), as well as, according to Lebedev, intravenous injections of quinine in a dose of 0.3-1.5 in 0.85% NaCl. With variable success, autogenous blood therapy (which often temporarily stops itching), serotherapy, lactotherapy and calcium chloride, as well as thyroidin, have been tried. In some cases, ultraviolet rays and heliotherapy (Meshchersky) have been successfully applied. Externally - indifferent powders (Amylum, Zinc. oxyd. aa), ointments (Ung. boric, Ung. Xeroformii 2% etc.), baths (t° 35°), sometimes with the addition of Kalii hypermang. until pink color. Rational prevention does not exist due to the unclear nature of the disease.