49 - Keratosis Pilaris and Other Follicular Keratotic Disorders

Question 1

Orthokeratosis of the follicular ostium and infundibulum

Answer 1

Follicular keratosis

Question 2

Variant of keratosis pilaris in which erythema is markedly noticeable, extending beyond the perifollicular skin

Answer 2

Keratosis pilaris rubra or keratosis follicularis rubra

Question 3

Hyperpigmentation in addition to erythema and follicular papules
Involves the preauricular and maxillary areas

Answer 3

Erythromelanosis follicularis faciei et colli

Question 4

Dermoscopic findings of thin, short hair shafts that are coiled or twisted within the follicular ostia

Answer 4

Keratosis pilaris

Question 5

Strongly associated with Noonan and cardiofaciocutaneous syndromes

Answer 5

Keratosis pilaris

Ulerythema ophryogenes

Question 6

Group of rare disorders characterized by follicular keratosis, inflammation, and secondary atrophic scarring, alopecia, or both

Answer 6

Keratosis pilaris atrophicans

Question 7

The umbrella of keratosis pilaris atrophicans classically covers 3 clinical entities

Answer 7

Ulerythema ophryogenes
Atrophoderma vermiculatum
Keratosis follicularis spinulosa decalvans

Question 8

Allelic to ichthyosis follicularis, atrichia, and photophobia (IFAP) syndrome

Answer 8

Keratosis follicularis spinulosa decalvans

Question 9

Onset in infancy
Erythema and small keratotic follicular papules involving the lateral third of the eyebrows
Progression usually ceases after puberty

Answer 9

Ulerythema ophyrogenes

Question 10

Erythema and follicular plugging on the cheeks that progresses to reticular, atrophic scarring
“Worm-eaten” and “honeycomb” morphology
Alopecia is not a feature of the disease
Sparse open and closed comedones and milia may be found

Answer 10

Atrophoderma vermiculatum

Question 11

X-linked disorders characterized by the triad of follicular hyperkeratosis, alopecia or atrichia, and photophobia

Answer 11

Keratosis follicularis spinulosa decalvans

Ichthyosis follicularis, atrichia, and photophobia syndrome

Question 12

Alopecia in KFSD vs IFAP syndrome

Answer 12

KFSD - progressive, scarring alopecia that begins in childhood
IFAP syndrome - nonscarring, congenital atrichia

Question 13

Y/N - KFSD phenotype is less severe than IFAP

Answer 13

Yes

Question 14

Variant of KFSD characterized by persistent pustule formation, especially on the scalp
More severe inflammation, and alopecia is progressive after puberty

Answer 14

Folliculitis spinulosa decalvans

Question 15

Gene mutation in KFSD and IFAP syndrome

Answer 15

MBTPS2

Question 16

Folliculocentric viral infection occurring in the setting of immune suppression

Answer 16

Trichodysplasia spinulosa

Question 17

Most common affected area in trichodysplasia spinulosa

Answer 17

Central face

Question 18

Ecological agent in trichodysplasia spinulosa

Answer 18

Trichodysplasia spinulosa-associated polyomavirus

Question 19

Hypertrophic hair bulb with an expanded inner root sheath whose cells contain numerous eosinophilic trichohyaline granules

Answer 19

Trichodysplasia spinulosa