Collagen diseases — commonly referred to as collagen vascular disease or connective tissue disease — are in a class of autoimmune disorders in which the body’s immune system attacks its own tissues. Bodies are held together by tissues made up of two proteins (collagen and elastin) that connect each person’s internal structures. For individuals diagnosed with collagen vascular disease, their collagen and elastin become inflamed, and, as a result, the proteins and the body parts they connect become damaged.
While each of the following types of connective tissue disease varies regarding symptoms and viable treatment options, they do have one thing in common. All autoimmune diseases are chronic conditions that require management throughout a patient’s lifetime. For patients with connective tissue disease, such as lupus, dermatomyositis, scleroderma, or other skin conditions, our dermatology associates at Advanced Dermatology and Skin Cancer Center are committed to helping find an effective, personalized treatment that meets their needs while providing compassionate care. Contact us to schedule an appointment today.
Lupus
Lupus erythematosus is a collection of autoimmune diseases in which the human immune system becomes hyperactive and attacks healthy tissues. It can be confined to the skin — discoid lupus erythematosus (DLE) — without presenting signs that the whole body is affected; present a certain degree of systemic findings, but mostly manifest in the skin — subacute lupus — or manifest as a systemic disease with significant complications and present with characteristic skin findings — acute lupus erythematosus (ALE).
Discoid Lupus Erythematosus (DLE)
Discoid lupus erythematosus, or DLE, is the most common chronic form of cutaneous lupus. It’s characterized by a discoid lupus rash — persistent, scaly, and disc-like plaques that can cause changes in pigmentation, scarring, and hair loss. Lesions from DLE usually occur in areas of the body regularly exposed to the sun’s ultraviolet (UV) rays, including the scalp, face, ears, and torso. Factors leading to DLE include genetic predisposition, exposure to sunlight, toxins, and hormones. DLE can affect both males and females of any age. Signs of localized DLE include the following.
- Early signs of lupus include dry, red patches
- Red or hyperpigmented plaques with adherent scales
- Follicular keratosis beneath the surface scales
- Scarring, resulting in white patches and tissue loss
- Temporary or permanent patches of hair loss
- Hypertrophic lupus erythematosus (red, thickened plaques)
The main concern for patients with DLE is the unsightly appearance of plaques, but the plaques may also cause discomfort, soreness, or itchiness. Discoid lupus rash treatment options may include year-round protection from sun exposure, intermittent courses of topical corticosteroids, and systemic therapy, which may be used alone or in combination with other therapies.
Subacute Cutaneous Lupus Erythematosus (SCLE)
Subacute cutaneous lupus erythematosus, or SCLE, is a subtype of cutaneous lupus erythematosus that appears as a widespread, non-scarring, photosensitive (light-sensitive) rash. SCLE typically presents with more dramatic and extensive lesions than that of DLE on areas of the body exposed to sunlight and with multiple geometrically varied or circular lesions on the back or upper torso. This lupus body rash presents as annular plaques with raised borders and a central clearing. The plaques combine to form multiple circular patterns, which may or may not have an overlying scale. In some cases, this type of lupus flare up can appear with vesicles, crusting, large, fluid-filled blisters, and symmetrical rashes distributed on sun-exposed skin, such as the upper arms, outer arms, and neck. Treatment for SCLE includes sun protection and topical therapy or oral therapy.
Acute Cutaneous Lupus Erythematosus (ACLE)
Acute cutaneous lupus erythematosus, or ACLE, usually presents with a butterfly rash on the face and other sunlight-exposed areas, along with symptoms such as fever, weakness, arthritis, and several additional systemic findings. Specific features of this condition include a malar eruption or butterfly rash; erythematous papular rash on the arms, sometimes with large plaques that extend on large areas of the body; photosensitivity; mouth ulcers; and blisters and erosions. These lesions typically do not produce scarring, although changes in skin color may occur. The quicker ACLE is diagnosed and treated, the better the prognosis for the patient.
Dermatomyositis
Dermatomyositis is a serious, uncommon inflammatory disease characterized by muscle weakness and a distinctive skin rash. This condition can affect both adults and children — juvenile dermatomyositis — and it typically affects a greater number of females than males. While there is no known cure for dermatomyositis, periods of symptom improvement can occur. Dermatomyositis treatment can help clear the skin rash and help patients regain muscle function and strength. The exact cause of dermatomyositis is unknown, but research points to viral infection of the muscles or immune system deficiencies as possible culprits. Environmental factors, such as viral infections, sun exposure, certain medications, and smoking, may likewise play a role.
