Lupus is an autoimmune disease.

Symptoms may be local and limited to the skin , or generalized with marked systemic involvement.

No sharp line can be drawn between the different types.

(A) The localized variety occurs in three forms:

(i) The chronic discoid type is the commonest variety. It especially affects women around 40 yrs of age. Lesions occur most frequently on the face where they assume a butterfly distribution or persists as discrete discoid patches. The scalp may be involved. A sharply defined plaque of congestive erythema is covered by scales, underneath which small plugs extend into the enlarged hair follicles. As congestion subsides atrophic scarring and telangiectasia become evident. Slow or rapid centrifugal extension may occur.

Telangiectasias are small dilated blood vessels near the surface of the skin or mucous membranes, measuring between 0.5 and 1 millimeter in diameter. They can develop anywhere on the body but are commonly seen on the face around the nose, cheeks, and chin. They can also develop on the legs, specifically on the upper thigh, below the knee joint, and around the ankles.

Some telangiectasia are due to developmental abnormalities that can closely mimic the behaviour of benign vascular neoplasms. They may be composed of abnormal aggregations of arterioles, capillaries, or venules. Because telangiectasias are vascular lesions, they blanch when tested with diascopy.

(ii) In the acute localized edematous type the lesions are circumscribed edematous plaques, which may completely disappear or may develop into chronic discoid lesions.

(iii)In the chronic disseminated type, numerous extensive lesions of the chronic discoid type are present but systemic symptoms are slight or absent.

(B)Systemic Lupus Erythematosus (Disseminated Lupus Erythematosus)may follow discoid and other local skin lesions, it much more commonly starts as a generalized disease. The manifestations in different organs are numerous.

Symptoms vary in different cases.-(1) Pain in joints is the first to be complained of in 90% of patients. There may be a general aching of the joints and muscles (resembling fibrositis), a migratory form of polyarthritis or a chronic form hardly distinguishable from rheumatoid disease. (2) A severe constitutional disturbance follows , with a high evening temperature , profuse sweating, loss of appetite and loss of weight. (3) Anaemia is usually noticed and this is accompanied by a polymorph leucopenia; sometimes a severe haemolytic anaemia arises. (4)Skin changes are of several different types and tend to occur late; purpura, petechiae, ulcers in mouth and less commonly rashes which resemble lichen planus or psoriasis. Alopecia areata may be found. (5) Enlarged and sometimes tender superficial lymph glands are sometimes associated with enlargement of the liver and spleen. (6) Pleurisy often occurs but large effusions are rare; pulmonary infiltration and consolidation may give rise to severe dyspnoea. (7) The heart and blood vessels may show a variety of changes; myocardial damage gives rise to enlargement and failure, and pericarditis or endocarditis can be found. Vascular occlusion of the vessels of the fingers gives rise to the Raynaud phenomena and on occasion local gangrene occurs. (8) Renal involvement causes albuminuria microscopic haematuria and renal casts ; oedema and later uraemia are always of serious significance. (9) Epileptiform convulsions, hemiplagia and psychoneurosis are mainly the result of vascular damage to the brain. (10) Abdominal pain , nausea and vomiting may be met.The natural course is subject to spontaneous remissions.

Antiphospholipid syndrome, also known as “sticky blood,” is an autoimmune disorder in which the body makes antibodies to its own blood proteins. Antiphospholipid syndrome (APS) can occur in individuals without any associated disease. This is called primary APS. The disorder may also occur with systemic lupus erythematosus (SLE) or another autoimmune disorder. This is called secondary APS.

How frequently antiphospholipid syndrome occurs is not yet known. APS antibodies can be found in as many as 50% of people with lupus and in about 1-5% of the rest of the population. Antiphospholipid syndrome most commonly occurs in young to middle-aged adults, but it can begin at any age.

Symptoms

Along with the antibodies, the body begins producing blood clots. The blood clots can block arteries and veins and thus cut off blood supply to a part of the body. The symptoms the individual experiences come from the effects of the blood clots:

Veins or arteries of the arms or legs – may cause pain, swelling, numbness, tingling in the hands or feet, or leg ulcers. If the blood supply was completely cut off to a part, such as a toe, the individual could lose the toe.

Arteries of the heart – may cause chest pain or heart attack. The individual may also have a heart murmur or heart valve problems.

Blood vessels of the skin – may cause bruises (purpura) or a blotchy, bluish rash called livedo reticularis.

Blood vessels of the brain – if a clot cuts off blood supply to a part of the brain, this causes a stroke. An individual with APS may also experience migraine headaches or seizures.

Placenta during pregnancy – Women with APS may have frequent miscarriages or premature births.

Serious antiphospholipid syndrome, called catastrophic APS, occurs when many internal organs develop blood clots over a period of days to weeks.

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