Lupus Erythematosus

LUPUS ERYTHEMATOSUS (Lupus erythematosus, seborrhoea congestiva (Hebra), erythema centrifugum (Biette), herpes cretace (Devergie), scrofulide erythemateuse, maladie de Cazenave; erythema atrophicans; ulerythema centrifugum (Unna); atrophodermatitis centrifuga (Sommasoli); lupus superficialis, lupus sebaceus, lupus seborrheicus (Volkmann)), a dermatosis first described in 1827 by Ray as a disease of the sebaceous glands. Cazenave (1851) was the first to propose considering this dermatosis as a variety of ordinary lupus and described it under the name lupus erythematosus. L. e. is not very rare. In England, among 20,000 skin patients, L. e. is observed in 1.5% (MacLeod). In America, according to data from the American Dermatological Association, L. e. constitutes 0.32% of all skin diseases; in Germany - 0.11% (in Hamburg) - 0.12% (in Berlin) of all dermatoses (Jadassohn). In the USSR, L. e. also occurs not infrequently. Through the polyclinic of the State Venereal Institute over 6 years (1922-27), out of a total of 4,765 skin diseases, L. e. was noted in 140 patients, i.e., it occurs more frequently than lichen planus, contagious molluscum, multiform erythema, and almost reaches the number of common dermatoses, such as Gibert's roseola (Agronik).-Characteristic signs of the disease: erythema with various degrees of persistent vasodilation and plastic infiltrate, hyperkeratosis and atrophy. The clinical picture of L. e. shows great polymorphism and depends on the predominant development of one or another of the indicated signs. Usually the disease begins with the appearance of a limited red spot, slightly elevated, which gradually expands, becoming covered in scales in the center. The scales are difficult to remove: they bear on their lower surface spines, which are firmly fused with the underlying tissue. After removing the scales, the dilated follicular openings are revealed. The process has a tendency to spontaneous healing, forming a smooth, shiny area of scar-modified skin.-Chronic and acute forms of L. e. are distinguished. Acute forms occur significantly less frequently. Chronic forms are divided into 2 clinical types. 1. L. e. discoides (fixus chronicus). It is characterized by the localization of one or more disks on some area of the face, mainly on the cheeks, auricles and hairy part of the head. Most often the nose is affected (87.2%), cheeks (69.8%), hairy part of the head (27.2%); lesions are significantly less frequently found on the fingers of the hands, chin (Olesov). Mucous membranes [see separate table (pp. 487-488), fig. 3] (according to Sequeira) are affected in 28%. The lesion is always deep, leaving deep scar changes upon regression. A characteristic feature is the persistence of lesions and chronic course. In 18% (Olesov), the disks merge, involving the bridge of the nose and cheeks [see separate table (pp. 487-488), fig. 2], taking the shape of a butterfly or bat (vespertilio). 2. L. e. migrans (syn.: L. e. disseminatus, aggregates, L. e. symmetricus or erythema centrifugum-Biette); characterized by symmetrical localization, superficial infiltrate, bright inflammatory coloration and mobility. Primary lesions can increase in size very quickly, merge and form continuous, extensive areas of involvement. In this form of the disease, rapidly alternating periods of worsening and improvement are observed. After recovery, superficial scar-like changes remain, sometimes barely noticeable. Transitions from the discoid form to the migratory form and back are observed. L. e. acutus (syn.: L. e. exanthematicus or L. e. disseminatus acutus) is rare. Acute and subacute forms are distinguished. The acute form was first described by Kaposi (1872). It develops either as a primary disease - L. e. acutus d'emblée (Pernet) (lup. erythem. aigu d'emblée) or, more often, as a transition from the chronic form. It is characterized by rapid course high temperature (often above 40°), often joint pains, protein in the urine, and general severe septic phenomena. Usually after 2-8 months, the disease ends in death. The subacute form proceeds more slowly and may end with a transition to the chronic form with subsequent recovery and vice versa - the chronic form can transition to subacute and acute forms. Clinically, L. e. acutus begins with the appearance of erythematous disks on the face, which soon merge, giving diffuse involvement. Due to edema, erythema and febrile condition, the lesion resembles erysipelas in appearance, which gave Kaposi reason to call it 'erysipelas perstans faciei,' and Kreibich - 