8 - 49 - KERATOSIS PILARIS AND OTHER FOLLICULAR KERATOTIC DISORDERS Flashcards
KERATOSIS PILARIS AND OTHER FOLLICULAR KERATOTIC DISORDERS have the common finding of?
follicular keratosis – orthokeratosis of the follicular ostium and infundibulum
_________ protrude from the orifices, producing a rough sensation on palpation of the skin
Keratotic plugs
KP prevalence is higher in populations with what associated conditions?
- 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.
Affected areas of KP?
lateral cheeks, extensor aspects of the upper arms and buttocks
rarely may be more extensive, extending to the distal limbs and the trunk
Affected areas for
1. Children
2. adolescents and adults
younger children – face and arms
adolescents and adults – extensor arms and legs
KP is accentuated and improved by
Accentuated - ambient humidity
Improved - during summer
variant of KP where erythema is markedly noticeable extending beyond the perifollicular skin?
Keratosis pilaris rubra (KPR, Keratosis follicularis rubra)
Findings are usually limited to the cheeks, forehead, and neck
similar and likely related condition characterized by hyperpigmentation in addition to erythema and follicular papule
Erythromelanosis follicularis faciei et colli (EFFC)
Erythromelanosis follicularis faciei et colli (EFFC) involves what sites?
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
Erythromelanosis follicularis faciei et colli (EFFC) is seen primarily in what population
adolescents and young adults, most commonly in males
KP arises because of what?
defective keratinization of the follicular epithelium
could arise because of mutations in FLG (the cause of IV), hyperandrogenism, insulin resistance, or other genetic or metabolic abnormalities
Follicular hyperkeratosis could arise because of
- mutations in FLG (the cause of IV),
- hyperandrogenism
- insulin resistance or other genetic or metabolic abnormalities
- hair shaft defect or sebaceous etiology and follicular plugging
Dermoscopy of KP?
thin short hair shafts that are coiled or twisted within the follicular ostia
KP histopathology findings?
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.
KP prognosis?
improve by adolescence or early childhood
persist into later adult life in 1/3 of patients.
Treatment of KP?
- 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.
Keratosis pilaris atrophicans (KPA) is a group of rare disorders characterized by
follicular keratosis;
inflammation;
and secondary atrophic scarring, alopecia, or both.
Ulerythema ophryogenes
(UO; also called keratosis pilaris atrophicans faciei [KPAF]) particulary affects the?
Eyebrows
Involvement of the cheeks and forehead can occur, but the scalp and eyelash hair are normal.
Ulerythema ophryogenes onset at what age group?
Infancy
Ulerythema ophryogenes presents as?
erythema and small keratotic follicular papules involving the lateral third of the eyebrows
may slowly progress through childhood to involve more of the eyebrows, leading to alopecia
Involvement of the cheeks and forehead can occur, but the scalp and eyelash hair are normal.
Ulerythema ophryogenes progression?
slowly progress through childhood to involve more of the eyebrows, leading to alopecia
- Progression usually ceases after puberty, but the sequelae are permanent
What is the clinical presentation of Atrophoderma vermiculatum (AV; also called folliculitis ulerythematosa reticulata)
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.
Population affected in Atrophoderma vermiculatum (AV; also called folliculitis ulerythematosa reticulata)?
usually starts in childhood, between 5 and 12 years old,
Etiology /mode of inheritance of AV?
sporadic, although an affected father and daughter have been reported, suggesting possible autosomal dominant inheritance.
KP and UO are strongly associated with?
Noonan syndrome and Cardiofaciocutanous syndrome
80% with CFC have KP
90% with CFC have UO
What is the triad of KERATOSIS FOLLICULARIS SPINULOSA DECALVANS (KFSD) AND ICHTHYOSIS FOLLICULARIS, ATRICHIA, AND PHOTOPHOBIA (IFAP) SYNDROME?
- Follicular hypereratosis
- Alopecia or atrichia
- Photophobia
KP is strongly associated with what diseases?
ichthyosis vulgaris (IV) and atopic dermatitis (AD)
Other conditions in which KP is more prevalent or more prominent include hypothyroidism, Cushing syndrome, insulin-dependent diabetes, obesity or high body mass index, and Down syndrome.
Generalized KP-like skin changes have recently been described as a common cutaneous side effect of what medication?
RAF inhibitor
predominantly involves the cheeks and leads to a striking honeycomb-like atrophy
Atrophoderma vermiculatum
Alopecia is NOT a feature
predominantly affects the eyebrow area, causing scarring alopecia
Ulerythema ophryogenes (KP atrophicans faciei)
The umbrealla of Keratosis pilaris Atrophicans (KPA) has classically covered three clinical entities. What are these?
- ulerythema ophryogenes (UO; also called keratosis pilaris atrophicans faciei [KPAF]),
- atrophoderma vermiculatum (AV; also called folliculitis ulerythematosa reticulata),
- keratosis follicularis spinulosa decalvans (KFSD)
UO has also been reported in association with what syndomes?
isolated woolly hair, Rubinstein-Taybi syndrome, Cornelia de Lange syndrome, and 18p monosomy
Syndromes Associated with Follicular Atrophoderma
Hallmark of KERATOSIS FOLLICULARIS SPINULOSA DECALVANS (KFSD)
Progressive scarring alopecia
Hallmark of ICHTHYOSIS FOLLICULARIS, ATRICHIA, AND PHOTOPHOBIA (IFAP) SYNDROME
Non-scarring congenital atrichia
Characterized by folliculocentric, skin-colored to erythematous papules with spiny spicules, typically affecting the central face
TRICHODYSPLASIA SPINULOSA
Trichodysplasia spinulosa is caused by what virus
Caused by trichodysplasia spinulosa-associated polyomavirus
what part of the hair follicle is affected in trichodysplasia spinulosa?
Inner root sheath
histopathologic finding of trichodysplasia spinulosa
hypertrophic hair bulb with an expanded inner root sheath whose cells contain numerous eosinophilic trichohyaline granules
