Description

This disease commences by the appearance of a soft tubercle, which slowly but gradually increases in size until it has attained the volume of a pea or small hazel-nut. By this time a crust will have appeared on its summit. On its removal an ulcer will be exposed. This gradually increases both as to area and depth, and after a time, five or ten years perhaps, the tubercle will have disappeared, and in its place we find a more or less extensive ulceration, characterized by irregular, overhanging margins of nearly normal or even less than normal consist with perhaps a softish tubercular development similar in character and appearance to the original tubercle. As the ulceration advances not only the skin but also the tissues beneath it are destroyed, leading to great disfigurement of the affected parts. During the progress of the affection additional and, not rarely, symmetrically developed tubercles may appear and slowly degenerate into ulcers. Before, however, these secondary developments have attained any great size the patient will probably have succumbed. In my own experience, phthisis pulmonalis has been the usual termination. The course of Lupus exedens is often exceedingly chronic, a case at present under observation having already lasted eighteen years. After a time, in some cases, the morbid action appears to change, and an epitheliomatous condition supervenes, evidenced by everted and hardened margins, and more or less pain. As a rule, Lupus exedens, pure and simple, is not specially painful. A portion only of the ulcer may become epithelionatous, the remainder preserving its primitive lupoid features.

Histology

In the cases which I have personally examined, the characteristic microscopic feature has been the occurrence of sharply defined aggregations of small non-stratified cells, without much or any diffuse cellular infiltration.*

* Except by the aid of the microscope.

Diagnosis

There are but three of the commoner affections with which Lupus ezedens could, by any chance, be confounded. These are, Syphilis, Lupus vulgaris, and Epithehoma. In the first instance the duration of the lesion is sufficiently characteristic. In an early stage of Lupus exedens I must confess I do not know of any pathognomonic sign by which it may be distinguished from an early condition of Lupus vulgaris. In a late stage the depth of the ulceration is sufficient. From Epithelioma it is to be dis-tinguished by the character of the primitive nodule, of the margin, the course and the frequently multiple lesion, the pain, finally by the micro-scropical appearances. In Lupus the nodule is softer than the surrounding normal tissue, in Epithelioma it is harder; in lupus the margin is ir-regular and often undermined and not hardened, in epithelioma, it is thickened, looking as if everted and hard. In lupus the progress is very slow, in epithelioma more rapid; in lupus there may be several lesions in different stages of development, in epithelioma there is rarely more than a single lesion; lupus is comparatively painless, epithelioma is frequently painful. In lupus exedens we have the microscopical features above spoken of, and without any proliferation of the stratum Malpighii; in epithelioma we find, in the early stages, prolongations downward of the stratum Malpighii, and later stratified cell-nests.

Prognosis

In the early stages, that is, before the lesion has attained a diameter of more than one or two centimetres, the prognosis is good, provided the lesion be so situated that it can be thoroughly removed. The prognosis, however, becomes less favorable as the lesion increases in size, owing to the difficulty of removing the whole of it, until, finally, a condition may be reached, in which operative interference would not be justifiable, not alone in consequence of the extent of the lesion, but also the presence of a cachectic condition that in many cases would by this time have developed.

Etiology Of Lupus

There is little question in the author's mind that Lupus may very properly be considered as one of the expressions of the Scrofulous Diathesis. This is the view almost universally held in France and England and Italy, and only disputed, I believe, by the Vienna school, and a few of its adherents in this country.

Thompson (83) considers that Lupus is "but the local manifestation of a general disease," a view that is probably correct; and later English dermatologists, as Wilson, Tilbury Fox, Anderson, Hutchinson, and others believe that this general disease is the scrofulous diathesis.