Perforating Disorders

Question 1

What is the hallmark of perforating disorders?

Answer 1

Transepidermal elimination of dermal components

Question 2

What are the main components of the perforating material?

Answer 2

Collagen and/or elastic fibers

Question 3

What is the most common type of perforating disorder?

Answer 3

Acquired perforating disorder in the setting of scratching in the setting of chronic kidney disease

Question 4

What is the time of onset for familial reactive perforating collagenosis?

Answer 4

Childhood

Question 5

What is the most common location for familial reactive perforating collagenosis?

Answer 5

Arms, hands, sites of trauma

Question 6

What is the perforating substance in familial reactive perforating collagenosis?

Answer 6

Collagen

Question 7

What is the onset of elastosis perforans serpinginosa?

Answer 7

Childhood, early adulthood.

This is variable in the penicillamine-induced EPS

Question 8

What are the most common locations for elastosis perforans serpignosa?

Answer 8

Neck, face, arms, other flexural areas

Question 9

What is perforating folliculitis?

Answer 9

Starts often in early adulthood on the trunk and extremities. The material is necrotic and it is really just normal folliculitis leading to rupture and expression of these materials.

Question 10

What percent of dialysis patients get perforating dz?

Answer 10

10%

Question 11

What is the perforating material in the acquired perforating dermatosis?

Answer 11

Necrotic material, collagen, or (rarely) elastic tissue

Question 12

What group is more susceptible to perforating calcific elastosis?

Answer 12

Very rare, more common in black women

Question 13

What is the most common location for perforating calcific elastosis?

Answer 13

Abdomen /periumbilical

Question 14

What is the perforating substance in perforating calcific elastosis?

Answer 14

Calcified elastic tissue

Question 15

What are the acquired perforating dermatoses?

Answer 15

Acquired reactive perforating collagenosis, Kyrle’s disease, and perforating folliculitis

Question 16

What is the clinical presentation of acquired perforating dermatosis?

Answer 16

Presents with erythematous skin-colored or hyperpigmented papules and papulonodules with central keratotic core - favors extensor extremities

Question 17

What are treatments for acquired perforating dermatosis?

Answer 17

Topical antipruritics (pramoxine), ILK, Unna boot, NB or BB-UVB, topical or oral retinoids

Question 18

What is the histological characteristics of acquired perforating dermatosis?

Answer 18

Composed of transepidermal elimination of collagen and/or elastic fibers + hyperkeratosis a/w epidermal hyperplasia

• Can appear like a ‘reactive’ process b/c collagen and/or elastic fibers seen as ‘foreign’

key is you see the keratotic plug extending through the epidermis, leads to crusted plug

Question 19

What types of perforating disorders have collagen only in the plug?

Answer 19

Familial reactive perforating collagenosis and reactive perforating collaginosis

Question 20

What percentage of elastosis perforans serpiginosa is inherited?

Answer 20

40%

Question 21

What is the material extruded in elastosis perforans serpignosa?

Answer 21

Elastin only

Question 22

What is the clinical apperence of elastosis perforans serpignosa?

Answer 22

Multiple annular erythematous hyperkeratosis papules (+/- papules)

Question 23

What is the histology of elastosis perforans serpignosa?

Answer 23

Hyperplastic epidermis with transepidermal elimination of dermal elastic fibers via channels - surface commonly has scale-crust

Question 24

What is the clinical appearance of perforating calcific elastosis?

Answer 24

p/w verrucous keratotic papules and plaques
- papules localized to the periphery
- Most common in the periumbilical / abdomen >> breast (if superimposed CKD)

Question 25

Histology of perforating calcific elastosis?

Answer 25

Elastic fibers that are coated with calcium