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punctate palmoplantar keratoderma
ICD-10 Codes
Description
Punctate Palmoplantar Keratoderma (PPK) is a rare skin condition characterized by the presence of small, hyperkeratotic growths on the palms and soles.
Typical Characteristics:
- Multiple Small Bumps: PPK typically presents with multiple small bumps or papules on the palms and soles.
- Hyperkeratosis: The skin lesions are caused by excessive epidermal thickening, leading to hyperkeratosis (thickening of the outer layer of the skin).
- Pressure Areas Affected: Focal keratodermas mainly affect pressure areas, such as the palms and soles.
Types of PPK:
- Punctate-type Keratoderma: This type results in tiny bumps on the palms and soles.
- Buschke-Fischer-Brauer Syndrome (PPKP1): A very rare hereditary skin disease characterized by punctate palmoplantar keratoderma.
Age of Onset:
- Adolescence: Punctate Palmoplantar Keratoderma often appears in adolescence, with epidermal thickening of the palms and soles characterized as hyperkeratotic growths.
References:
- [1] A Knowles (2023) - Palmoplantar keratodermas are a family of diseases distinguished by hyperkeratosis of the palms and soles.
- [2-4, 7, 9] Various sources describe PPK as a heterogeneous group of conditions characterized by small hyperkeratotic growths on the palms and soles.
Note: The information provided is based on the search results and citations within the context.
Additional Characteristics
- Small, hyperkeratotic growths
- Multiple small bumps or papules
- Excessive epidermal thickening
- Hyperkeratosis (thickening of outer skin layer)
- Pressure areas affected (palms and soles)
- Tiny bumps on palms and soles
- Epidermal thickening with hyperkeratotic growths
Signs and Symptoms
Punctate palmoplantar keratoderma is a rare skin condition characterized by tiny skin spots on the palms and soles. The typical signs and symptoms of this condition include:
- Multiple small, hyperkeratotic papules: These are hard, wart-like bumps that appear on the palms and soles
Additional Symptoms
- Multiple small, hyperkeratotic papules
Diagnostic Tests
Punctate palmoplantar keratoderma (PPPK) is a rare genetic disorder characterized by the presence of small, firm papules on the palms and soles. Diagnosing PPPK can be challenging, but several diagnostic tests can help confirm the condition.
Clinical Examination A thorough clinical examination is essential in diagnosing PPPK. The examination should include a detailed history of the patient's symptoms, as well as a physical examination of the skin lesions on the palms and soles [6].
Skin Biopsy A skin biopsy may be performed to rule out other conditions that can cause similar skin lesions. However, a biopsy is not always necessary for diagnosing PPPK [4].
Molecular Analysis Molecular analysis, such as genetic testing, is considered the gold standard for confirming the diagnosis of PPPK. This type of testing can identify specific genetic mutations associated with the condition [5].
Other Diagnostic Tests In addition to clinical examination and molecular analysis, other diagnostic tests may be performed to rule out other conditions that can cause similar symptoms. These tests may include:
- Imaging studies: Imaging studies such as X-rays or ultrasound may be performed to rule out other conditions that can cause similar symptoms [11].
- Blood tests: Blood tests may be performed to rule out other conditions that can cause similar symptoms [10].
Treatment and Management While there is no cure for PPPK, various treatment options are available to manage the condition. These include topical treatments such as emollients, keratolytics, and topical retinoids [7].
Additional Diagnostic Tests
- Blood tests
- Skin Biopsy
- Imaging studies (X-rays or ultrasound)
- Clinical Examination
- Molecular Analysis (genetic testing)
Treatment
Punctate palmoplantar keratoderma, also known as Brauer-Buschke-Fischer syndrome, is a rare genetic disorder characterized by the formation of small, punctate lesions on the palms and soles. The treatment for this condition typically involves a combination of topical and systemic therapies.
Topical Treatments
- Keratolytic agents: Salicylic acid, propylene glycol, and water are commonly used to soften and remove the thickened skin [4].
- Topical retinoids: These have been shown to be effective in treating punctate palmoplantar keratoderma, particularly when combined with other treatments [3][7].
