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keratosis pilaris atrophicans

Description

Keratosis pilaris atrophicans (KPA) refers to a group of clinically related syndromes in which inflammatory keratosis pilaris leads to atrophic scarring [3]. This condition is characterized by the presence of follicular, horny papules surrounded by an erythematous halo on the cheeks, forehead, chin, and eyebrows [2][8].

KPA can result in alopecia (hair loss) due to the inflammatory keratotic papules causing damage to the hair follicles [6]. It is a group of idiopathic genodermatoses that consists of three unique clinical entities: atrophoderma vermiculatum, keratosis pilaris atrophicans faciei, and keratosis pilaris atrophicans corporis [7].

The symptoms of KPA can vary in severity and may include:

  • Follicular, horny papules surrounded by an erythematous halo
  • Alopecia (hair loss)
  • Atrophic scarring

It is essential to note that KPA is a genetic disorder of keratinization of hair follicles of the skin [9]. The exact cause of KPA is unknown, but it is believed to be related to abnormalities in the keratinization process.

References: [2] GL Fekete · 2021 · Cited by 2 [3] Keratosis pilaris atrophicans refers to a group of clinically related syndromes in which inflammatory keratosis pilaris leads to atrophic scarring (see p. 433). [6] Jun 4, 2024 — Keratosis pilaris atrophicans is a group of related disorders characterized by inflammatory keratotic papules that may result in alopecia ... [7] Keratosis pilaris atropicans is a group of idiopathic genodermatoses that consists of three unique clinical entities: atrophoderma vermiculatum, keratosis pilaris atrophicans faciei, and keratosis pilaris atrophicans corporis. [8] by GL Fekete · 2021 · Cited by 2 — Clinically, it presents with follicular, horny papules surrounded by an erythematous halo of the cheeks, forehead, chin and eyebrows, and it is ... [9] May 12, 2022 — Keratosis pilaris (KP) is a genetic disorder of keratinization of hair follicles of the skin. It is an extremely common benign condition ...

Additional Characteristics

  • Atrophic scarring
  • Follicular, horny papules surrounded by an erythematous halo
  • Alopecia (hair loss)

Signs and Symptoms

Keratosis pilaris atrophicans faciei, a variant of keratosis pilaris, presents with distinct signs and symptoms.

  • Initial signs: The condition begins with photophobia, tearing, ophthalmitis, conjunctival, and corneal inflammation in the first weeks or months of life [1].
  • Follicular papules: Erythematous follicular papules on the face are usually the first sign, followed by a gradual hair loss on the affected areas [3].
  • Rough texture: Patients often report a rough texture (gooseflesh or chicken skin-like appearance) and overall poor cosmetic appearance [6].
  • Hair loss: Thinning or loss of the lateral portion of the eyebrows is a common clinical finding, often seen in conjunction with traditional keratosis pilaris symptoms [7].
  • Chronic inflammation: Chronic inflammation leads to fibrosis, atrophy, shrinkage of the hair bulb, and alopecia [8].

These signs and symptoms can vary in severity and may be accompanied by other skin manifestations. It's essential to consult a dermatologist for an accurate diagnosis and treatment plan.

References: [1] The initial signs are photophobia with tearing, ophthalmitis, and conjunctival and corneal inflammation, which occur in the first weeks or months of life; ... [3] Keratosis pilaris occurs due to abnormal keratinisation of the upper portion lining of the hair follicle. The keratin fills the follicle instead of exfoliating. [6] May 12, 2022 — Keratosis pilaris (KP) patients often report a rough texture (gooseflesh or chicken skin like appearance) and overall poor cosmetic ... [7] Sep 25, 2017 — Thinning or loss of the lateral portion of the eyebrows is a common clinical finding and is often seen in conjunction with traditional keratosis ... [8] Jun 4, 2024 — Chronic inflammation leads to fibrosis, atrophy, shrinkage of the hair bulb, and alopecia.

Additional Symptoms

  • fibrosis
  • atrophy
  • alopecia
  • tearing
  • ophthalmitis
  • conjunctival inflammation
  • corneal inflammation
  • erythematous follicular papules on the face
  • rough texture (gooseflesh or chicken skin-like appearance)
  • thinning or loss of the lateral portion of the eyebrows
  • shrinkage of the hair bulb
  • photophobia
  • chronic inflammation
  • hair loss

Diagnostic Tests

Diagnostic Tests for Keratosis Pilaris Atrophicans

Keratosis pilaris atrophicans, a variant of keratosis pilaris, is a benign skin condition characterized by the appearance of small, rough bumps on the skin. While there are no specific laboratory tests to aid in the diagnosis of keratosis pilaris atrophicans, visual examination and dermoscopy can be used to confirm the diagnosis.

  • Visual Examination: The physical findings of keratosis pilaris atrophicans are limited to the skin, with the skin of the outer-upper arms and thighs being commonly affected. Upon gross examination, the skin may appear rough and dry, with small bumps or papules visible to the naked eye [8].
  • Dermoscopy: Dermoscopy can be used as an adjunctive tool in cases where the diagnosis is uncertain. It involves examining the skin under a dermatoscope, which can help to visualize the characteristic features of keratosis pilaris atrophicans, such as hyperkeratosis and hypergranulosis [6].
  • Genetic Testing: While not specifically used for diagnosing keratosis pilaris atrophicans, genetic testing may offer promise for precise diagnosis and tailored treatment strategies in cases where the condition is associated with underlying genetic mutations [4].

