Definition. An acute or chronic inflammatory disease of the skin, characterized by the appearance of successive crops of variously sized blebs seriously affecting the general health, and pursuing an indefinite course, often with fatal termination.
Symptoms. Pemphigus is a rare affection as a distinct disease. It is best considered in two general types, pemphigus vulgaris and pemphigus foliaceus, with subordinate varieties known as pemphigus vegetans, pemphigus acutus, and pemphigus neonatorum.
Pemphigus vulgaris (pemphigus chronicus) is the commonest form of the disease, and is characterized by the appearance of primary bullae in crops recurring from time to time for an indefinite period. The eruption may appear on almost any part of the body, but is more abundant usually upon the extremities. At first bilateral, it often may become symmetrical, although irregularity in distribution is as common as arrangement in groups. The round or oval blebs, filled with a translucent fluid, vary in size from a pea to a hen;s egg, or even larger. They rise abruptly from the skin, and although they may have a reddened base, rarely present an areola. Each attack is associated with some systemic disturbance, such as chills, fever, vomiting, malaise, etc. the lesions may vary from one or two to a hundred or more, but each individual group pursues a fairly uniform course. Whether they rupture or gradually dry up, they form, with the roof wall, brownish crusts which in few days fall off and leave beneath a new epidermis of a reddish or purplish color. Brownish pigmentation may persist for a few weeks. The local sensations may be slight, or itching. burning, pain or tension may be belt, but accidental or excoriation will increase these subjective sensation. Extreme variations from the ordinary type of chronic pemphigus may be distinct, but are usually rare. From the mildest from they may involve the mucous membranes of the mouth, conjunctiva, etc., or they may become infiltrated with active ulceration of the superficial layers of the skin.
One form is characterized by the development of peculiar vegetations (pemphigus vegetans-so named by Neumann). About seventy of these cases have been recorded as occurring in America, and the general symptoms of fever, languor and malaise, followed in nearly every case by a sore throat or mouth and pain on eating and swallowing, preceded the cutaneous lesions. The mucous membrane of the affected areas will show poorly developed blebs, which become denuded, excoriated and exquisitely tender. After a variable interval of a few days or weeks, ordinary pemphigoid lesions develop on the hands, feet, groins, axillae, and other parts of the surface, but little tendency is shown toward universal involvement. Here its resemblance to the common form of pemphigus ceases. Instead of drying up, the lesions become excoriated, ulcerate and extend by concentric peripheral growth. the original lesions often crust over, while new blebs form about them, especially in the genito-crural or axillary regions, or about the mouth or nose, where fungoid, papillary, or wart-like growths develop, accompanied by a sticky, offensive secretion. Some patches may heal, but new groups tend to cause a greater surface involvement which, together with the painful state of the mouth and throat, interfere with nutrition, exhaust the strength, and death occurs within a few weeks or months.
Pemphigus acutus is an acute form which runs a rapid, febrile course, terminating with death or recovery within from two to six weeks. When this variety occurs in infancy (pemphigus neonatorum) it usually attacks infants exposed to unsanitary surroundings, and such outbreaks in hospitals and other institutions appear to be contagious or epidemic in nature. There is usually antecedent fever, which remits with the first crop of bullae. Other crops follow in rapid succession without any special predilection. It is questionable if acute pemphigus is rightly considered as a variety of pemphigus because many authorities have asserted its infective origin from definite poisons. It is perfectly possible for it to appear in apparently healthy and well-nourished children, but it is comparatively rare among adults. Among the latter, a number of malignant and fatal cases have been reported in butchers and others working among animals, who have suffered from some trivial wound. Hence again, it would seem that as regards these cases, a direct parasitic causal factor might be responsible.
