Keratosis pilaris atrophicans

Keratosis pilaris atrophicans
Synonyms	Atrophoderma vermiculatum, Folliculitis rubra, Keratosis pilaris atrophicans faciei, Ulerythema ophryogenes
Pronounce	N/A
Specialty	N/A
Symptoms	Follicular hyperkeratosis, erythema, atrophy
Complications	Scarring, alopecia
Onset	Childhood
Duration	Chronic
Types	N/A
Causes	Genetic
Risks	Family history
Diagnosis	Clinical diagnosis, skin biopsy
Differential diagnosis	Keratosis pilaris, Ichthyosis vulgaris, Lichen spinulosus
Prevention	N/A
Treatment	Emollients, keratolytics, topical retinoids
Medication	N/A
Prognosis	N/A
Frequency	Rare
Deaths	N/A

Keratosis Pilaris Atrophicans (KPA) is a rare type of keratosis pilaris (KP), which is a common skin condition. KPA is characterized by the presence of rough, small bumps on the skin, similar to KP, but it also leads to scarring and hair loss in the affected areas. This condition is considered a variant of keratosis pilaris and falls under the category of follicular keratosis. It primarily affects the hair follicles on the scalp, face, and occasionally the eyebrows and eyelashes, leading to progressive, irreversible hair loss and atrophic scarring.

Symptoms and Signs

Keratosis Pilaris Atrophicans presents with several distinct features:

• Follicular papules: Small, rough bumps around hair follicles.
• Atrophy: Thinning of the skin in the affected area.
• Alopecia: Hair loss, which can be progressive and permanent.
• Scarring: Scar formation in the place of lost hair follicles.

The condition is often noticed in childhood or adolescence and may worsen during puberty. It is not contagious, painful, or itchy, but it can cause cosmetic concerns and emotional distress.

Types

Keratosis Pilaris Atrophicans can be classified into three main types:

1. Keratosis Pilaris Atrophicans Faciei (KPAF): This type primarily affects the face, especially the cheeks, and is more common in children.
2. Atrophoderma Vermiculatum: A variant that leads to a honeycomb appearance of the skin, usually on the cheeks.
3. Ulerythema Ophryogenes: This type affects the eyebrows and, sometimes, the forehead, leading to loss of eyebrow hair and scarring.

Causes and Risk Factors

The exact cause of Keratosis Pilaris Atrophicans is unknown, but it is believed to be genetic in nature, with several cases reported in families. It is associated with a mutation in the LMBR1 gene on chromosome 7. Other risk factors include having a family history of KP or KPA and certain genetic disorders such as Noonan syndrome and Cardiofaciocutaneous syndrome.

Diagnosis

Diagnosis of KPA is primarily clinical, based on the appearance of the skin and a family history of similar conditions. Dermatologists may perform a skin biopsy to confirm the diagnosis and rule out other conditions.

Treatment

There is no cure for Keratosis Pilaris Atrophicans, but treatments are available to manage symptoms and improve the appearance of the skin. Options include:

• Topical treatments: Retinoids, corticosteroids, and emollients to smooth the skin and reduce inflammation.
• Laser therapy: To reduce redness and improve skin texture.
• Hair transplantation: In cases of significant hair loss.

Prognosis

The prognosis for individuals with KPA varies. While the condition can cause permanent hair loss and scarring, it does not affect overall health. Early intervention can help manage symptoms and improve cosmetic appearance.

See Also

• Keratosis Pilaris
• Skin Conditions
• Genetic Disorders

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