67 - Scleredema and Scleromyxedema

Question 1

Skin disorder characterized by increased mucin content

Answer 1

Mucinoses

Question 2

Skin disorder characterized by excessive collagen deposition

Answer 2

Sclerosing disorders

Question 3

Skin disorder characterized by fibroblast hyperplasia

Answer 3

Fibrosing disorders

Question 4

Cellular component of the pathogenesis of mucinoses, sclerosing disorders, and fibrosing disorders

Answer 4

Fibrocyte (fibroblast)

Question 5

Excessive mucin production in

Answer 5

Pretibial myxedema

Question 6

Collagen deposition in

Answer 6

Scleroderma

Question 7

Combination of excessive mucin and collagen deposition

Answer 7

Scleredema

Question 8

Excessive mucin deposition and fibrocyte hyperplasia

Answer 8

Scleromyxedema

Nephrogenix systemic fibrosis

Question 9

Fibroblasts are derived from _____-positive hematopoietic precursors

Answer 9

CD34

Question 10

More than half of cases of scleredema occur in childhood or adolescence, most commonly after a/an

Answer 10

Upper respiratory tract infection

Question 11

_____ infection is most commonly identified in scleredema

Answer 11

Streptococcal

Question 12

Disease development in adulthood in association with _____ is the second most common presentation of scleredema

Answer 12

Adult-onset diabetes mellitus

Question 13

Appear to be the major fibroblast products that are increased in scleredema-affected skin

Answer 13

Type 1 collagen

Hyaluronate

Question 14

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

Answer 14

Scleredema

Question 15

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

Answer 15

Sceleredema

Question 16

Postinfectious scleredema usually abates in

Answer 16

1 to 2 years

Question 17

_____-associated scleredema is more chronic and can be resistant to many therapies

Answer 17

Gammopathy

Question 18

Most patients with scleromyxedema have a

Answer 18

Monoclonal paraproteinemia, typically designated as monoclonal gammopathy of undetermined significance

Question 19

Face is involved in most cases, resulting in significant deformity, “bovine facies”
Trunk and extremities are usually affected

Answer 19

Sclermyxedema

Question 20

A monoclonal paraproteinemia of undetermined significance, usually _____ is identified in most patients with scleromyxedema

Answer 20

Immunoglobulin G-kappa type

Question 21

_____ is normal by definition in scleromyxedema

Answer 21

Thyroid function

Question 22

Classical histopathologic findings consist of superficial to mid-dermal mucin deposition with admixed fibroblast proliferation
Inflammation is rarely a prominent feature

Answer 22

Scleromyxedema

Question 23

Close histologic resemblance to scleromyxedema

Answer 23

Nephrogenic systemic fibrosis

Question 24

(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

Answer 24

Scleromyxedema

Nephrogenic systemic fibrosis