A system of practical medicine. By American authors. Vol. 4

by Unknown

This disease is multiform and protean in character, consisting in the formation of herpetic, erythematous, vesicular, pustular, and bullous lesions, occurring separately or in various combinations, accompanied with itching and burning sensations and pursuing usually a chronic course with relapses.

This affection, which until recently has been confounded with other cutaneous diseases, is rare, although as its peculiar features become belter known numerous cases will doubtless be reported. It was first described by one of us (Duhring) in a paper read before the American Medical Association in 1884. It is an inflammatory disease of an herpetic character, the various lesions showing more or less tendency to group. In some of its forms it bears likeness to erythema multiforme and herpes iris, while in other cases it is allied to pemphigus. It varies greatly in the degree of development. The causes are varied, though in many cases they are neurotic in their nature; thus, the disease may follow shock to the nervous system. It is also met with accompanying the parturient state. In some cases it is septicæmic in origin. It is also at times due to irregular menstruation. As to sex, while more frequent in women, it is also encountered in men. In severe cases there is more or less constitutional disturbance, consisting of malaise, slight fever, and constipation, accompanying the onset of the disease or its relapses and exacerbations. Increased heat of skin, itching, and burning are also prominent symptoms at such periods.

The disease manifests itself in the erythematous, vesicular, bullous, pustular, and multiform varieties. The erythematous variety is characterized by patches or a diffuse efflorescence of an urticarial or erythema-multiforme-like nature, the similarity to the latter process being sometimes marked. The disease may remain in this form, or, as is usually the case, may pass into other varieties, especially the vesicular. This latter is the usual form of the disease. It is characterized by variously-sized, flat or raised, irregularly-shaped or stellate, glistening vesicles, as a rule without marked areolæ. They are usually firm and distended, are often difficult to detect, and have an herpetic look, being grouped into clusters of two, three, or more. Here and there they are aggregated into patches. When in close proximity they tend to coalesce, forming large irregularly-shaped, oblong, or lobulated vesicles, or even blebs. The eruption is usually profuse. The most striking symptom is the itching, which in most cases is severe or even intense. The vesicles make their appearance, as a rule, slowly, several days or a week being required for their complete development. This variety of dermatitis herpetiformis (formerly described with the name herpes gestationis) is liable to be confounded with vesicular eczema, but the irregularity in the size and shape of the vesicles; their angular or stellate outline, giving them a puckered look; their firm, tense walls, showing no disposition to spontaneous rupture,—will all serve in the diagnosis. In some cases the constitutional disturbance and the magnitude of the eruption, as regards profusion, distribution, and multiformity, will also be apparent.

In the bullous variety the lesions are more or less typical blebs, variable as to size and shape, seated upon a slightly inflamed or non-inflammatory base. They tend to group into small clusters, in which case the skin between them will be red, as occurs in herpes zoster. Together with the blebs, vesicles and small or even minute whitish pustules will usually be found, the combination of these varied lesions being sometimes remarkable. The blebs generally rupture or are broken by injury, and become the seat of yellowish or brownish crusts. This variety of the disease is liable to be confounded with pemphigus, but differs in its marked herpetic and more inflammatory aspect.

The pustular variety is generally less clearly defined than the vesicular, because the lesions are usually intermingled with vesicles, vesico-pustules, and blebs. The pustules are acuminate, rounded, or flat, are variable as to size, and are whitish or yellowish in color. The smallest are generally flat, sometimes being no larger than a pinpoint or pinhead, while those that attain the size of a pea are rounded or acuminate, and are surrounded with a marked red areola. The largest are flat, and incline to spread out and to run together, forming patches which later become covered with greenish crusts. Grouping occurs here as in the other varieties, and is sometimes peculiar in that a central pustule may be surrounded by a variable number of smaller pustules in a circinate form, as in herpes iris. This variety of the disease is the same condition described by Hebra with the title impetigo herpetiformis.

The papular manifestation is an ill-defined form of disease, consisting of small reddish, firm, more or less grouped papules, resembling in general appearance the papular lesions sometimes met with in abortive herpes zoster. They resemble at times also certain phases of relapsing chronic papular eczema. Owing to itching and scratching they are generally excoriated.

Finally, there remains to be described the multiform variety, which consists of several of the foregoing varieties occurring in combination, a phase of the disease which is not infrequent. It comprises erythematous, sometimes slightly raised, urticarial patches of variable size and shape, often marginate or confluent, and of a reddish, yellowish, or variegated color. In addition, there may be present more or less well-defined irregularly-shaped or rounded maculo-papules and flat patches of infiltration, papules, and papulo-vesicles in various stages of evolution. Vesicles, blebs, and pustules may also exist, together with pigmentation. Thus it will be noted there exists a mixture or combination of lesions, calling to mind the peculiarities of eczema, although the process is both more capricious and varied in its behavior.

It must also be stated that the disease may at any period change its type; thus the vesicular variety may exist for weeks or months, to be followed by a crop of blebs or of pustules. The mingling of several varieties at one or another period in the course of the affection is usually a marked feature. It is variable in its course, but is in most cases chronic, and not infrequently is of many years' duration. It inclines to persist and to show itself in distinct crops or attacks at irregular intervals, the patient in the mean time being comparatively free of eruption. Relapses are common. It is in most cases very rebellious to treatment. The prognosis should be guarded. The pustular and bullous varieties are the most grave, and at times may prove fatal, especially in connection with the parturient state.

Concerning the treatment, with the knowledge now at hand but little encouragement can be given. The general state of the patient should receive attention, and the cause inquired into and modified or remedied if possible. The therapeutics must be conducted on general principles. Arsenic and its preparations do not seem to be of value, at least in the cases that have fallen under our observation. Locally, the remedies most useful are those usually employed in chronic eczema and in pemphigus.