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.

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 the start of scleroderma look like?

Nearly everyone who has scleroderma experiences a hardening and tightening of patches of skin. These patches may be shaped like ovals or straight lines, or cover wide areas of the trunk and limbs. The number, location and size of the patches vary by type of scleroderma.

How does Arndt Gottron scleromyxedema work?

ARNDT GOTTRON SCLEROMYXEDEMA: SUCCESSFUL RESPONSE TO TREATMENT WITH STEROID MINIPULSE AND METHOTREXATE NCBI Skip to main content Skip to navigation Resources

What kind of mucinosis does scleromyxedema cause?

Scleromyxedema, also known as generalized and sclerodermoid lichen myxedematosus or Arndt-Gottron disease, is a primary cutaneous mucinosis characterized by a generalized papular and sclerodermoid cutaneous eruption that usually occurs in association with monoclonal gammopathy [1].

What’s the difference between scleromyxedema and lichen myxedematosus?

Scleromyxedema should be distinguished from localized lichen myxedematosus, a form of lichen myxedematosus that presents with waxy, firm papules and plaques involving limited areas. Unlike scleromyxedema, sclerotic features, systemic involvement, and monoclonal gammopathy are absent in localized lichen myxedematosus.

How many people have scleromyxedema in the world?

Scleromyxedema is a rare disorder of unknown cause, with fewer than 150 reported cases in literature. It usually affects middle-aged adults of 30–50 years and is clinically characterized by a widespread symmetric eruption of 2–3 mm firm, waxy, dome-shaped papules, commonly over the hands, forearms, head and neck region, upper trunk, and thighs.

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. Who treats scleromyxedema? “Dermatologists are often the first specialists to see these patients and to make the diagnosis of…