Is scleromyxedema fatal?

Is scleromyxedema fatal?

Scleromyxedema is described as an unpredictable disease, but is usually progressive, debilitating, and lethal in the absence of a successful treatment. Death may result from complications with extracutaneous involvement or treatment side effects.

Is scleromyxedema an autoimmune disease?

Scleromyxedema (SM) is a rare disorder which initially presents with waxy skin stiffness and maculopapular lesions. It also has non-dermatologic manifestations, such as serum paraproteinemia and myopathies, and is sometimes associated with other autoimmune disorders.

What is lichen myxedematosus?

Lichen myxedematosus (LM; also called papular mucinosis) is a chronic idiopathic cutaneous mucinosis characterized by lichenoid papules, nodules, and/or plaques; mucin deposition and a variable degree of fibrosis in the dermis; and an absence of associated thyroid disease [1].

What monoclonal gammopathy is associated with generalized lichen myxedematosus?

Key features. Papular mucinosis (lichen myxedematosus) is composed of linear arrays of waxy papules. Scleromyxedema is a variant in which the papules coalesce with diffuse sclerosis of the skin. An immunoglobulin (Ig) G lambda gammopathy is associated with scleromyxedema and many cases of papular mucinosis.

What is the difference between scleroderma and Scleromyxedema?

Scleromyxedema presents with 2-4 mm waxy papules distributed symmetrically on the arms, hands, and face. [2] It is differentiated from scleroderma by the papular component and the absence of teleangiectasia.

How common is Scleromyxedema?

Summary. Scleromyxedema is a rare, severe skin disorder.

What causes Scleromyxedema?

Scleromyxedema is often associated with visceral disease. Gastrointestinal findings are most common. Dysphagia from esophageal involvement often occurs, and the stomach or intestine may also be affected. Pulmonary complications with dyspnea caused by restrictive or obstructive disease are also common.

What is mucinosis skin?

Mucinoses are a diverse group of uncommon skin disorders. All involve accumulation in the skin of abnormal amounts of mucin. This is a jelly-like complex carbohydrate substance, called hyaluronic acid, that occurs normally as part of the connective tissue in the dermis or mid-layer of the skin.

Is Morphea contagious?

It may be caused by an unusual reaction of your immune system. In people at increased risk of morphea, it could be triggered by injury to the affected area, medications, chemical toxins, an infection or radiation therapy. The condition isn’t contagious.

Who treats Scleromyxedema?

“Dermatologists are often the first specialists to see these patients and to make the diagnosis of this disabling and potentially fatal condition, whose therapy is challenging,” he said. From his European multi-center study of 30 patients with scleromyxedema and from a personal trial on eight patients, Dr.

What does mucinosis look like?

Typically there are pink papules (small bumps) or plaques (larger raised or thickened areas of skin) with prominent pores which are the hair follicle openings. The mucin (gelatinous clear material) may ooze from these pores, especially if the area is touched or squeezed.

How is mucinosis treated?

Plaquelike cutaneous mucinosis treatments are mostly based on case reports. Antimalarial drugs and topical or systemic corticosteroids are the most frequently used.

Is there a cure for follicular mucinosis?

[2] Clinicohistopathologic correlation favored a diagnosis of follicular mucinosis in our patient. There are no specific treatments for idiopathic FM. A wait and see approach is usually recommended and most cases resolve within 2 to 24 months.

Is there a cure for follicular Mucinosis?

What are the side effects of morphea?

Morphea can cause a number of complications, including:

• Self-esteem issues.
• Movement problems.
• Widespread areas of hardened, discolored skin.
• Loss of hair and sweat glands.
• Eye damage.

Is follicular mucinosis itchy?

The mucin (gelatinous clear material) may ooze from these pores, especially if the area is touched or squeezed. There is usually loss of hair in the affected areas, sometimes accompanied by scarring. The skin lesions may be itchy.

How common is follicular mucinosis?

Follicular mucinosis is a rare disorder. The primary form occurs in children and young adults. In the absence of other disorders, it commonly shows localized lesions of the face, head and neck that have a tendency to resolve within two months to two years.