Dermatitis Herpetiformis.
(Synonyms: Hydroa Herpetiforme (Tilbury Fox); Herpes Gestationis (Bulkley); Pemphigus Prurigiuosus; Duhring's Disease.)
Give a definition of dermatitis herpetiformis.
Dermatitis herpetiformis is a somewhat rare inflammatory disease, characterized by an eruption of an erythematous, papular, vesicular, pustular, bullous or mixed type, with a decided disposition toward grouping, accompanied by itching and burning sensations, with, as a rule, more or less consequent pigmentation, and pursuing usually a chronic course with remissions.
Describe the erythematous type of dermatitis herpetiformis.
The character of the eruption in the erythematous type resembles closely that of erythema multiforme and of urticaria, especially the former. The efflorescences usually make their appearance in crops, and are more or less persistent; fading sooner or later, however, and giving place to new outbreaks. Vesicles are often intermingled, developing from erythematous and erythemato-papular lesions or arising from apparently normal skin.
It may continue in the same type, or change to the vesicular, bullous or other variety.
Describe the papular type of dermatitis herpetiformis.
This is rarely seen as consisting purely of papular lesions, but is commonly associated with the erythematous and vesicular varieties. In a measure it resembles the papular manifestations of erythema multiforme, with a distinct disposition toward group formation. The papules tend, sooner or later, to develop into vesicles, new papular outbreaks occurring from time to time; or the whole eruption changes to the vesicular or other type of the disease. It is not a common type.
Describe the vesicular type of dermatitis herpetiformis.
This is the common clinical type of the disease, and is characterized by pin-head to pea-sized, rounded or irregularly-shaped, distended or flattened and stellate vesicles, occurring, for the most part, in irregular and segmental groups of three or more lesions, seated either upon apparently normal integument or upon hyperæmic or inflammatory skin. They exhibit no tendency to spontaneous rupture, but after remaining a shorter or longer time, are broken or disappear by absorption. The lesions tend to appear in crops. It may, as it not infrequently does, continue in the same type, or it may become more or less erythematous or bullous in character. In not a few instances pustules, few or in numbers, are at times intermingled.
Describe the pustular type of dermatitis herpetiformis.
This is rare. It is similar in its clinical characters to the vesicular type, except that the lesions are pustular. It is met with, as a rule, in association with the vesicular and bullous varieties of the disease.
Describe the bullous type of dermatitis herpetiformis.
The bullous expression of the disease is usually of a markedly inflammatory nature, often innumerable blebs, small and large, appearing almost continuously, and in some instances involving the greater part of the surface. The lesions arise from erythematous skin, from preëxisting vesicles or vesicular groups, or from apparently normal integument. There is a marked disposition to appear in clusters. A change of type to the erythematous or vesicular varieties is not unusual.
Describe the mixed type of dermatitis herpetiformis.
In this type the eruption is made up of erythematous patches, vesicles, bullæ, and often with pustules intermingled, appearing irregularly or in crops, and with a tendency to patch or group formation.
Describe the characters of the vesicles, pustules and blebs.
As a rule, these several lesions, especially the vesicles and blebs, are somewhat peculiar: they are usually of a strikingly irregular outline, oblong, stellate, quadrate, and when drying are apt to have a puckered appearance. They are herpetic in that they show little disposition to spontaneous rupture, occur in groups, and are usually seated upon erythematous or inflammatory skin—in some respects similar to the groups of simple herpes and herpes zoster.
What is to be said in regard to the subjective symptoms?
The subjective symptoms are usually the most troublesome feature of the disease, consisting of intense and persistent itching and a feeling of heat and burning.
Are there any constitutional symptoms in dermatitis herpetiformis?
As a rule, not, excepting the distress and depression necessarily consequent upon the intense itchiness and loss of sleep. In the pustular and bullous varieties there may be mild or grave systemic symptoms, but even in these types the constitutional involvement is, in most instances, slight in comparison to the intensity of the cutaneous disturbance.
What is the course of dermatitis herpetiformis?
Extremely chronic, in most instances lasting, with remissions, indefinitely. The skin is rarely entirely free. From time to time the type of the disease may undergo change. From the continued irritation and scratching more or less pigmentation results.
What is to be said in regard to the etiology?
The disease is in many instances essentially neurotic, and in exceptional instances septicæmic. Pregnancy and the parturient state are factors in some instances (so-called herpes gestationis). It is possible in some instances that the eruption may be an expression of a mild toxemia of gastro-intestinal origin. In some cases no cause can be assigned. In the majority of patients the general health, considering the violence of the eruptive phenomena, remains comparatively undisturbed.
Nervous shock and mental worry are factors in some cases. Polyuria, with sugar in the urine, has occasionally been noted. Eosinophile cells have been found both in the vesicles and the blood. In some instances—exceptionally, it is true—the disease has appeared shortly after vaccination.
Mention the diagnostic features of dermatitis herpetiformis.
The multiformity of the eruption, the characters of the lesions, the disposition to grouping, the absence of tendency to form solid sheets of eruption (as in eczema), the intense itching, history, chronicity and course. In doubtful cases, an observation of several weeks will always suffice to distinguish it from eczema, erythema multiforme, herpes iris and pemphigus, diseases to which it at times bears strong resemblance.
Give the prognosis of dermatitis herpetiformis.
An opinion as to the outcome of the disease should be guarded. It is exceedingly rebellious to treatment, and relapses are the rule. Exceptionally the bullous and pustular varieties prove eventually fatal. The erythematous and vesicular varieties are the most favorable.
State the treatment to be advised.
There are no special remedies. Constitutional treatment must be conducted upon general principles. A free action of the bowels is to be maintained. In occasional instances arsenic in progressive doses seems of value. Externally protective and antipruritic applications, such as are employed in the treatment of eczema and pemphigus, are to be employed:—
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Other valuable applications are: lotions of carbolic acid, of liquor carbonis detergens, of boric acid; alkaline baths, mild sulphur ointment and carbolized oxide-of-zinc ointment, and dusting-powders of starch, zinc oxide, talc and boric acid. A two- to ten-per-cent. ichthyol lotion or ointment is sometimes of advantage; thiol employed in the same manner has also been commended.