49 - Keratosis

Question 1

KERATOSIS PILARIS AND OTHER FOLLICULAR KERATOTIC DISORDERS have the common finding of?

Answer 1

follicular keratosis – orthokeratosis of the follicular ostium and infundibulum

Question 2

_________ protrude from the orifices, producing a rough sensation on palpation of the skin

Answer 2

Keratotic plugs

Question 3

KP prevalence is higher in populations with what associated conditions?

Answer 3

• ichthyosis vulgaris
  -atopic dermatitis (association with AD is not independent of IV), -hypothyroidism,
  -cushing,
  -insulin dependent DM,
  -obesity or high BMI
  -down syndrome.
• Noonan syndrome. Cardiofaciocutaneous (CFC) syndrome.

Question 4

Affected areas of KP?

Answer 4

lateral cheeks, extensor aspects of the upper arms and buttocks

rarely may be more extensive, extending to the distal limbs and the trunk

Question 5

Affected areas for
1. Children
2. adolescents and adults

Answer 5

younger children – face and arms
adolescents and adults – extensor arms and legs

Question 6

KP is accentuated and improved by

Answer 6

Accentuated - ambient humidity
Improved - during summer

Question 7

variant of KP where erythema is markedly noticeable extending beyond the perifollicular skin?

Answer 7

Keratosis pilaris rubra (KPR, Keratosis follicularis rubra)

Question 8

similar and likely related condition characterized by hyperpigmentation in addition to erythema and follicul. bb ar papule

Answer 8

Erythromelanosis follicularis faciei et colli (EFFC)

Question 9

Erythromelanosis follicularis faciei et colli (EFFC) involves the

Answer 9

preauricular and maxillary areas, usually in a symmetric distribution, with spread in some casesto the temples and sides of the neck (the suffix “colli” refers to the neck) and trunk

Question 10

Erythromelanosis follicularis faciei et colli (EFFC) is seen primarily in what population

Answer 10

adolescents and young adults, most commonly in males

Question 11

KP arises because of

Answer 11

defective keratinization of the follicular epithelium

Question 12

Follicular hyperkeratosis could arise because of

Answer 12

1. mutations in FLG (the cause of IV),
2. hyperandrogenism
3. insulin resistance or other genetic or metabolic abnormalities
4. hair shaft defect or sebaceous etiology and follicular plugging

Question 13

Dermoscopy of KP?

Answer 13

thin short hair shafts that are coiled or twisted within the follicular ostia

Question 14

KP histopathology findings?

Answer 14

varying degrees of follicular hyperkeratosis, dilatation of the upper dermal vessels, perivascular lymphocytic inflammation and atrophy or absence of sweat glands.
EFFC - hyperpigmentation of the basal layer.

Question 15

KP prognosis?

Answer 15

improve by adolescence or early childhood
persist into later adult life in 1/3 of patients.

Question 16

Treatment of KP?

Answer 16

• avoiding drying or irritating skin care products
• regular use of bland emollients
• Keratolytic preparations
  containing urea, lactic acid, or salicylic acid may soften and smooth KP
• Topical retinoids may also be tried if keratolytics are not helpful, but these preparations may aggravate associated erythema, limiting their value.
  -short courses of low-potency topical corticosteroids calm irritation and itch , do not improve the keratotic plugging.
• Vascular or pigment specific laser - modestly improves the erythema or hyperpigmentation
  associated with KP.

Question 17

Keratosis pilaris atrophicans (KPA) is a group of rare disorders characterized by

Answer 17

follicular keratosis;
inflammation;
and secondary atrophic scarring, alopecia, or both.

Question 18

Ulerythema ophryogenes
(UO; also called keratosis pilaris atrophicans faciei [KPAF]) particulary affects the?

Answer 18

Eyebrows

Involvement of the cheeks and forehead can occur, but the scalp and eyelash hair are normal.

Question 19

Ulerythema ophryogenes onset at what age group?

Answer 19

Infancy

Question 20

Ulerythema ophryogenes presents as?

Answer 20

erythema and small keratotic follicular papules involving the lateral third of the eyebrows

Question 21

Ulerythema ophryogenes progression?

Answer 21

slowly progress through childhood to involve more of the eyebrows, leading to alopecia

• Progression usually ceases after puberty, but the sequelae are permanent

Question 22

Ulerythema ophryogenes progression?

Answer 22

slowly progress through childhood to involve more of the eyebrows, leading to alopecia

• Progression usually ceases after puberty, but the sequelae are permanent

Question 23

What is the clinical presentation of Atrophoderma vermiculatum (AV; also called folliculitis ulerythematosa reticulata)

Answer 23

erythema and follicular plugging on the cheeks that progresses to reticular, atrophic scarring (worm eaten, honeycomb)

the forehead, ears, upper lip, and rarely the neck and extremities can be affected
as well.

Question 24

Population affected in Atrophoderma vermiculatum (AV; also called folliculitis ulerythematosa reticulata)?

Answer 24

usually starts in childhood, between 5 and 12 years old,

Question 25

Etiology /mode of inheritance of AV?

Answer 25

sporadic, although an affected father and daughter have been reported, suggesting possible autosomal dominant inheritance.

Question 26

KP and UO are strongly associated with?

Answer 26

Noonan syndrome and Cardiofaciocutanous syndrome

80% with CFC have KP
90% with CFC have UO

Question 27

What is the triad of KERATOSIS FOLLICULARIS SPINULOSA DECALVANS (KFSD) AND ICHTHYOSIS FOLLICULARIS, ATRICHIA, AND PHOTOPHOBIA (IFAP) SYNDROME?

Answer 27

Follicular hypereratosis
Alopecia or atrichia
Photophobia