Pemphigus
What do you understand by pemphigus?
Pemphigus is an acute or chronic disease characterized by the successive formation of irregularly-scattered, variously-sized blebs.
Name the varieties met with.
Two varieties are usually described—pemphigus vulgaris and pemphigus foliaceus.
Describe the symptoms and course of pemphigus vulgaris.
With or without precursory symptoms of systemic disturbance, irregularly scattered blebs, few or in numbers, make their appearance, arising from erythematous spots or from apparently normal skin. They vary in size from a pea to a large egg, are rounded or ovalish, usually distended, and contain a yellowish fluid which, later, becomes cloudy or puriform. If ruptured, the rete is exposed, but the skin soon regains its normal condition; if undisturbed, the fluid usually disappears by absorption. Each lesion runs its course in several days or a week.
A grave type of pemphigus is exceptionally observed in the newborn—pemphigus neonatorum.
What course does pemphigus vulgaris pursue?
Usually chronic. The disease may subside in several months and the process come to an end, constituting the acute type. As a rule, however, the disease is chronic, new blebs continuing to appear from time to time for an indefinite period.
In what respects does the severe form of pemphigus vulgaris differ from the ordinary type?
In the severe or malignant type the eruption is more profuse; there is marked, and often grave, systemic depression, and the lesions are attended with ulcerative action.
Describe the symptoms and course of pemphigus foliaceus.
In this, the grave type of the disease, the blebs are loose and flaccid, with milky or puriform contents, rupturing and drying to crusts, which are cast off, disclosing the reddened corium. New blebs appear on the sites of disappearing or half-ruptured lesions, and the whole surface may be thus involved and the disease continue for years, compromising the general health and eventually ending fatally.
In some cases of pemphigus (pemphigus vegetans) a vegetating or papillomatous condition develops from the base of the lesion, with an offensive discharge; it is usually a grave type of the malady.
Exceptionally cases (dermatitis vegetans) are met with which have a close similarity in their symptoms to pemphigus vegetans, but in which the eruption is more or less limited to the genitocrural region. The disorder is not malignant and usually yields to cleanliness and antiseptics.
What is the character of the subjective symptoms in pemphigus?
The subjective symptoms consist variously of heat, tenderness, pain, burning and itching, and may be slight or troublesome.
What is known in regard to the etiology of pemphigus?
The causes are obscure; general debility, overwork, shock, nervous exhaustion, and septic conditions (microörganisms) are thought to be of influence. There seems no doubt that those who have to do with cattle products, especially butchers, are subjects of acute and usually grave pemphigus. Vaccination has exceptionally been responsible for the disease, probably through some coincidental infection. The disease is not contagious, nor is it due to syphilis. It may occur at any age.
It is a rare disease, especially in this country. Most of the cases diagnosed as pemphigus by the inexperienced are examples of bullous urticaria, bullous erythema multiforme, and impetigo contagiosa.
What is the pathology?
The lesions are superficially seated, usually between the horny layer and upper part of the rete. Round-cell infiltration and dilated blood vessels are found about the papillæ and in the subcutaneous tissue. The contents of the blebs, always of alkaline reaction, are at first serous, later containing blood corpuscles, pus, fatty-acid crystals, epithelial cells, and occasionally uric acid crystals and free ammonia.
From what diseases is pemphigus to be differentiated?
From herpes iris, the bullous syphiloderm, impetigo contagiosa and dermatitis herpetiformis.
How do these several diseases differ from pemphigus?
The acute course, small lesions, concentric arrangement, variegated colors, and distribution, in herpes iris; the thick, bulky, greenish crusts, the underlying ulceration, the course, history, and the presence of concomitant symptoms of syphilis, in the bullous syphiloderm; the history, course, distribution, the character of the crusting, and the contagious and auto-inoculable properties of the contents of the lesions, in impetigo contagiosa; the tendency to appear in groups, the smaller lesions, the intense itchiness, course, multiform characters of the eruption and the disposition to change of type in dermatitis herpetiformis,—will serve as differential points.
State the prognosis of pemphigus.
Its duration is uncertain, and the issue may in severe cases be fatal. In the milder types, after months or several years, recovery may take place.
The extent and severity of the disease and the general condition of the patient are always to be considered before an opinion is expressed.
Pemphigus neonatorum usually ends fatally.
Give the treatment of pemphigus.
Both constitutional and local measures are demanded. Good nutritious food and hygienic regulations are essential. Arsenic and quinia are the most valuable remedies. The former, in occasional instances, seems to have a specific influence, and should always be tried, beginning with small doses and increasing gradually to the point of tolerance and continued for several weeks or longer. The remedy should not be set aside as long as there are signs of improvement, unless the supervention of stomachic, intestinal or other disturbance demand its discontinuance. Other tonics, such as iron, strychnia and cod-liver oil, are also at times of service. The blebs should be opened and the parts anointed or covered with a mild ointment. In more general cases bran, starch and gelatin baths, and in severe cases the continuous bath, if practicable, are to be used.