8 - 51 - POROKERATOSIS

Question 1

What are the clinical variants of porokeratosis?

Answer 1

1. Porokeratosis of Mibelli - classic form
2. Disseminated Superficial Actinic Porokeratosis - most common type
3. Disseminated Superficial Porokeratosis
4. Disseminated Superficial Porokeratosis of Immunosuppression
5. Linear Porokeratosis
6. Punctate Porokeratosis
7. Porokeratosis palmaris et plantaris disseminata (porokeratosis punctata palmaris et plantaris)
8. CDAGS syndrome (craniosynostosis and clavicular hypoplasia, delayed closure of the fontanel, anal anomalies, genitourinary malformations, skin eruption), also referred to as CAP syndrome (craniosynostosis, anal anomalies, and porokeratosis)

Question 2

most common type of porokeratosis

Answer 2

disseminated superficial actinic porokeratosis

Question 3

presents with multiple papules distributed symmetrically on sun-exposed areas

Answer 3

disseminated superficial actinic porokeratosis

Question 4

presents at birth or in childhood with lesions distributed along Blaschko lines

Answer 4

Linear porokeratosis

Question 5

appears during or after adolescence as 1- to 2-mm papules on the palms or soles

Answer 5

Punctate porokeratosis

Question 6

corresponds to the hyperkeratotic border and extends throughout the stratum corneum in histologic sections

Answer 6

thin column of parakeratotic cells (cornoid lamella)

Question 7

Malignant epithelial neoplasms are reported in all subtypes except for what variant?

Answer 7

Punctate porokeratosis

Question 8

mode of inheritance of porokeratosis

Answer 8

Autosomal dominant

Question 9

the only autosomal recessive among the clinical variants of porokeratosis

Answer 9

CDAGS Syndrome

craniosynostosis and clavicular hypoplasia, delayed closure of the fontanel, anal anomalies, genitourinary malformations, skin eruption

Question 10

PK is more common in what skin complexion?

Answer 10

fair-skinned individuals

Question 11

Porokeratosis of Mibelli and porokeratosis palmaris et plantaris disseminata are twice as likely to affect men or women?

Answer 11

MEN

Question 12

Disseminated superficial actinic porokeratosis is twice as likely to occur in men or women?

Answer 12

WOMEN

Question 13

Although widespread, lesions of DSAP typically spare what areas?

Answer 13

palms, soles, and mucous membranes

Question 14

Disseminated superficial porokeratosis (DSP) has its onset in what decades of life?

Answer 14

third or fourth decade of life

Question 15

Lesions primarily are morphologically identical to those of DSAP, occur on the extremities, and are typically distributed symmetrically but do not spare sun-protected areas as in DSAP

Answer 15

Disseminated superficial porokeratosis (DSP)

Question 16

area of predilection of Disseminated superficial porokeratosis (DSP)

Answer 16

Extensors

Question 17

Linear porokeratosis occurs in what age group?

Answer 17

early childhood

Question 18

This variant have the **highest potential for malignant degeneration **of all the porokeratoses.

Answer 18

Linear porokeratosis

Question 19

appears during adolescence or adulthood and may be seen concomitantly with other types of porokeratosis

Answer 19

Punctate porokeratosis

Question 20

The cutaneous manifestations are strikingly consistent, with the development of small, widespread porokeratotic papules from 1 month of age in affected individuals, predominantly affecting the face and extremities, with reported photoaggravation of lesions.

Answer 20

CDAGS syndrome (craniosynostosis and clavicular hypoplasia, delayed closure of the fontanel, anal anomalies, genitourinary malformations, skin eruption), also referred to as CAP syndrome (craniosynostosis, anal anomalies, and porokeratosis)

Question 21

A splicing mutation in __________________, involved in the cholesterol biosynthetic pathway, has been shown to cause Porokeratosis of Mibelli.

Answer 21

phosphomevalonate kinase (PMVK)

Question 22

This gene has recently been identified as a causative gene in DSAP

Answer 22

mevalonate kinase (MVK) gene

Question 23

T/F. Although characteristic of porokeratosis, the cornoid lamella is not pathognomonic and may also be found in other conditions.

Answer 23

True

Can also be seen in s viral warts, some ichthyoses, and nevoid hyperkeratoses

Question 24

Therapeutic options for porokeratosis

Answer 24

If the lesions are problematic or cosmetically unacceptable, treatment with potent topical steroids, keratolytics, topical retinoids, topical 5-fluorouracil,46 imiquimod 5%, calcipotriol, 47 anthralin, 48 cryotherapy,49 carbon dioxide laser, 50 pulsed dye laser, 51 or Nd:YAG (neodymium-doped yttrium aluminum garnet) laser may be considered

Question 25

1st line treatment for porokeratosis

Answer 25

Question 26

2nd line treatment for porokeratosis

Answer 26

Question 27

3rd line treatment for porokeratosis

Answer 27

Question 28

prognosis of porokeratosis

Answer 28

The disease is generally considered a benign process; however, malignant degeneration may occur. Malignancy is thought to arise in 7% to 11% of individuals

Question 29

course of porokeratosis

Answer 29

The porokeratoses are generally chronic and progressive, with lesions increasing in size and number with time. Typically, this process occurs over decades in PM, but may be rapid in DSAP, particularly after sun exposure.

Question 30

most frequently associated tumor and may be invasive

Answer 30

SCC

Question 31