The integumentary symptoms that result from diseases or disorders that affect the strong, fibrous insoluble protein found in the connective tissue and the vascular structures within tissues.

Scleroderma is a hardening and tightening of the skin which makes the skin appear shiny and smooth.

Calcinosis cutis is when calcium deposits form in the skin.

Subacute cutaneious lupus erythematosus (SCLE) is an uncommon skin rash that is made worse by sunlight.

Dermatomyositis is a patchy and usually a bluish-purple color rash that appears on the face, eyelids, chest, nail cuticle areas, knuckles, knees, or elbows.

Collagen vascular diseases can result in manifestations that localize in independent body systems, or that become widespread and affect multiple systems. The following conditions reflect manifestations that affect the integumentary system.

Although the exact cause of scleroderma is unknown, primary vessel injury or autoimmune dysfunction may contribute to the cause. It may also be linked to exposure to environmental toxins, occupational hazards, such as silica dust, or to individuals who work with vibrating machinery, plastics, or in the mining industry.

Calcinosis cutis, a condition resulting from systemic scleroderma (see Analogous Diagnostic Code: 6399-6350 Scleroderma), is caused by abnormal calcium or phosphate metabolism resulting in persistent hypercalcemia, or hyperphosphatemia, or both. This results in excessive deposits of calcium salts and phosphate in the blood vessel walls. Dietary calcium intake is not a contributing factor in this condition, though certain dietary alterations may be of minor benefit.

Evidence suggests subacute cutaneous lupus erythematosus is due to the body's immune system over reacting to the effects of sunlight on the skin. It can also be caused by particular medications.

The exact cause of dermatomyositis is unknown. Possible theories include infection, allergic response, and cell-mediated immune system abnormality. The conditions may be associated with various connective tissue disorders, such as rheumatoid arthritis (see Diagnostic Code: 5002 Rheumatoid arthritis) or lupus erythematosus (see Diagnostic Code: 6350 Lupus erythematosus). Genetic predisposition may be a possible cause.

Scleroderma manifestations of the skin are characterized by increase in the connective tissue, resulting in excessive collagen in the dermis. Morning stiffness and polyarthralgia may be early symptoms. Scarring, vascular destruction, fibrotic, degenerative and inflammatory skin changes occur. The skin becomes leathery, hard, stiff, and may become permanently taut and shiny. There may be infection; finger ulcerations; thick, tight skin; edema of the hands, fingers, face, feet, or trunk; and Raynaud's phenomenon. The resulting edema may limit motion over certain joints, such as the wrists, elbows, and shoulders. Fingers and toes may be cyanotic, cool to touch, and sensation to temperature in the hands and fingers may be diminished.

Calcinosis cutis is manifested by abnormal deposits of calcium in the form of small, firm, and white to white-yellow papules that form in the tissues under the skin. The papules discharge a milky white substance. Deposits occur commonly on the hands, fingers, and near joints but can deposit in other areas of the body.

Subacute cutaneious lupus erythematosus is characterized by a rash that appears red and scaly. It mainly appears on the sun exposed areas of the upper body. As the rash heals, the patient may be left with a purple or gray mark for a while.

Dermatomyositis causes capillary injury and necrosis. The usual skin manifestation of dermatomyositis appears as a dusky, red skin rash of the face, neck, shoulders, anterior chest, upper back, and arms. The forearms, elbows, knuckles, periungual area, knees, and ankles are areas where the rash is prominent. Edema, and a purple-red rash over the eyelids are characteristic. Linear telangiectasia and hyperemia of the posterior nail fold may be evident.

Complete history and physical examination may be necessary. Depending on the specific condition, various testing may be indicated. These may include blood studies, skin or muscle biopsy, microscopic capillary nail-bed study, radiological studies, urological studies, autoantibody studies, and electromyogram (EMG).

There is no specific treatment for scleroderma. Anti-inflammatory agents and nonsteroidal anti-inflammatory drugs (NSAIDs) may offer some relief of pain and inflammation. Agents that inhibit collagen thickening may be given. Physical and occupational therapy may be implemented. Nitroglycerine ointment may be applied to fingers with severe ischemia. Bacterial ointment for ulcerative infection may be used. Gloves may add protection and warmth to hands. Other measures may be directed at treating or preventing secondary systemic complications of involved organs.

Calcinosis cutis has no known specific treatment. For extensive, painful deposits, surgery may be done. Dietary changes may offer minor benefit in some cases, such as restricting phosphorous or calcium in the presence of hyperphosphatemia or hypercalcemia, or a ketogenic diet that may prevent crystallization by lowering tissue pH.

Subacute cutaneious lupus erythematosus can be treated with topical corticosteroid creams or ointments. Sunscreen available through prescription may also be provided. Anti-malarial drugs may also be provided, as these types of drugs have anti-inflammatory properties.

Dermatomyositis may be treated with corticosteroids, immunosuppressive therapy, and physical therapy.

Collagen vascular diseases systemically affect multiple system organs that can lead to life-threatening complications. Systemic effects of excess collagen in scleroderma alters normal function of internal organs such as the lungs, kidney, heart, and gastrointestinal (GI) tract. Job modification may be necessary. Special assistive devices may be required to maintain functional ability. Physical therapies may be needed long term to preserve joint mobility and muscle strength. There can be recurrence of calcinosus cutis after surgical removal of deposits. Dermatomyositis has an association with concurrent malignant disease in men over 40 years of age. There may be residual muscle weakness. The potential for recovery of function is poor. Assistive personnel may be necessary for the individual to carry on routine activities of daily living (ADL).

None.

• 38 CFR 4.118 (a) For the purposes of this section, systemic therapy is treatment that is administered through any route (orally, injection, suppository, intranasally) other than the skin, and topical therapy is treatment that is administered through the skin.  

• 38 CFR 4.118 (b) Two or more skin conditions may be combined in accordance with 38 CFR 4.25 only if separate areas of skin are involved. If two or more skin conditions involve the same area of skin, then only the highest evaluation shall be used.