Dermal Hypertrophies

Question 1

What is the difference between hypertrophic scars and keloids?

Answer 1

Hypertrophic = raised and confined by the wound margin

Keloid = Keloids extend beyond the wound margin

Question 2

Epidemiology of hypertrophic/keloid scars?

Answer 2

Higher incidence in darkly pigmented individuals of African ancestry.

Familial, possibly an autosomal dominant mode of inheritance with incomplete clinical penetrance and variable expression.

Tend to form in individuals between 10 and 30 yo

Question 3

What are the 3 phases of normal wound healing?

Answer 3

Inflammatory

proliferative/granulation

maturation/remodeling

Question 4

What is the the cell and mechanism thought to contribute to the collagen synthesis increase seen in keloids?

Answer 4

Keloidal fibroblasts, and the mechanism is increased TGF-beta and PDGF, decreased production of MMP’s, reduced rate of apoptosis

Question 5

What is a difference in timing between hypertrophic and keloid scars?

Answer 5

Keloids have delayed onset, hypertrophic scars happen w/ the injury (they heal that way)

Question 6

Do keloids have myofibroblasts within nodules?

Answer 6

Somewhat controversial, but usually no or at least decreased as compared to hypertrophic scars where it is more classic to have increased myofibroblasts

Question 7

In what types of scars can mast cells be seen in?

Answer 7

Both hypertrophic and keloids

Question 8

What is the difference in treatment response between keloids and hypertrophic scars?

Answer 8

For resistant lesions, radiation can be considered in keloids, otherwise tx is fairly parellel

Question 9

What is the histology of hypertrophic scars?

Answer 9

Increased fibroblasts/collagen oriented both parallel to the skin surface w/ whorled nodules and vertically oriented vessels

Question 10

Histology of keloids?

Answer 10

Thick hyalinized collagen bundles in a haphazard array with mucinous ground substance and an increased number of fibroblasts.

Early on, there are abundant deposits of fibrillary collagen within the reticular dermis, while mature keloids often have strikingly thick, glassy, homogeneous collagen bundles; the latter are composed of multiple densely packed fibrils and are oriented haphazardly throughout the dermis.

There are fewer, if any, vertically oriented blood vessels, compared with hypertrophic scars.

Both keloids and hypertrophic scars have an absence of subepidermal adnexal structures.

CD34 neg (DFSP), Factor XIIIa neg (DF)

Question 11

What is the best tx of keloids?

Answer 11

Prevention by avoiding non-essential surgeries

Can also use radiation therapy after an excision, cumulative dose of 28 Gy over 2-3 days.

Question 12

What is Dupuytren’s contracture?

Answer 12

Thickening of palmar and digital fascia

• You get flexion contracture of affected digits

Question 13

What cells are present in increased numbers in dupuytren’s contracture?

Answer 13

Myofibroblast proliferation is present followed by excess collagen synthesis

Question 14

Treatment options for Dupuytren’s Contracture?

Answer 14

Fasciectomy/fasciotomy, injection of collagenase

Question 15

What cytokines are thought to be involved in Dupuytren’s contracture?

Answer 15

TGF-Beta₁ and TGF-Beta2

Question 16

Clinical features of Dupuytren’s contractures?

Answer 16

Patients often present late in the course of their disease with flexion contractures of the affected digits.

MC ulnar distribution, ring finger

Begins with a nodule in the fascia of the palm, which grows at varying rates to produce a cord, and then it contracts, producing a flexion contracture

Complications: nerve injury, loss of joint mobility, development of reflex sympathetic dystrophy

Question 17

Histology of Dupuytren’s contracture?

Answer 17

Nodule: the proliferation of myofibroblasts, which subsequently align along lines of tension.

Cord: collagen thickening and myofibroblasts disappear.

Question 18

Main treatments for Dupuytren’s contractures?

Answer 18

Surgical approaches are the mainstay of therapy

These include: Fasciectomy releasing the joint contracture, Postop splinting and ROM exercises are important for a good outcome

Recurrences are common

Injectable collagenase (from Clostridium histolyticum) up to three monthly injections are done per cord, followed by a finger-extension procedure the following day.

Question 19

What is the clinical apperence of cutis verticis gyrata?

Answer 19

In primary CVG, overgrowth of the scalp develops at puberty and progresses to produce symmetric gyrate or cerebriform folding of the skin

aligned A-P direction on the crown and vertex

soft to spongy on palpation

terminal hair density may be reduced on the folds, but not in the furrows.

Question 20

What is the difference between primary and secondary cutis verticis gyrata?

Answer 20

Primary CVG can be subdivided into essential (isolated) and non-essential (associated with neurologic and/or ophthalmologic abnormalities) forms, both of which have a male predominance.

Secondary CVG is less common and has a more equal sex distribution. Onset varies from birth to adulthood and secondary forms may be asymmetric, depending on the underlying etiology

Question 21

What are some disease associations for cutis verticis gyrata?

Answer 21

Primary essential: none

Primary non-essential: neurologic abnormalities (mental retardation, seizures)

Secondary: many, acromegaly, myxedema, insulin resistance, Turn and Noonan syndromes, Fragile X, Klinefelter, TS, Hyper IgE, Graves, Paraneoplastic

Question 22

Histology of cutis verticis gyrata?

Answer 22

Normal skin or diffuse dermal thickening w/ packed hyalinized collagen and increased fibroblasts

Question 23

What are the clinical features of juvenile hyaline fibromatosis/infantile systemic hyalinosis?

Answer 23

Cutaneous: Thickened skin and hyperpigmentation overlying bony prominences (ISH more), perianal nodules, small pearly papules on ears and face, scalp nodules (JHF)

Oral: Thickening of the mucosa, Gingival hypertrophy, periodontal ligament replacement by hyaline fibrous material

MSK: Joint contractures and tumors, osteolytic lesions (JHF)

Visceral involvement can occur in ISH

Question 24

What are some differences between juvenile hyaline fibromatosis and infantile systemic hyalinosis?

Answer 24

ISH: Presents earlier (first 6 months), death in early childhood, visceral involvement, Thickened skin and hyperpigmentation overlying bony prominences

JSF: Presents later (early childhood), no visceral involvement but has skeletal and joint (debilitating), survival into adulthood. Scalp nodules and osteolytic bone lesions

Question 25

Histology of infantile systemic hyalinosis and juvenile hyaline fibromatosis?

Answer 25

Increased # of fibroblasts embedded in hyalinized connective tissue stroma that is homogenous, amorphous, and acidophilic (PAS-positive)

Question 26

What is an elastoma (nevus elastics)?

Answer 26

Firm, skin-colored to yellowish papule which can be clustered

Usually on the trunk

Type of connective tissue nevus

Question 27

What syndrome can elastoma (nevus elastics) be associated with?

Answer 27

Buschke-Ollendorff syndrome

Question 28

What is Elastofibroma dorsi?

Answer 28

Large mass in the deep subcutaneous tissue of the infrascapular area

• Favors older women and Japanese
• May be due to chronic mechanical stress

Question 29

What syndromes are associated with connective tissue nevi?

Answer 29

Tuberous Sclerosis: Shagreen patch

MEN-1: pedunculated collagenomas, facial angiofibromas+endocrine neoplasia

Buschke-Ollendorf: collagenomas or elastomas + osteopikilosis

Proteus: cerebriform plantar connective tissue nevi