Acute, or systemic lupus erythematosus

Acute lupus erythematosus may develop spontaneously (a particularly severe form) or in exacerbation of chronic erythematosus. Various stress effects, infections and exposure to ultraviolet rays may be among the causes of the development of chronic discoid or disseminated lupus erythematosus into the acute systemic form. The disease prevails among females at the age of 20 to 40 years. It sets in with a prodromal period characterized by elevation of body temperature and pain in the joints. The skin lesions are pleomorphic, develop violently, and are commonly localized on the face, scalp, and trunk. Erythematous, mildly scaly foci appear first, they spread rapidly and coalesce. Urticaria, bullae and, less frequently, pustules form on these erythematous patches.

The lesions often resemble seborrhoids and the foci of affection in erythema exudativum multiforme and toxico-allergic dermatitis. Itching and burning may be felt.

In very rare cases with systemic lupus erythematosus there are no lesions on the skin and mucous membranes or hardly-noticeable erythematous-oedematous weakly circumscribed foci form.

Various internal organs and systems are involved in acute systemic lupus erythematosus: the cardiovascular system (endocarditis, myocarditis, phlebitis, Raynaud's syndrome, hypotension), the respiratory system (interstitial pneumonia often with accompanying interlobular pleurisy), etc. In affection of the renal glomerular apparatus (lupus nephritis), the prognosis is unfavourable. Vasculitis and polyserositis with involvement of the joints (arthritis, arthralgia), pericarditis, the abdominal syndrome, and periarteritis nodosa may occur. Enlargement of the spleen, liver (interstitial or parenchymatous hepatitis with disturbance of protein, fat, pigment metabolism and antitoxic functions), and lymph nodes is often encountered. Asthenovegetative disorders, meningism, and epileptiform phenomena may develop. Patients complain of increased fatiguability, general weakness, depression, irritability, loss of sleep, increased sweating, anorexia, and nausea. A diagnosis of episcleritis, corneal ulcer, conjunctivitis or angiopathy of the vessels of the fundus of the eye may be made. The blood is characterized by lymphopenia, leucopenia, thrombopenia, a shift of the differential count to the left, and hyper-gammaglobulinemia. A stable increase in the ESR is a characteristic symptom (up to 60-70 mm/hr). A high fever and the discovery of LE cells supplement the picture of systemic erythematosus the clinical picture of which is characterized by a diversity of manifestations mainly because of the complicated immunological and auto-allergic complex of symptoms.

Histopathology. The principal abnormality, fibrinoid degeneration of the collagenous substance, is demonstrated best of all in the viscera but is usually found in the skin also. Besides, vacuolar degeneration and atrophy of the cells of the stratum basale are seen clearly in the epidermis and prevalence of leucocytic infiltration of the papillary and subpapillary layers in the dermis.