Pemphigus foliaceus is so distinct that it might almost be looked upon as a separate disease die it not, in some instances, follow common pemphigus. the lesions consist of flaccid blebs usually developing from apparently normal skin. They may contain so little fluid as to be only slightly raised above the surrounding skin, or as an effect of gravitation, these may settle to one side, appearing like a partly filled blister. The contents soon become turbid, then purulent, and often sanious. Individual blebs will rapidly rupture, and the wall acquires a feeble adhesion to the center of its base, while the periphery separates, curls up, and presents a resemblance to dead leaves. Hence, the adjective foliaceus applied to it by Casenave. The denuded skin left exposed after the rupture does not dry up, but exudes continually, reforming thin crusts. Even if new epidermis does form, it is soon rubbed off or separated by renewed exudation or bullae. While at first the disease may only involve a small area, it gradually and symmetrically spreads until, in the course of months or years, every part of the surface is affected, rarely excepting the palms and soles. But even these parts may become thickened, dried and cracked, so that the disease practically may be said to be universal in distribution. The odor which pervades the room of such a patient is pronounced and sickening. Burning and smarting to a marked degree, and itching to a variable degree, as well as affected, in which case the blebs soon macerate, and appear as grayish, membraneous patches. This diphtheritic covering is easily shed, leaving a raw, reddish-brown surface and according to its location, mastication, deglutition, respiration or vocalization may be seriously impaired. If the conjunctiva is affected, vision may be lost from atrophy, and there may be adhesion of membrane to the eyeball. The nails become thin, curved, furrowed, and are sometimes thrown off. The hair gets brittle and falls out in large spots, while furuncles, abscesses and fissures may add to the patient’s suffering. The bodily temperature may remain comparatively normal, but in advanced cases there is a low type of fever, probably due to secondary infection, which, together with insomnia and gastrointestinal disorders, resulting in extreme emaciation, render the patient very vulnerable to any intercurrent disease. Remissions of this form of pemphigus lead one to false hopes of recovery.
Etiology and Pathology. The causes of many acute cases can, no doubt, be traced to microorganisms, although the action of toxins developed from various sources appear to explain best the symptoms of the disease. In many cases, there are marked changes in the central and peripheral nervous system. Pernet, Demme and Whiphouse all refer to the presence of a diplococcus in the bleb contents, but whether the bacteria are primary or secondary is a question to be settled. Predisposing factors, such as organic and functional disorders of the nervous system, or a lowered state of the general health, are probably necessary. It can be said positively that little is known concerning the etiology of the majority of cases.
The bullae are usually superficially situated between the rete and the corneous layer, or on the upper part of the former. There is great dilatation of blood and lymph vessels, together with cellular infiltration of their walls, edema of the papillary layer, and changes in the elastic tissue fibres. There is also a high-grade hemic eosinophilia, as is found in dermatitis herpetiformis.
Diagnosis. Pemphigus is to be distinguished from other affections in which bullous lesions develops, but usually the comparison of the diagnostic features of any form of pemphigus will leave little doubt as to the diagnosis. The blebs of dermatitis herpetiformis are associated with other lesions, papules, vesicles, pustules, or erythema. They pursue a variable course, accompanied by marked itching or burning. Syphilitic bullae are rare, except in early infancy; then they show marked preference for the palms and soles. The bullae occurring in leprosy would be associated with anesthesia or other typical symptoms of that disease. The blebs of varicella are easily recognized by their consecutive occurrence and acute course. Impetigo contagiosa will present pustular lesions, is contagious and auto-infectious but easily cured. The bullous lesions of erythema multiforme are associated with other inflammatory lesions, and occur chiefly on the dorsal surfaces of the hands and feet. While the wheals of urticaria may become blebs their mode of evolution, ephemeral course, with burning, stinging or itching sensation, should be diagnostic. The crusts or scales of generalized psoriasis, dermatitis exfoliativa, lichen ruber, or eczema rubrum might possibly be confounded with advanced pemphigus foliaceus, but the history of the primary lesions, their evolution, especially the lack of flaccid bullae, should serve to differentiate them from pemphigus. some drug eruptions include bullous manifestations, but this fact can be gathered by inquiry as to the medicines used within the few preceding weeks, and such a conditions is naturally self-limited. The surrounding or circumstances attending artificially produced lesions (dermatitis factitia), such as might occur among malingerers, insane or mentally defective people, usually explain these conditions.
American homeopath, he directed several hospitals in New York.
Professor of dermatology.
Served as Lieut. Colonel during the 1st World War.
See his book online: American homeopathy in the world war