Dermatomyositis Symptoms
The signs and symptoms of this condition can either appear suddenly or develop over time. The most common symptoms of dermatomyositis include skin changes, in which a heliotrope rash (violet or dusky toned rash) develops on the face, eyelids, knuckles, elbows, knees, chest, and back. The rash can be painful and itchy, and it is often the first sign of the condition.
Another common symptom associated with dermatomyositis is muscle weakness. Progressive muscle weakness involves the muscles located closest to the hips, thighs, shoulders, upper arms, and neck, among other parts of the body located closest to the trunk. The weakness typically affects both the left and right sides of the body and becomes more severe over time.
Dermatomyositis Complications
Possible complications of dermatomyositis include the following.
- Aspiration pneumonia. Patients with dermatomyositis may breathe in foods or liquids, including saliva, into their lungs due to difficulty breathing.
- Breathing difficulties. In cases where the chest muscles are affected, patients can experience breathing problems, such as shortness of breath.
- Calcium deposits. Calcium deposits can occur in the muscles, skin, and connective tissues as the disease progresses over time.
- Difficulty swallowing. In cases where the esophagus muscles are affected, patients may have difficulty swallowing, which may lead to malnutrition and weight loss.
Dermatomyositis Treatment
There is no cure for dermatomyositis. Available treatments, however, can improve your skin as well as your muscle function and strength. Treatments for this condition may include medications, such as corticosteroids, antimalarial medications, and sunscreens; therapy, such as physical therapy, speech therapy, and dietetic assessment; and other procedures, such as intravenous immunoglobulin (IVIg) and surgery to remove painful calcium deposits as well as to prevent recurring skin infections. Contact us for more information about treatment options.
Scleroderma
Scleroderma, or systemic sclerosis, is a chronic, connective tissue disease generally classified as part of the autoimmune rheumatic diseases. The name of the disease comes from two Greek words — “sclero,” meaning “hard” and “derma,” meaning “skin. Hardening and tightening of the skin is the most visible manifestation of the disease. Scleroderma typically affects women more often than men, and it most commonly develops between the ages of 30 and 50. While there is no known cure for scleroderma, various treatments can help ease symptoms and improve the quality of life for patients diagnosed with this disease.
Scleroderma Symptoms
There are several different scleroderma types, including localized scleroderma, morphea scleroderma, linear scleroderma, and systemic scleroderma. In some patients, scleroderma affects only the skin, but in others, it can affect the blood vessels, organs, and the digestive tract. The following includes common symptoms associated with scleroderma. If the following applies to you or you seek a scleroderma diagnosis, contact our office to schedule an appointment.
Localized Scleroderma
Localized scleroderma causes changes usually found in only a few skin or muscle regions, which rarely spread elsewhere. Often, localized scleroderma is relatively mild, and the internal organs are not affected. Localized scleroderma is characterized by the accumulation of collagen in the skin, leading to the appearance of thick, hard scaled, or patches.
Morphea Scleroderma
Morphea scleroderma (localized morphea) can develop at any age and usually presents with solitary or multiple smooth, white or yellow patches on the skin with a purplish halo that is hardened or firm to the touch and may itch. As the lesions progress, they may expand, and the skin may lose pigment.
Linear Scleroderma
In linear scleroderma, patients develop thickened streaks of waxy skin on the arm, leg, or forehead instead of patches. In some cases, it forms a long crease on the head or neck commonly called “en coup de sabre” as it resembles a saber or sword wound. This form of scleroderma affects deeper layers of the skin as well as surface layers.
Systemic Scleroderma
Systemic scleroderma, or systemic sclerosis, can affect the skin, esophagus, stomach, bowels, lungs, kidneys, and heart, among other internal organs. It can likewise affect the blood vessels, muscles, and joints. The tissues become hard and fibrous, resulting in reduced function and efficiency. In diffuse scleroderma, skin thickening occurs quickly and is widespread.
Scleroderma Causes
Scleroderma results from an accumulation and overproduction of collagen — a fibrous protein that makes up the body’s connective tissues, including the skin — within the body’s tissues. The causes for abnormal production of collagen in scleroderma patients is unknown; however, the immune system is believed to play a significant role. Scleroderma is likely caused by a combination of environmental factors, genetics, and immune system issues.
Scleroderma Treatment
In some patients, skin problems from scleroderma dissipate on their own within 2-5 years of development. However, this is not the case for many individuals diagnosed with the disease. Treatments for scleroderma may include medications to treat or slow skin changes, dilate blood vessels, reduce digestive symptoms, relieve pain, and prevent infections. Physical and occupational therapies can help patients manage pain, improve strength and mobility, and maintain independence when performing daily tasks.