What are the pathological changes associated with scleroderma?
The clinical and pathologic manifestations result from three distinct processes: 1) severe fibroproliferative vascular lesions of small arteries and arterioles, 2) excessive and often progressive deposition of collagen and other extracellular matrix (ECM) macromolecules in skin and various internal organs, and 3) …
What is morphea scleroderma?
Morphea is an autoimmune disease that causes sclerosis, or scarlike, changes to the skin. Autoimmune diseases occur when the immune system, which normally protects us from bacteria, viruses, and fungi, mistakenly attacks a person’s own body.
What is morphea profunda?
Subcutaneous morphea (also known as morphea profunda) is noted by deep, bound-down, sclerotic plaques. This occurs much deeper in the skin and because of this the typical inflammatory changes and coloration is not generally seen.
What is the diagnostic criteria for scleroderma?
Skin thickening of the fingers extending proximal to the MCPs is sufficient to be classified as SSc, if that is not present, seven additive items apply with varying weights for each: skin thickening of the fingers, finger tip lesions, telangiectasia, abnormal nailfold capillaries, interstitial lung disease or pulmonary …
What does linear morphea look like?
Linear morphea features a single band of thickened, discolored skin. Usually, the indented band runs down an arm or leg, but it might also extend down your forehead. This is referred to as en coup de sabre because it makes the skin look as though it’s been struck by a sword.
What does skin morphea look like?
Morphea is a rare skin condition characterized by small red or purple patches that develop firm white or ivory centers. The affected skin becomes tight and less flexible. Morphea (mor-FEE-uh) is a rare condition that causes painless, discolored patches on your skin.
Is morphea related to lupus?
Morphea is an autoimmune disorder (such as type I diabetes, lupus, vitiligo, or multiple sclerosis, among others). While morphea does not affect lifespan, it can significantly affect a patient’s appearance or have symptoms including itching and pain.
What is the histology of scleromyxedema?
The histology of scleromyxedema is characterized by a triad of mucin deposition, fibroblast proliferation, and fibrosis. 48 This occurs primarily in the mid and upper reticular dermis ( Fig. 147.3 ). Elastin fibers tend to be decreased in quantity and the epidermis may be thin.
What is the difference between localised and generalised scleromyxoedema?
The localised form presents with firm, waxy papules with localised distribution and without systemic features. The generalised form (also known as scleromyxoedema) presents with widespread flesh-coloured papules arranged in lines and progressive skin thickening.
What is the difference between lichen myxedematosus and scleromyxedema?
Lichen myxedematosus and scleromyxedema are related disorders.242 Lichen myxedematosus is the localized form, characterized by a papular, nodular, or plaque eruption, most commonly on the face and hands (also known as papular mucinosis). Scleromyxedema is characterized by a generalized papular and sclerodermoid eruption of the skin.
What are the histologic features of systemic scleroderma?
C. Histological features are indistinct. Localized scleroderma and systemic sclerosis have indistinct histologic findings and require clinical correlation for their classification and subclassification. Systemic sclerosis may involve, in addition to skin and soft tissue, which of the following organs?