What is scleromyxedema?
Scleromyxedema, also known as diffuse/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].
How is scleromyxedema diagnosed?
A skin biopsy of a back sample revealed mucin deposition in the dermis between thick and dissociated collagen fiber bundles, suggesting the diagnosis of scleromyxedema.
What is the most common paraproteinemia in scleromyxedema?
Adults in the third to fifth decades or older are most commonly affected. A paraproteinemia, particularly of immunoglobulin Gλ (IgGλ) type, is almost invariably present in scleromyxedema and sometimes in papular mucinosis.
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 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 is mucinosis skin?
Cutaneous focal mucinosis is a skin condition characterized by the presence of mucin in the dermis. It can be primary (in which mucin is the main histologic finding resulting in a clinically distinct lesion) or secondary (which is associated with disorders in which mucin is an additional finding) [1-5].
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 is reticular erythematous Mucinosis?
Reticular erythematous mucinosis (REM) is a rare chronic dermatosis that is typically characterized by reticular macular erythema or erythematous papules and plaques on the central area of the chest and back of middle-aged women. The pathogenesis and etiology of REM remain undefined at present.
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.
What is the difference between scleroderma and scleromyxedema?
How common is scleromyxedema?
What causes mucinosis?
The cause of the condition is unknown, but it is commonly associated with monoclonal gammopathy. It has also been reported in association with bone marrow cancers as well HIV infection, hepatitis C, exposure to toxic oil and contaminated L-tryptophan.
What is follicular Mucinosis?
Follicular mucinosis is a rare cutaneous mucinosis characterized by accumulation of mucin at the external root sheath and sebaceous glands [1]. It manifests clinically as follicular papules distributed on the trunk, proximal limbs, scalp, and face; it may also exist as indurated plaques [2].
What does reticular rash look like?
Livedo reticularis is a reticular, reddish-violet discoloration of the skin that typically affects the limbs, although it can be generalized. The rash becomes worse with exposure to cold temperatures.
What is reticular skin discoloration?
Livedo reticularis (LR) is a skin symptom. It refers to a netlike pattern of reddish-blue skin discoloration. The legs are often affected. The condition is linked to swollen blood vessels. It may get worse when the temperature is cold.
Is there a cure for follicular Mucinosis?
Follicular mucinosis is suggested to be a localized form of cutaneous T-cell lymphoma. There exists no treatment of choice for follicular mucinosis.
What does livedo reticularis rash look like?
People typically use the phrase “mottled skin” to describe livedo reticularis, a condition that causes a blotchy or web-like pattern of red, blue, or purple lines to appear across the skin. In very deep skin tones, the pattern may be dark brown.