Pemphigus Vulgaris

This form of pemphigus vere accounts for approximately 75 per cent of the total number of all forms of pemphigus cases. The dermatosis begins with affection of the oral and throat mucosa, as a rule, after which the skin of the trunk, limbs, inguinal region, axillae, face and external genitals is involved in the process. The course is characteristically severe and chronic. Patients died within a few months or one and a half to two years in the precorticosteroid era. The disease sets in suddenly with the abrupt appearance of bullae on an apparently healthy skin. The bullae are at first tense and then rapidly become flaccid. They range in size from a pea to a bean but may be larger, and are filled with clear, and later with cloudy serous fluid.

Weeping, eroded surfaces with polycyclic contours form rapidly in the process of evolution. In regeneration of the epidermis, stratified brownish crusts form after which a brown pigmentation remains on the areas of the foci. In other cases (when the process tends to spontaneous remission or under the effect of steroid therapy) the tops of the bullae remain intact, because the exudate resolves, and they gradually collapse and turn into a thin crust, which then drops off. Spontaneous remissions alternate with recurrences, as a rule. In a benign course of the process the patient's general condition hardly changes. In a malignant course, the body becomes exhausted, septic fever sets in and is particularly severe if secondary infection develops; there are also eosinophilia, retention of sodium and chlorides in the tissues and a diminution in the protein content. Changes may be found in the blood protein fractions and in the content of immunoglobulins A, G and M. Intercurrent diseases of the liver, kidneys, lungs and heart may develop.

Patients suffering from pemphigus experience no itching as a rule.

Sometimes the numerous erosions cause severe pain which intensifies when the dressing is changed and when the patient alters position. The lesions are particularly painful when located in the mouth, on the lips, and the genitals.

The degenerative changes in the Malpighian layer, which are embraced by the term 'acantholysis' are the morphological base of a significant clinical and diagnostic sign known as Nikolsky's sign (phenomenon). It consists in the following. If one pulls the ruptured top of the bulla, the epidermis on the apparently healthy skin is separated; when the apparently healthy skin area between the bullae or erosions is rubbed, the upper layers of the epidermis are also separated, and finally, when apparently healthy skin areas remote from the foci of affection are rubbed, the upper layers of the epidermis are easily injured. Besides, when an intact bulla is pressed with a finger one can see the fluid separating the adjacent epidermal areas and the bulla enlarging on the periphery. This last diagnostic test in pemphigus is called the Asboe-Hansen's sign. Despite the high diagnostic value of Nikolsky's sign, it cannot be regarded strictly pathognomonic for true pemphigus, because it is also encountered in other dermatoses (particularly in congenital epidermolysis bullosa, Ritter's disease, Lyell's syndrome). It is positive in almost all pemphigus patients in the phase of exacerbation, but may be negative in other periods of the disease.

Modern methods of treatment make it possible to considerably delay the onset of recurrences in many such patients, whereas the use of maintenance steroid therapy prolongs the patients' life for many years after the disease.