It's worth noting that diagnostic tests are not typically necessary to diagnose keratosis pilaris atrophicans, as the condition can be diagnosed through visual examination alone. However, in cases where the diagnosis is uncertain or there are concerns about other underlying conditions, dermoscopy and/or genetic testing may be used to aid in diagnosis.

References:

[1] May 12, 2022 — No specific laboratory tests aid in the diagnosis of keratosis pilaris (KP). The diagnosis of keratosis pilaris is very straightforward and ...

[4] by DD Desai · 2024 — Genetic testing offers promise for precise diagnosis and tailored treatment strategies, yet its utility in these conditions remains ...

[6] Diagnostic Tests & Interpretation ... Diagnosis is visual. Dermoscopy may be of assistance if diagnosis is in doubt (1). ... Hyperkeratosis, hypergranulosis, and ...

[8] May 12, 2022 — Physical findings of keratosis pilaris (KP) are limited to the skin. Upon gross examination, the skin of the outer-upper arms and thighs is ...

Additional Diagnostic Tests

  • Genetic Testing
  • Dermoscopy
  • Visual Examination

Treatment

Treatment Options for Keratosis Pilaris Atrophicans

Keratosis pilaris atrophicans, a type of keratosis pilaris, is a skin condition characterized by the appearance of follicular plugs and atrophy of the affected skin. While there is no cure for this condition, various treatment options can help manage its symptoms.

Topical Treatments

Several topical treatments have been found to be effective in improving the symptoms of keratosis pilaris atrophicans:

  • Pimecrolimus (Elidel) and tacrolimus (Protopic): These topical immunomodulators have been shown to reduce inflammation and improve skin texture [1].
  • Diclofenac gel: This topical NSAID has been found to be effective in reducing inflammation and improving symptoms, although it can be expensive and not frequently covered by insurance [3].
  • Tretinoin: A topical retinoid available as creams or gels, tretinoin has been shown to improve skin texture and reduce the appearance of follicular plugs [5].

Other Treatment Options

In addition to topical treatments, other options have been explored:

  • Pulse dye laser: This treatment can help reduce inflammation and improve skin texture [2].
  • Intense pulsed light (IPL) therapy: Similar to pulse dye laser, IPL therapy can also help reduce inflammation and improve symptoms [2].

Important Notes

It's essential to note that while these treatments can be effective in managing the symptoms of keratosis pilaris atrophicans, there is no cure for this condition. Additionally, treatment options may vary depending on individual circumstances.

References:

[1] Context 1: May 12, 2022 — Keratosis pilaris may be treated with topical immunomodulators such as pimecrolimus (Elidel) or tacrolimus (Protopic).

[2] Context 2: Pulse dye laser or intense pulsed light (IPL) therapy can help reduce inflammation and improve skin texture.

[3] Context 3: An emerging treatment option is topical diclofenac gel applied daily to affected areas; however, it is often very expensive and not frequently covered by insurance.

[5] Context 5: Tretinoin topical is available as 0.025%, 0.05%, and 0.1% creams and 0.01%, 0.025%, 0.04%, and 0.1% gels.

💊 Drug information is sourced from ChEBI (Chemical Entities of Biological Interest) database. Always consult with a healthcare professional before starting any medication. Click on any medication name for detailed information.

Differential Diagnosis

Keratosis pilaris atrophicans faciei (KP-AF) is a variant of keratosis pilaris that affects the face and can be challenging to diagnose due to its similarities with other skin conditions. Here are some key points to consider when making a differential diagnosis for KP-AF:

  • Acne Vulgaris: KP-AF can be confused with acne vulgaris, particularly in its early stages. However, KP-AF typically presents with hyperkeratotic papules and follicular plugs, whereas acne is characterized by comedones, papules, and pustules [3].
  • Disseminate and Recurrent Infundibular Folliculitis: This condition can also present with follicular hyperkeratosis and atrophy, similar to KP-AF. However, it typically involves the trunk and limbs, rather than the face [3].
  • Eruptive Vellus Hair Cysts: These cysts can be mistaken for KP-AF due to their follicular location and keratinous content. However, they are usually larger and more fluctuant than KP-AF lesions [3].
  • Follicular Eczema: This condition can present with erythematous, hyperkeratotic papules, similar to KP-AF. However, it typically involves the scalp or other areas of the body, rather than the face [5].

To make an accurate diagnosis of KP-AF, it's essential to consider these differential diagnoses and rule out other conditions that may present with similar symptoms.

References:

[3] - May 10, 2022 — Differential Diagnoses · Acne Vulgaris · Disseminate and Recurrent Infundibular Folliculitis · Eruptive Vellus Hair Cysts · Follicular Eczema. [5] - by MN Landis · Cited by 3 — Chronic inflammation leads to fibrosis, atrophy, shrinkage of the hair bulb, and alopecia.

Additional Differential Diagnoses

  • Acne Vulgaris
  • Disseminate and Recurrent Infundibular Folliculitis
  • Eruptive Vellus Hair Cysts
  • Follicular Eczema

Additional Information

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An ichthyosis that is characterized by perifollicular keratosis and inflammation that progresses to atrophy and scarring of the facial skin.
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It is not intended to be a substitute for professional medical advice, diagnosis, or treatment. Always seek the advice of your physician or other qualified healthcare provider with questions about your medical condition.