Subacute Lupus Erythematosus

Subacute lupus erythematosus may develop from the chronic discoid form or may occur spontaneously (d'emblee). The clinical picture is much less pronounced than that in the acute form of the disease. In some cases, the process takes the course of persistent erythema and resembles erysipelas of the face (erysipelas perstans faciei). A demarcated area of oedema, hyperemia, and scaling forms on the skin of the face in such cases. Restricted erythematous patches may occur on the skin of the limbs, chest, and scalp. The disease develops slowly and for a lengthy period of time is manifested only by erythematous-papular lesions and the asthenovegetative syndrome, after which chronic hepatitis, pleurisy, and other symptoms of systemic affection of the body appear, though they are less pronounced than in the acute form.

Histopathology. Vacuolar degeneration of the stratum basale and oedema of the dermis are demonstrated more cleary than in chronic discoid erythematosus. Follicular hyperkeratosis and the inflammatory infiltration are less apparent.

The diagnosis is based on the signs of cicatrizing erythematosis (erythema, follicular hyperkeratosis, cicatricial atrophy), changes in the blood (leucopenia, lymphopenia, a shift to the left in the differential count, anemia, thrombocytopenia, increased gamma globulin content, a high ESR), demonstration of LE cells in the patients's blood, and detection of antinuclear and anti-antinuclear antibodies in blood of some patients by fluorescent microscopy.

Treatment is prescribed in accordance with the clinical form of the disease. Chronic forms of cicatrizing erythematosus are treated effectively with antimalarial agents: chingaminum syn. Resoquine (chloroquine diphosphate), Plaquenil (hydroxychloroquine), 0.25 g taken twice a day in cycles of five to ten days with intervals of two to five days between them.

Corticosteroids have a favourable effect in systemic lupus erythematosus: prednisolone or methylprednisolone (daily dose of up to 50-80 mg), triamcinolone (daily dose 30-40 mg), dexamethasone (daily dose 5-10 mg). Treatment is begun with the maximum doses mentioned above and then the daily dose is gradually reduced to the minimum maintenance dose. Combination of steroids and antimalarial agents is very effective.

A beneficial therapeutic effect is caused by vitamins, especially those of the B complex, possessing a photodesensitizing effect. Intramuscular injections of vitamin B₆ or vitamin B₁₂ (300 µg injected at an interval of two to three days) (treatment consists of 15 injections) are recommended. Oral vitamin therapy includes nicotinic acid (0.05 g three times a day), folic acid (0.01-0.02 g three times a day), pantothenic acid (0.05-0.1 g three times a day), riboflavin (0.005 g three times a day), aevit (one capsule two or three times a day), and ascorutin (one tablet three times a day). Vitamins intensify the therapeutic activity of corticosteroids and antimalarial drugs and lessen their toxicity.

Immunosuppressants or cytostatics (cyclophosphan) are prescribed in severe cases in a complex with corticosteroids and vitamins.

External treatment is accomplished with steroid creams (Celestoderm, Flucinar, Ftorocort, Sinaphlan, Elocom, Diprosalic) and injection of hydrocortisone emulsion, a 5 per cent chloroquine diphosphate or delagil (chloroquine) solution around the foci. Cryotherapy may be prescribed in localized discoid cicatrizing erythematosus.

As a preventive measure, the skin (on the face in particular) should be protected from insolation and heat rays. Creams which cause the same protective effect and pastes containing salol and triethanolamine salt of para-aminobenzoic acid in 5-10 per cent concentration are recommended for the same purpose. Broad-spectrum sunscreens and sun-protective measures, including lifestyle changes and clothing, are perhaps the most important initial measures.

Prognosis. The diversity of the prognosis in patients with lupus erythematosus should be emphasized. Chronic discoid cicatrizing erythematosus, for instance, is benign and very rarely transforms into the acute form. The systemic form of the disease, which occurs in exacerbation of chronic discoid or, more frequently, disseminated lupus erythematosus, follows a more favourable course than that taken by the spontaneously occurring (d'emblee) form. The last form is marked by a torpid course and resistance to treatment, and the prognosis is therefore grave.

