Scleroderma Health Dictionary

A form of circumscribed SCLERODERMA.... morphoea

Also known as scleroderma, a rare autoimmune disorder that can affect many organs and tissues, particularly the skin, arteries, kidneys, lungs, heart, gastrointestinal tract, and

joints. The condition is 3 times as common in women and is most likely to appear between the age of 30 and 50.

The number and severity of symptoms varies. The most common symptom is Raynaud’s phenomenon. Also common are changes in the skin, especially of the face and fingers, which becomes shiny, tight, and thickened, leading to difficulty with movements. Other parts of the body may also be affected, leading to difficulty in swallowing, shortness of breath, palpitations, high blood pressure, joint pain, or muscle weakness. Progression of scleroderma is often rapid in the first few years and then slows down or even stops. In a minority of people, degeneration is rapid, and leads to death from heart failure, respiratory failure, or kidney failure.

There is no cure for scleroderma, but many of the symptoms can be relieved.... systemic sclerosis

A group of diseases affecting CONNECTIVE TISSUE. The term is really outdated since there is no evidence that collagen is primarily involved. Fibrinoid NECROSIS and VASCULITIS are two ‘characteristics’, and autoimmunity reaction may occur in the connective tissue. The latter affects blood vessels and causes secondary damage in the connective tissue. Such conditions are sometimes described as collagen vascular diseases, examples being RHEUMATOID ARTHRITIS, SYSTEMIC LUPUS ERYTHEMATOSUS (SLE), and SCLERODERMA.... collagen diseases

They may be local to the SKIN, or a manifestation of systemic disorders – inherited or acquired. Some major types are described below.

Others appear under their appropriate alphabetical headings: ACNE; ALBINISM; ALOPECIA; ALOPECIA AREATA; APHTHOUS ULCER; BASAL CELL CARCINOMA; BOILS (FURUNCULOSIS); BOWEN’S DISEASE; CALLOSITIES; CANDIDA; CHEILOSIS; CHEIRAPOMPHOLYX; DANDRUFF; DERMATOFIBROMA; DERMATOMYOSITIS; DERMATOPHYTES; DERMOGRAPHISM; ECTHYMA; ERYSIPELAS; ERYTHEMA; ERYTHRASMA; ERYTHRODERMA; ESCHAR; EXANTHEM; FUNGAL AND YEAST INFECTIONS; HAND, FOOT AND MOUTH DISEASE; HERPES GENITALIS; HERPES SIMPLEX; HERPES ZOSTER; IMPETIGO; INTERTRIGO; KELOID; KERATOSIS; LARVA MIGRANS; LICHEN; LUPUS; MADURA FOOT; MELANOMA; MILIARIA; MOLLUSCUM CONTAGIOSUM; MOLE; MYCOSIS FUNGOIDES; NAEVUS; ORF; PEDICULOSIS; PEMPHIGUS; PHOTOCHEMOTHERAPY; PHOTODERMATOSES; PITYRIASIS; PORPHYRIAS; PRURITUS; PSORIASIS; RINGWORM; ROSACEA; SARCOIDOSIS; SCABIES; SCLERODERMA; URTICARIA; VITILIGO; WARTS; XANTHOMATA.

Skin cancer Primary cancer is common and chronic exposure to ultraviolet light is the most important cause. BASAL CELL CARCINOMA is the most common form; squamous cell carcinoma is less common and presents as a growing, usually painless nodule which may ulcerate. Squamous cancer may spread to regional lymph glands and metastasise, unlike basal cell cancer. Occupational exposure to chemical carcinogens may cause squamous carcinoma – for example, cancer from pitch warts or the scrotal carcinoma of chimney sweeps exposed to coal dust in earlier centuries. Squamous carcinoma of the lip is associated with clay-pipe smoking.

Cancer may arise from the population of melanocytes of the skin (see MELANOCYTE; MELANOMA).

Apart from these three most frequent forms of skin cancer, various forms of cancer can arise from cells of the dermis, of which LYMPHOMA is the most important (see also MYCOSIS FUNGOIDES).

Lastly, secondary deposits from internal cancer, particularly from the breast, may metastasise to the skin.

