The collagen diseases consist of rheumatoid arthritis, lupus erythematosis, dermatomyositis, scleroderma, periarteritis nodosum and serum sickness. All of these can have significant dermatologic manifestation. Of these six, the three so-called collagen diseases; lupus, dermatomyositis and scleroderma will be discussed here. They are considered to be autoimmune diseases (the body reacts against it self).
Lupus erythematosis can be confined to the skin without systemic findings (discoid LE), have some systemic problem but mostly skin manifestations (subacute lupus) or be a much more complicated systemic disease with characteristic skin findings (acute LE).
Discoid lupus presents with red, scaly plaques which develop atrophy (loss of skin substance) with scarring, loss of hair and pigment changes which may be increased pigmentation or complete loss of it. The lesions normally occur on the light exposed areas of the body particularly the face, upper torso and scalp. Therapy is usually quite effective but scarring is often permanent if treatment is delayed.
Subacute LE normally presents with more dramatic and extensive skin lesions, again in light exposed areas and with multiple circular or geometrically varied lesions on the back or upper torso.
Acute LE usually presents with a seriously sick patient often with a butterfly rash over the face and on light exposed areas along with fever, weakness, arthritis, and a host of other systemic findings. The quicker it’s diagnosed and treated, the better the patient usually does.
Dermatomyositis is a serious disease involving the skin and muscles. It can occur in children, in adults and in association with malignancies. The onset is usually abrupt, often following sun exposure with progressive muscle weakness and the development of a cutaneous eruption. Most diagnostic findings of the skin changes occur on the face and hands. Swelling around the eyes, often with dermatitis with a peculiar heliotrope (purplish) coloration. The changes on the hands are reddish plaques or flat-topped violaceous papule-like lesions or dermatitis occurring over the dorsum (top) of the fingers known as Gotton’s papules with sparing of the skin between the knuckles. Telangiectasia over the cuticles of the nails is often found although seen with the other collagen diseases as well. Calcification around joints may be pronounced in children.
Scleroderma (hard skin) occurs in several forms. Most commonly, morphea, (localized morphea) can develop at any age and presents with solitary or multiple reddish smooth patches on the body which are very firm to touch. As the lesions progress, they expand peripherally with a violacous border that gradulaly hardens in the center and often becomes depigmented. The lesions are usually asymptomatic and are more of a cosmetic problem if on an exposed part of the body. On he face, they are often linear running from the anterior scalp down the forehead, eyebrow to end someplace along the line as far as the upper lip. A significant loss of tissue down to bone can be seen. This is referred to as the coup de sabre (sabers’ slash). These can be quite disfiguring and difficult to treat.
Acrosclerosis is a commonly seen variety of sclerodema involving the face with a pinched, tight appearance with decreased ability to open the mouth. Decreased mobility of the hands, tapered fingers with frequent painful ulceration in part of the clinical picture. Spasmodic restriction of the blood flow of the hand with pain and associated color changes often are seen (Raynauds phenomena). Cutaneous calcification may occur around joints of hands and elsewhere. Telengectasia (small superficial blood vessels) in the areas of involvement are often prominent. Decreased esophageal motility (difficulty swallowing) may be a significant problem. When all of the above are found in the same patient, it is called the CREST syndrome.
Systemic scleroderma is seen with many of the above findings but also includes significant internal problems especially involving the lungs. kidneys and gastrointestinal tract. Therapy is often requires a multidiscipline approach.