67 - Scleredema and Scleromyxedema Flashcards
Skin disorder characterized by increased mucin content
Mucinoses
Skin disorder characterized by excessive collagen deposition
Sclerosing disorders
Skin disorder characterized by fibroblast hyperplasia
Fibrosing disorders
Cellular component of the pathogenesis of mucinoses, sclerosing disorders, and fibrosing disorders
Fibrocyte (fibroblast)
Excessive mucin production in
Pretibial myxedema
Collagen deposition in
Scleroderma
Combination of excessive mucin and collagen deposition
Scleredema
Excessive mucin deposition and fibrocyte hyperplasia
Scleromyxedema
Nephrogenix systemic fibrosis
Fibroblasts are derived from _____-positive hematopoietic precursors
CD34
More than half of cases of scleredema occur in childhood or adolescence, most commonly after a/an
Upper respiratory tract infection
_____ infection is most commonly identified in scleredema
Streptococcal
Disease development in adulthood in association with _____ is the second most common presentation of scleredema
Adult-onset diabetes mellitus
Appear to be the major fibroblast products that are increased in scleredema-affected skin
Type 1 collagen
Hyaluronate
Acute onset of nonpitting induration of neck, shoulders, and upper back skin which may be followed by involvement of the face and arms
Affected skin appears smooth and waxy, with tense dermal induration and prominent follicular ostia, at times imparting a “peau d’orange” appearance
Scleredema
Punch biopsies reveal a nontapered (square) appearance
Fibroblasts are normal in number and morphology
Collagen bundles are slightly thickened and separated from each other by subtle deposits of mucin
Sceleredema
Postinfectious scleredema usually abates in
1 to 2 years
_____-associated scleredema is more chronic and can be resistant to many therapies
Gammopathy
Most patients with scleromyxedema have a
Monoclonal paraproteinemia, typically designated as monoclonal gammopathy of undetermined significance
Face is involved in most cases, resulting in significant deformity, “bovine facies”
Trunk and extremities are usually affected
Sclermyxedema
A monoclonal paraproteinemia of undetermined significance, usually _____ is identified in most patients with scleromyxedema
Immunoglobulin G-kappa type
_____ is normal by definition in scleromyxedema
Thyroid function
Classical histopathologic findings consist of superficial to mid-dermal mucin deposition with admixed fibroblast proliferation
Inflammation is rarely a prominent feature
Scleromyxedema
Close histologic resemblance to scleromyxedema
Nephrogenic systemic fibrosis
(Scleromyxedema/Nephrogenic systemic fibrosis) tends to be resctricted to the upper half of the dermis and (scleromyxedema/nephrogenic systemic fibrosis) becomes more prominent in the lower dermis and then extends to pannicular septae
Scleromyxedema
Nephrogenic systemic fibrosis