Dermatitis and eczema These are broadly synonymous, and the terms are frequently interchangeable. Eczema is a pattern of in?ammation with many potential causes. Dermatitis is commonly used to suggest an eczema caused by external factors; it is a common pattern of in?ammation of the skin characterised by redness and swelling, vesiculation (see VESICLE), and scaling with intense itching and often exudation (weeping). Fissuring, thickening (licheni?cation – see LICHEN) and secondary bacterial infection may follow. Dermatitis can affect any part of the body. It may be genetically detemined or due to other ‘internal’ factors, such as venous HYPERTENSION in a leg, or stress. Often it is ‘external’ in origin – due to strong irritants or chemical allergens. (See also ALLERGY; ALLERGEN.) ATOPIC DERMATITIS is genetic in origin and usually begins in infancy. It may persist for years, and ASTHMA, allergic RHINITIS and conjunctivitis (see under EYE, DISORDERS OF) – ‘hay fever’ – may be associated. Atopic children tend to have multiple allergies, especially to inhaled allergens such as house-dust mite, cat and dog dander and pollens. Allergy to foods is less common but potentially more dangerous, especially if to nuts, when it can cause acute URTICARIA or even ANAPHYLAXIS. Atopic subjects are particularly prone to persistent and multiple verrucae (see WARTS) and mollusca (see MOLLUSCUM CONTAGIOSUM) and to severe HERPES SIMPLEX infections. (See also ATOPY.)

EXFOLIATE DERMATITIS (PITYRIASIS RUBRA)

Generalised exfoliation and scaling of the skin, commonly with ERYTHEMA. Drugs may cause it, or the disorder may be linked with other skin diseases such as benign dermatoses and lupus erythematosus (see under LUPUS). SUMMER POMPHOLYX is an acute vesicular eczema of the palms and soles recurring every summer. Inhaled allergens are a frequent cause. VENOUS (STASIS) DERMATITIS begins on a lower calf, often in association with PURPURA, swelling and sometimes ulceration. Chronic venous hypertension in the leg, consequent on valvular incompetence in the deep leg veins owing to previous deep vein thrombosis (see VEINS, DISEASES OF), is the usual cause. NEURODERMATITIS A pattern of well-de?ned plaques of licheni?ed eczema particularly seen on the neck, ulnar forearms or sides of the calves in subjects under emotional stress. IRRITANT CONTACT DERMATITIS Most often seen in an industrial setting (occupational dermatitis), it is due to damage by strong chemicals such as cutting oils, cement, detergents and solvents. In almost all cases the hands are most severely affected. ALLERGIC CONTACT DERMATITIS, in contrast, can affect any part of the body depending on the cause – for example, the face (cosmetics), hands (plants, occupational allergens) or soles (rubber boots). Particularly common allergens include metals (nickel and chromate), rubber addititives, and adhesives (epoxy resins).

Treatment Avoidance of irritants and contact allergens, liberal use of EMOLLIENTS, and topical application of corticosteroid creams and ointments (see CORTICOSTEROIDS) are central.... skin, diseases of

The abnormal deposition of calcium salts in the skin, muscles, or connective tissues, forming nodules. The condition occurs in connective tissue disorders such as scleroderma or dermatomyositis. (See also calcification.)... calcinosis

Types of autoimmune disorders that often affect blood vessels and produce secondary connective tissue damage. They include rheumatoid arthritis, systemic lupus erythematosus, polyarteritis nodosa, scleroderma, and dermatomyositis.... connective tissue diseases

n. a skin disease thought to be a type of generalized *scleroderma. It also has features of *Raynaud’s disease, with the hands, face, and feet being mainly affected.... acrosclerosis

(ANA) an autoantibody directed against nuclear membranes, found in systemic *lupus erythematosus and other autoimmune rheumatic diseases, such as *Sjögren’s syndrome, *scleroderma, and inflammatory *myositis.

The different types of ANAs are defined by their target antigen and different profiles have been correlated with clinical features.... antinuclear antibody

n. 1. a crackling sound or grating feeling produced by bone rubbing on bone or roughened cartilage, detected by palpation on movement of an arthritic joint. Crepitus in the knee joint is a common sign of *chondromalacia patellae in the young and *osteoarthritis in the elderly. 2. a similar sound heard with a stethoscope over an inflamed lung when the patient breathes in. 3. a similar sound heard over an inflamed extensor tendon in the hand in *scleroderma caused by thickening of the skin, or over a tendon injured by repetitive use in de Quervain’s *tendovaginitis.... crepitus

a disease with features in common with systemic *lupus erythematosus, *polymyositis, and *scleroderma. It is characterized by high levels of antibodies to ribonucleoprotein and most commonly affects women between 20 and 40 years of age.... mixed connective tissue disease

(osteoclasia) n. dissolution of bone through disease, commonly by infection or by loss of the blood supply (ischaemia) to the bone. In acro-osteolysis the terminal bones of the fingers or toes are affected: a common feature of some disorders involving blood vessels (including *Raynaud’s disease), *scleroderma, and systemic *lupus erythematosus.... osteolysis

the formation of thrombi in arterioles and capillaries, leading to haemolytic anaemia and *thrombocytopenia. The term encompasses primary *haemolytic uraemic syndrome and *thrombotic thrombocytopenic purpura, as well as the microangiopathies that can complicate pregnancy (pregnancy-related haemolytic uraemic syndrome, *HELLP syndrome), *malignant hypertension, *scleroderma, *antiphospholipid antibody syndrome, organ transplantation, and cancer.... thrombotic microangiopathy