SCLERODERMA (SCLERODERMIA)

At the peak of its development, scleroderma is characterized by focal or diffuse marked induration (hardening of the tissue as the result of affection of the connective-tissue fibres. The processes of sclerosis may be localized in any organ but are most marked in the skin and subcutaneous tissue and are of a long duration. Circumscribed or localized (morphea guttata or linearis), diffuse (generalized, or systemic), and mixed forms of scleroderma are distinguished. The question concerning the identity of the circumscribed and diffuse forms (as the identity of chronic discoid and systemic lupus erythematosus) cannot be considered solved. Some scientists believe them to be a manifestation of a single pathological process, others distinguish them strictly and claim that they differ in pathogenesis and in the characteristic clinical picture.

Three stages are distinguished in the development of circumscribed scleroderma: oedema, hardening (sclerosis), and atrophy. The stage of oedema lasts a few weeks and rapidly changes to the stage of hardening. In this stage the skin is smooth, glossy, stretched and has a pasty consistency; its colour may vary from that of normal skin to bright-red, sometimes with a bluish tinge. The short-lived first stage is often overlooked. The stage of hardening is characterized by a hard skin which is cold to the touch and cannot be displaced from the underlying tissues and which it is impossible or hardly possible to grasp in a fold. A light-blue ring around the foci of affection is a characteristic sign in this stage (ring of peripheral growth). The foci themselves have a wax-yellow or dirty-grey colour. In the stage of atrophy, the skin becomes thin, white and resembles parchment. The subcutaneous fat and muscles may also atrophy. In such cases the skin lies directly on the bones.

The etiology and pathogenesis of scleroderma have still not been disclosed to the end.

The pathological process develops mostly in the vascular wall and the structural elements of the connective tissue. The accumulation in the body of some biologically active substances, e.g. serotonin, facilitates vascular spasm. Impaired interrelationship of hyaluronic acid and hyaluronidase also plays a very important role. Under the effect of diminished hyaluronidase activity, accumulation of mucopolysaccharides, and fibrinoid degeneration of the connective-tissue elements, oedematous-indurative changes occur first which are later followed by intensified synthesis of collagen. According to many authors, scleroderma may develop as a peculiar allergic reaction to the penetration of heterogenous proteins or the formation of aggressive auto-antibodies. This opinion is based on the fact that scleroderma occurs after vaccination, transfusion of blood, and the administration of foreign serum for therapeutic purposes.

In scleroderma, as in other ‘collagenoses’, not only the collagenous, elastic, and reticular fibres but the interstitial adhesive substance and cells, including the terminal branchings of the blood vessels and the nerve endings, suffer. The titres of antibodies to the body's own tissues grow in the patient's serum, dysproteinemia develops (mostly hypergammaglobulinemia). All this makes diseases of the connective tissue an immunological problem.

The underlying factor of the pathogenesis of systemic connective-tissue diseases is disorganization of cellular and humoral immunity associated with the thymus-dependent system.

At the same time, endocrine disorders (inhibited activity of the hypophyseal-adrenal system, thyroid dysfunction, gonadal dysfunction, i.e. hyperoestrogenism), neurotic disorders, cooling, injury, and genetic factors are of definite importance in the development of deep dysproteinemic changes with prevalent localization of the pathological process in the basic substance of the connective tissue of the skin, vessels and internal organs.

Scleroderma is encountered both in children of any age (beginning with the first few days of life) and in elderly individuals. Like cicatrizing erythematosus, it develops most frequently in females. Cases may occur in which focal scleroderma transforms to the diffuse, systemic, form. These two forms may therefore be regarded as varieties of a single pathological process occurring with involvement of the skin and internal organs. This point of view, however, has not gained general acceptance.