'erythema perstans'. Against the background of the mentioned redness, weeping and crusts may appear; then a certain similarity with acute eczema is obtained. Often on the back of the hands, especially on the fingers, reddish-blue spots appear, on which vesicles form, sometimes with hemorrhagic content, which gives great similarity with multiform exudative erythema or erythema from chilblains. Usually after the involvement of the face and hands, erythematous disks appear on the trunk and extremities; sometimes they have a follicular character. Upon regression of these elements, slight scar-like atrophy may form, and then the lesion takes on the appearance characteristic of L. e. chronicus. The lesion often involves the hairy part of the head, where sharply defined erythematous disks appear, covered with scales and giving scar-like atrophy upon regression. L. e. usually begins between the ages of 20 and 40, rarely in childhood: 2 years (Rona), 3 years (Kaposi and Ukhin), 5 years (Boeek), even more rarely - later: 56 years (Olesov), 66 years (MacLeod), 72 years (Ukhin). Women suffer more often than men: 3.1 cases of L. e. in women per 1 case in men (Olesov). Approximately the same ratios are given by other authors (Ukhin, MacLeod, Jadassohn). The acute form is especially often observed in women: out of 10 cases - 9 women and 1 man (Pernet). Certain professions predispose to the disease. Statistics show that housewives, blacksmiths, foundry workers and other people who have to work in conditions of frequent temperature changes from heat to cold and vice versa suffer more often than people of other professions (MacLeod, Sladkovich). Trauma and chemical irritations can also play a provoking role (Thibierge, Olesov). Pathological anatomy. In chronic cases of L. e., hyperkeratosis is noted, with the epidermis thickened unevenly, resembling folds, edema of the papillary layer, abundant infiltrate in the papillary and subpapillary layers around dilated vessels, sebaceous and sweat glands. The infiltrate consists mainly of lymphoid cells. A pronounced tendency is noted to replace the infiltrate with fibroblasts, and then with scar tissue. The difference in histological pictures between acute and chronic L. e. lies in the fact that in the acute form of L. e., the signs of acute inflammatory process are more pronounced: exudation is more pronounced (up to blister formation), vessels are significantly dilated, sometimes - hemorrhages. In dermatology, no sharp distinction is made between the acute and chronic forms. The basis for this is the often observed development of the acute form from the chronic form or vice versa and the great similarity of the histological pictures of the lesion. Regarding the etiology of L. e., there is no unified opinion. At present, the following theories exist: tuberculous, external irritations, syphilitic, of various combined origin and septic.- Tuberculous theory. Cazenave, then Besnier were the first to express and then defend this theory. A review of the literature on this question was made by Civatte (1926). The main points confirming this theory: 1) Frequent coincidence of tuberculous lesions of the lungs and other organs with L. e. (Ehrmann, F. Falkenstein). 2) Frequent coincidence of L. e. with tuberculous adenitis. 3) Koch's bacillus in the lesions of L. e. 4) Positive result from inoculation of lesions into pigs. 5) In the case of Bloch and Ramel, the blood of a patient with L. e. acutus caused tuberculosis in pigs. 6) Fresh lesions of L. e. react sharply to the injection of tuberculin and methyl antigen. 7) Combination in the same patient of L. e. and L. vulgaris; L. e. on scars after tuberculous lesions; tubercles of L. vulgaris on disks of L. e., treated by scarification (quoted Olesov). 8) The existence of a special form L. vulgaris erythematoides (syn.: L. erythematoides Leloir's, L. erythematoides nodularis R. Crocker's), which is transitional between L. e. and L. vulgaris. Ehrmann and Falkenstein consider that L. e. results from the entry into the skin through the blood of weakened tuberculous bacilli 'probably' from the glands; they consider the acute form of L. e. as tuberculous sepsis. French dermatologists (Besnier and others) classify L. e. in the group of tuberculids.- Schaumann distinguishes L. e. into a special group, parallel but not identical to tuberculids. According to him, the pathogenesis of the disease lies in a special granulomatous disease of the glands, 'metascrofulous' lymphogranulomatosis. L. e. is a reflection on the skin of the process that occurs in the glands.

