LUPUS OF THE VULVA.
It is characterized by the appearance of nodules which by confluence cause a diffuse swelling of the skin (lupus hypertrophicus). The site of inception of the disease becomes indurated and sinks between the larger or smaller nodules to form fissures. The more tender the underlying tissue, the greater the tendency of the tumors to grow down ward and to undermine the integument (lupus perforans). In other places, particularly on the mons veneris, the disease tends to spread widely. It usually then creeps along one side while on the other cicatri zation is beginning. (Lupus exedens or exulcerans.) Eventually the disease, here and there cicatrized, creeps over the entire vestibule. (Lupus serpiginosus.) The disease is rare. First described in 1849, by Guibourt and Huguier, as a chronic ulcerated disease of the skin, a few instances have been recorded by Martin, Lorent, Veit, Matthews Duncan, Martineau and Winckel. The disease occurs usually between the ages of twenty to thirty.
Bernutz and Guerin distinguish lupus into the three varieties which we have noted above: 1. Superficial inflammation, serpiginous in char acter, subdivided into the erythematous and the tubercular form, and including the exedens (exulcernns) form. 2. Lupus perforans. 3. Lupus hypertrophicus, with the subdivisions. the erythematous and the ulcerous. Martineau accepts for the vulva only the two forms or periods, the erythematous and the ulcerous.
On all portions of the genitals which have the structure of the external skin, there appears a dull red, livid blue discoloration, associated with marked infiltration and hyperplasia of the skin. There appear, in some instances, flattened light yellow nodules, the size of a pigeon's egg, the internal surfaces of which appear like superficial granulating tumors. The surface is smooth, tense and shiny. On the surface of reddish blue masses appear deep red points the size of a lentil. At the same time there are marked projections which Martineau claims are simply tubercles.
These changes are ordinarily painless, and therefore this period of the disease may not be seen by the physician, for aside from disfiguration of the genitals the disease thus far does not inconvenience the patient.
In more advanced stages the tubercles tend to soften at the centre, and when this is scratched or injured a little abscess forms. The second period is one of ulceration. The nodules coalesce, in the form of ir regular margined growths, the edges of which are everted towards the skin. These growths are generally superficial, and rarely extend into the subcutaneous cellular tissue. The superficies is reddish, uneven, and not inclined to bleed. The secretion is a clear serum in slight amount, so that there is no crust formation. The lupus tumor slowly extends forwards; more and more of the neighboring skin becomes infiltrated, and the destructive process proceeds, while the parts first affected may be in process of cicatrization.
When the entire external surface of the external genitals has been invaded the internal organs are in turn affected. This happens only slowly, after the lapse, indeed, of years. The disease of the external genitals results in cicatricial contractions of the external opening, the vagina and the rectum. If the process extends inwards it may affect the organs in the peritoneal cavity and lead to intestinal contraction.
The glands in the neighborhood are usually affected. In one of Winckel's cases they were not, and in the other they were for a long period. The state of the general health may fdr years remain good. Supervening pregnancy has only an unfavorable influence in those cases where the vagina and the vulva have become narrowed by the cicatricial process, and the child can only be delivered by rupture or tearing of the parts. In one of Bernutz's cases there followed peritonitis and death.
Lupus, or esthiomene of the vulva and vagina, may if not interfered with progress towards cure. Intercurrent attacks of erysipelas have a notable influence. Cure, even as in lupus of the face, may follow, although Martineau states that in the case seen by him the cure was only apparent, since after two months there was recurrence. Ordinarily, how ever, the disease ends in death through the development of phthisis, Bright's, intestinal obstruction, or by perforation into the bladder or the rectum.