Boeck's Sarcoid

BOECK'S SARCOID (S. Voezk), a dermatosis described in 1899 for the first time under the name of benign sarcoid, later as miliary lupoid. Boeck established the following three varieties. I. Micronodular diffuse: the development of the rash (1st stage) sometimes occurs quite quickly and begins with swelling and redness of the skin and itching; then over the course of several weeks, nodules form, numerous, elevated, dense, hemispherical, ranging in size from a pinhead to a small pea, originating from the cutis, sharply delimited. The 2nd stage is the stage of flowering, tumescence: the light red color of fresh nodules relatively quickly takes on a bluish (livid) or brownish tint. When pressed with glass (diaskopy), the nodules are less transparent than in lupus, and at the same time, millet-like spots of yellowish, yellowish-brown, or yellowish-gray color are often clearly defined— a very characteristic sign, apparently occurring only in lupus pernio. In the following, regressive stage (3rd stage—pigmentation), the nodules flatten; their coloration is intensified with brownish tones; a fine desquamation occurs. Then, on larger elements, numerous dilated blood vessels appear (see Telangiectasia). Finally, the nodules resolve, leaving either pigment spots that later disappear without a trace, or fine scars. II. Macronodular variety consists of single, clearly elevated tumors originating from the cutis, ranging in size from a hazelnut to a walnut and larger, round or oval in shape, of the same color and evolution as in the micronodular variety. The stage of pigmentation and telangiectasias is most characteristic: a sharp contrast is evident between the peripheral rim, elevated, slightly desquamating, colored in an intensely brownish-yellow, with numerous brown millet-like spots clearly protruding upon diaskopy, and the center, more or less sunken, bluish or pale, abundantly penetrated by dilated vessels. The outcome is a traceless disappearance or a superficial scar. Softening or ulceration does not occur. III. Diffuse-infiltrating form is manifested as indistinctly delimited, initially red, then bluish or brownish-red, wide plaques, up to the size of a palm, slightly elevated, dense. The phenomenon of diaskopy, evolution, and outcome are analogous to those in the previous forms. This form is more often encountered in combination with the macronodular or micronodular, or both simultaneously. The preferred localization is the face and the extensor surface of the upper extremities, but the rash may appear on other areas. The general course is prolonged, many months, even years; recurrences are possible after more or less prolonged complete remissions. The tempo of eruption in the macronodular and diffuse-infiltrating forms is very slow; in the micronodular form—sometimes quite rapid, with repeated flare-ups, but subsequently the disease takes a chronic character. The influence of the rash on the general condition is slight. Lymph gland involvement, salivary gland involvement, iridocyclitis, bone involvement, and splenomegaly occur. The disease primarily affects women aged 15 to 40 years. - The histology of miliary lupoid is most fully developed by Kyrle. Initially, ordinary inflammatory phenomena are observed around the dilated vessels of the papillae and deeper layers of the cutis in the form of a moderate perivascular infiltrate, mainly of lymphocytes and proliferating connective tissue cells; here and there—small groups of epithelioid cells. Acid-fast tubercle bacilli are scattered in large numbers among the infiltrate, sometimes isolated, sometimes in clumps. Approximately from the end of the 3rd week of the existence of the rash element, massive accumulations, mainly of epithelioid cells, in the form of oval or round nests or irregularly shaped cords, separated by layers of fibrous connective tissue, come to the fore. At the height of the development of the process, the monomorphism of the cellular infiltrate is striking: epithelioid cells predominate; a moderate number of giant cells; lymphocytes are either absent or, in small numbers, only at the periphery of the epithelioid nests. In immediate proximity to the latter, the fibers of connective tissue are slightly thickened, forming a kind of capsule. Collagen fibers of the connective tissue layers are either normal or swollen, becoming homogeneous, sometimes hyalinically degenerated. Elastic fibers are absent both in the foci of the infiltrate and in the capsules, or preserved as shreds (mainly in the connective tissue layers). The vessels are either unchanged or dilated; sometimes the endothelium swells and proliferates; vessels with thickened, infiltrated walls are occasionally encountered. Rods of tbc are rare and solitary here. This longest stage is followed by 1) degeneration of the epithelioid cells undergoing necrobiosis and disintegration and 2) simultaneous development at the periphery of dying epithelioid nests—productive-inflammatory processes in the form of capillary neoplasia and granulation tissue, which ultimately transforms into scar tissue (atrophic scar). In the final stage, tubercle bacilli are not found. The etiology is apparently tuberculous, which is indicated by the presence of tubercle bacilli in the affected skin (Boeck, Volk, Kyrle), successful inoculation of the guinea pig (Kyrle), and frequent positive focal reaction in the tuberculin test (Behring and others). Prevention, since the etiology is not precisely established, is absent. - Treatment. Good results are obtained from the long-term use of arsenic in large doses. A splendid effect was observed from Roentgen rays. The combined treatment of neosalvarsan and tuberculin is also recommended.

The article concludes by noting that the etiology is likely tuberculous, supported by the presence of tubercle bacilli in the skin, successful guinea pig inoculation, and positive tuberculin tests. It emphasizes that prevention is currently impossible due to the lack of a definitive etiology. Regarding treatment, it highlights the efficacy of long-term arsenic administration in high doses and the beneficial effects of Roentgen rays. Additionally, it suggests that a combined approach using neosalvarsan and tuberculin can be effective.