The theory of external irritation is based on certain known cases in which it was more or less definitely established that external factors (light, chilling, shrapnel injury, etc.) served as a trigger for the development of L. e. The theory of syphilitic origin is mainly supported by some French authors (Ravaut, and others, and Kuznetsov). These authors base their conclusions on the coincidence of positive immunobiological reactions with the simultaneous existence of L. e. and on favorable results of antisyphilitic treatment. Supporters of the theory of various combined origins of L. e. believe that L. e. is a skin symptom that can be caused by tuberculosis, syphilis, diseases of the endocrine glands, staphylococcal or streptococcal infection (Levandovsky), and uric acid diathesis (Kolyada). Davydovsky and Vail believe that for the development of L. e., not so much a specific pathogen is important as the mechanism by which the disease-causing agent affects the body, to which they attach particular importance to the increased sensitivity of the nervous apparatus. Supporters of focal sepsis (Mac Leod and others) believe that L. e. is most often caused by streptococcus, which comes from a local focus located in the crypts of the tonsils, in carious teeth, in the uterus, etc. These authors consider that L. e. is a variety of erythema multiforme. There is an opinion that L. e. is associated with a disease of the sebaceous glands (seborrhoea congestiva Hebra) (Sladkovich), but this theory has very few supporters. Recognition of L. e. is sometimes difficult. In the initial stages before the development of atrophy, L. e. can simulate exudative erythema, psoriasis, and especially seborrheic eczema. Exudative erythema differs in its acute course, the elements do not have peripheral growth, do not peel, and do not cause atrophy. L. e. differs from psoriasis in that psoriatic scales on the lower surface do not have spines, there is no atrophy in psoriasis; in seborrheic eczema, the scales are loose, fatty, yellowish, the infiltrate is scanty, and there is no atrophy. Chronic forms of L. e. must be differentiated from lupus vulgaris erythematoides; in difficult cases, the question is resolved by biopsy. The tuberculous syphilid is generally not difficult to distinguish from L. e., but there are cases when the diagnosis can only be made by trial antisyphilitic treatment. When the mucous membranes of the cheeks, lips, etc. are affected, the diagnosis is facilitated by simultaneous skin lesions; in cases where only the mucous membrane is affected, the diagnosis is made on the basis of biopsy data. A severe and rare complication of long-existing and often scarred L. e. is epithelioma. The appearance of cancer is recognized by the change in the external appearance of the lesion. To confirm the diagnosis, a biopsy is performed (Bogrov). In the treatment of each individual case of L. e., it is necessary to adapt. In cases of severe inflammatory reaction, anti-inflammatory treatment is applied: compresses, ointments, and pastes. Superficial cases are treated with iontophoresis, d'Arsonval currents; according to Hollander, by smearing the foci of T-ra Jodi, internally - quinine (0.2-0.5), 8-10 minutes before smearing. More persistent cases are treated with freezing CO₂ (carbon snow), smearing with T-ra Jodi followed by the application of a heating compress from Ung. Hydr. pp. albi (1-2%); in other cases, plasters are applied: soap-salicylic, mercury. In recent times, treatment of L. e. with injections of tuberculin, methyl antigen of Negre and Boquet has become known; especially widely, treatment with gold preparations (crisalgan, trifal, aurifos, crisalbin) has been applied. Aurotherapy gives good results, but this method is not yet sufficiently studied. In all cases of L. e., in addition to external and internal treatment, it is necessary to recommend adherence to the regimen prescribed for tuberculous patients.