(Duhring’s disease; hydroa; Herpes gestations)
Definition. A rare, chronic, inflammatory disease of the skin, characterized by a mixed eruption of grouped macules, papules, vesicles, bullae and pustules, usually associated with marked subjective sensations.
Symptoms. More or less well defined constitutional disturbances such as malaise, constipation, chills or fever may precede the onset of the eruption, or its various aggravation. In a few cases, the systemic disturbances is very slight; it may even be absent, or it may continue for days after the eruption appears. More or less itching is felt in the skin for a few hours or days before the appearance of the eruption, or the latter may occur suddenly without any local warning. It is bilaterally and often symmetrically located, favoring the flexor surfaces of the wrists, forearms, abdomen, buttocks, and outer part of the thighs, but it may be generalized or limited without rule to any region. The lesions tend to occur in groups of two or more, and singly or combined in bead- like lines form unusual and varied shapes with a marked tendency toward incomplete, irregular or multiple circle formations. While there is a distinct tendency for one variety to pass into another, the commonest types are the vesicular, bullous, erythematous (macular), pustular, papular and multiform (mixed variety). Itching and burning may be a slight or marked symptom in any or all of these varieties. the course of the disease is essentially chronic, lasting for years in the form of relapses, or it may be continuous, and often a few lesions will persist indefinitely during intervals of comparative relief.
The vesicular form is the earliest as well as the most common and characteristic type of the disease. It presents pin-head to pea-sized, flat or raised, distended vesicles. These may be irregularly shaped, oblong, angular, stellate, or rarely rounded, and usually have an inflammatory areola. Primarily, they are aggregated in clusters of two or more lesions, which usually coalesce, but do not rapture spontaneously. The manifestation is essentially one of crops succeeding each other with great rapidity but, in exceptional cases, there may be a remission of weeks or months. When the outbreaks are frequent and the itching intense, the skin is likely to become infiltrated and excoriations ensue which in turn cause slight pigmentation.
The bullous form may develop from vesicles, erythematous patches, or arise from a sound skin. the bullae occurring in groups of three or more, often without areola, are distended with clear, milky, seropurulent fluid and are irregularly shaped. Within a few days, they are likely to be ruptured by scratching or otherwise broken and then dry into yellowish brown crusts. Pustular, vesicular and erythematous lesions are likely to be present, with severe burning and itching.
The erythematous form may be primary or secondary to other types of the disease. It first appears in small or large pinkish patches, which later become yellowish red. These later become sharply defined or merge into the skin. Often they are mingled with papules and wheals, sometimes with edematous swelling. The eruption tends to appear in groups, to persist and is widely distributed. The resemblance to erythema multiforme may be exceedingly close. Often these groups are associated with papules to such a degree that it is known as the erythematopapular form.
The Pustular form is very rare and approaches in clinical type impetigo herpetiformis. The lesions may consist of small, pin-head to pea-sized, flat pustules or elevated, rounded or acuminated types, situated upon an inflammatory base. Vesicles and blebs often complicate the pustules although the type is fairly constant through successive outbreaks. The pustules mature in from five to ten days, but new lesions arise, singly or in crops, and may prolong the attack for weeks or indefinitely. Aside from these features there is less tendency to grouping and diversity of arrangement of lesions than in the vesicular form. The course, however, is very severe, indication a graver general condition, and usually attended with greater systemic disturbance.
The papular form is the mildest form but it rarely exists alone or even characterized the general disease, except in association with erythematous or vesicular types.
The mixed form (multiform variety) is frequently a transitional variation from one of the foregoing forms to another, or it may be a polymorphous demonstration of erythema, papules, vesicles, pustules, blebs and pigmentation. This type, as a distinct entity, seldom persists.
A type of this disease which appears during or after pregnancy is known as herpes gestationis and has a great tendency to occur at each successive pregnancy with increasing severity and finally to assume the course of other forms of dermatitis herpetiformis.
Etiology and Pathology. This disease attacks both sexes and is most often found between the thirtieth and fiftieth years of life. Its neurotic origin is shown by the fact that antecedent nervous prostration, shock, worry, pregnancy, or mental, menstrual, gastrointestinal and renal disorders, or exposures precede it. Any cause which sufficiently disturbs the nutritive processes in the skin may give rise to it without much or any systemic disturbance. Winfield has reported a number of cases associated with glycosuria and a few fatal cases have been recorded as due to septicemia.
Pathological studies show an acute, inflammatory conditions of the corium, especially of the papillary layer, with the formation of vesicles between the corium and the epidermis and exudation of large numbers of leucocytes and eosinophiles. The lesions of dermatitis herpetiformis are nearly always superficial, never ulcerate or leave scars unless there is secondary infection present. Pigmentation, if it follows, is probably never permanent.
Diagnosis. In eczema, the vesicles are smaller, more regular in shape, less aggregated rupture sooner and give rise to continuous discharge and crusting. the papules of eczema are also smaller, regular in size and shape and do not tend to appear in groups. The blebs of pemphigus usually arise from sound skin, are larger and more regular in outline, do not show a tendency to become grouped or commingle with other lesions, and cause little itching. Herpes zoster shows a limited distribution, neuralgic pain and unilateral location. Erythema multiforme may appear much like an erythematopapular form of dermatitis herpetiformis but is usually limited to the back of hands, arms and the face, is not attended with acute itching and runs an acute course of ten to twenty days. Its vesicles or bullae are always secondary to its erythema which is not the case in dermatitis herpetiformis. Urticaria with its sudden eruption of ephemeral wheals, marked sensations of stinging and itching, without tendency to become grouped or arranged in broken or complete circles, and the absence of multiform lesions, should not be mistaken.
Prognosis is always guarded. While there is little danger as far as life is concerned, because the general health is not affected except in the more severe cases, the capriciously chronic nature of the disease and its tendency to recur should always be noted. If an estimate may be given from may own experience, two-thirds of the case recover; the fatal ones occurring among the pustular or bullous types, or in the very old or debilitated.
Treatment. The underlying cause of the nervous derangement should be discovered and removed, if possible. Hence, physiological methods are to be instituted as soon as indications for the same may be discovered. Benefit will be frequently experienced by a change from an animal to a vegetable diet, and the prohibition of such nerve disturbers as coffee, tea, alcoholic stimulants and tobacco. Local treatment is unnecessary except to promote cleanliness and relieve subjective sensations. Hot alkaline and saline baths, frequently of twenty to forty minutes, or even a continuous bath in aggravated cases, as recommended for pemphigus, may be useful. When the blebs have formed, they may be punctured to relieve the itching and a mild boric acid ointment form may prove beneficial but in the ordinary case lotions containing tar, such as liquor carbonis detergens (1 to 10 parts to 100 of water), carbolic acid, potassium permanganate (1:2000), ichthyol and resorcin are more serviceable. The general application of galvanic or high-frequency currents can be highly recommended.
An internal remedy prescribed on the very broadest basis, is of material assistance. The following may be studied: Ant. tart., Carbol. acid., Clematis, Croton tig., Iris vir., Kali brom., K. iod., LAch., Nat. mur., Phosphorus, Ranun. bulb., Rhus tox., Sepia, Silicea, Tellurium.