10 - 67 - SCLEREDEMA AND SCLEROMYXEDEMA

Question 1

Skin disorders that are characterized by increased mucin content, excessive collagen deposition, or fibrocyte hyperplasia are designated as

Answer 1

mucinoses, sclerosing disorders, or fibrosing disorders

Question 2

what fibrocyte product predominates in pretibial myxedema?

Answer 2

excessive mucin production

Question 3

what fibrocyte product predominates in scleroderma

Answer 3

collagen deposition

Question 4

what fibrocyte product predominates in scleredema

Answer 4

excessive mucin and collagen deposition

Question 5

excessive mucin and depostition and fibrocyte hyperplasia

Answer 5

scleromyxedema and nephrogenic systemic fibrosis

Question 6

Fibroblasts are derived from CD____-positive hematopoietic precursors

Answer 6

CD34

Question 7

scleredema is most common in what age groups?

Answer 7

chilhood or adolescence

Question 8

scleredema most commonly occurs after _____________

Answer 8

upper respiratory tract infection

Question 9

associated conditions of scleredema

Answer 9

Postinfectious

Diabetes mellitus

Paraproteinemia

Also hyperparathyroidism, connective tissue disease, HIV

Question 10

most commonly identified URTI etiology for scleredema

Answer 10

Streptococcal infection

Question 11

second most common presentation of scleredema

Answer 11

Diabetes mellitus–associated scleredema of adulthood

Question 12

clinical findings of scleredema

Answer 12

An acute onset of nonpitting induration of neck, shoulders, and upper back skin may be followed by involvement of the face and arms. Characteristically, the affected skin appears smooth and waxy, with tense dermal induration and prominent follicular ostia, at times imparting a “peau d’orange” appearance

Question 13

clinical findings of Scleromyxedema (also, lichen myxedematosus)

Answer 13

Question 14

major fibroblast products that are increased in scleredema-affected skin

Answer 14

Type 1 collagen and hyaluronate

Question 15

The skin of the _________ is a favored site for scleredema,

Answer 15

upper trunk (especially the back)

Question 16

stains for mucin

Answer 16

Alcian blue, colloidal iron

Question 17

histopathologic findings of scleredema

Answer 17

Punch biopsies of affected skin reveal a nontapered (square) appearance on low power. The proportion of dermis in dramatically increased in comparison to adjacent nonaffected skin (Fig. 67-2A). A decreased number or higher placement of eccrine units may be appreciated. Fibroblasts are normal in number and morphology. The collagen bundles are slightly thickened and separated from each other by subtle deposits of mucin.

Question 18

Question 19

prognosis and clinical course of scleredema

Answer 19

Postinfectious scleredema usually abates in 1 to 2 years.

Scleredema associated with adult-onset diabetes tends to be protracted, although some patients appear to improve with better glucose control.

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

Question 20

T/F Antibiotics do not appear to affect the course of postinfectious scleredema.

Answer 20

True

Question 21

Chronic, progressive condition characterized by dermal fibrosis and mucinosis and normal thyroid function.

Answer 21

SCLEROMYXEDEMA

Question 22

SCLEROMYXEDEMA is usually associated with what condition?

Answer 22

paraproteinemia (typically immunoglobulin G-kappa)

Question 23

Clinical variants of scleromyxedema

Answer 23

■ Generalized, confluent lichenoid eruption (scleromyxedema).

■ Discrete papular (rarely nodular) eruption on the trunk or extremities (lichen myxedematosus).

■ Localized or generalized lichenoid plaques (but distinct from plaque-like mucinosis/reticulated erythematous mucinosis).

■ Urticarial plaques

Question 24

Clinical findings of scleromyxedema

Answer 24

• The generalized lichenoid eruption consists of numerous minute (1 to 3 mm) papules scattered on the extremities and the trunk.
• Scleromyxedema presents with confluent lichenoid plaques. Individual lesions and plaques may exhibit marked erythema or hyperpigmentation. The face is involved in most cases, resulting in significant deformity, “bovine facies” (Fig. 67-3). The trunk and extremities are usually affected (Fig. 67-4A) and often results in decreased flexibility and range of motion in the involved areas

Question 25

classical histopathologic findings of scleromyxedema

Answer 25

The classical histopathologic findings in scleromyxedema consist of superficial to mid-dermal mucin deposition with admixed fibroblast proliferation.

Question 26

prognosis and clinical course of scleromyxedema

Answer 26

Localized, “self-healing” variants are usually confined to the skin and are self-limiting. However, prospective followup and caution is advised as some cases fitting criteria for localized disease have been associated with internal organ involvement or progression to more generalized disease (so-called atypical papular mucinosis).

Scleromyxedema usually follows a chronic and progressive clinical course with more systemic organ and tissue involvement, and a poor outcome is expected.

Respiratory failure, cerebral disease, and infection usually lead to a gradual decline and death.

Question 26

This medication had been used for scleromyxedema for decades to treat scleromyxedema with variable efficacy

Answer 26

Melphalan

Question 27

Therapeutic options for patients with scleromyxedema

Answer 27

Agents or therapies with reported efficacy include: glucocorticoids, 24 intravenous immunoglobulin, 25 thalidomide, 26 extracorporeal photophoresis, 27 interferon-α, 28 combination chemotherapy, 29and psoralen and ultraviolet A