Systemic Treatments
- Alitretinoin: This oral medication has been reported as a new treatment option for hereditary punctate palmoplantar keratoderma [2][6].
- Retinoids: Aromatic retinoids such as etretinate and acitretin have been found to be effective in treating this condition, particularly when used in combination with other treatments [9].
Other Treatments
- Cryotherapy: This has been used to treat punctate palmoplantar keratoderma, particularly for localized lesions [5].
- Mechanical debridement: Removing the thickened skin through mechanical means can be an effective treatment option [4].
It's worth noting that the effectiveness of these treatments may vary depending on individual cases and the severity of the condition. A comprehensive treatment plan should be developed in consultation with a dermatologist or other healthcare professional.
References: [1] JW Jo, 2018 - Case of punctate palmoplantar keratoderma type I treated with combination of low-dose oral acitretin and topical salicylic acid and steroid. [2] B Raone, 2014 - Alitretinoin: A new treatment option for hereditary punctate palmoplantar keratoderma (Brauer-Buschke-Fischer syndrome) [3] B Raone, 2014 - Alitretinoin: a new treatment option for hereditary punctate palmoplantar keratoderma (Brauer-Buschke-Fischer syndrome) [4] What is the treatment of palmoplantar keratoderma? · Emollients · Keratolytic agents (eg, 6% salicylic acid, 70% propylene glycol, 30% water) · Topical retinoids ... [5] Apr 15, 2019 — Treatment includes keratolytics, topical salicylic acid, mechanical debridement, excision, and topical and systemic retinoids. Punctate ... [6] May 2, 2024 — Cyrotherapy and keratolytic topical agents (salicylic acid in the form of poultices or a liquid) are the mainstay of treatment. A 10-second ... [7] by HK Park · Cited by 13 — Alitretinoin: A new treatment option for hereditary punctate palmoplantar keratoderma (Brauer-Buschke-Fischer syndrome). J Am Acad. Dermatol ... [8] by HK Park · 2016 · Cited by 13 — It has been reported that etretinate and acitretin, which are aromatic retinoids, are effective treatment modalities for MDM. [9] by HK Park · 2016 · Cited by 13 — It has been reported that etretinate and acitretin, which are aromatic retinoids, are effective treatment modalities for MDM.
Recommended Medications
- Cryotherapy
- Topical retinoids
- Mechanical debridement
- etretinate
- Etretinate
- water
- Water
- salicylic acid
- Salicylic Acid
- propane-1,2-diol
- Propylene glycol
- acitretin
- Acitretin
- 9-cis-retinoic acid
- alitretinoin
💊 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
Punctate palmoplantar keratoderma, also known as PPK, can be challenging to diagnose due to its similarity in appearance with other skin conditions. Here are some differential diagnoses that should be considered:
- Porokeratosis palmaris et plantaris: This condition is characterized by a solitary or multiple keratinous horn on the palms and soles, which can be mistaken for PPK.
- Warts: Warts can present as small, hyperkeratotic papules on the palms and soles, making them a differential diagnosis for PPK.
- Arsenical keratosis: This condition is caused by exposure to arsenic and can result in thickening of the skin on the palms and soles, similar to PPK.
- Calluses and corns: Calluses and corns are common conditions that can cause thickening of the skin on the palms and soles, which can be mistaken for PPK.
- Palmer pits: Palmer pits are small depressions in the skin on the palmar surface, which can be confused with PPK.
- Cole disease: This is a rare genetic disorder characterized by palmoplantar keratoderma, which can be similar to PPK.
- Darier's disease: This is a rare genetic disorder that affects the skin and can cause thickening of the skin on the palms and soles, similar to PPK.
These differential diagnoses should be considered when evaluating patients with punctate palmoplantar keratoderma. A thorough medical history, physical examination, and diagnostic tests may be necessary to rule out these conditions and confirm a diagnosis of PPK [1][3][4].
References: [1] - Context 3 [2] - Context 7 [3] - Context 4 [4] - Context 5
Additional Information
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- UMLS_CUI:C1274216
- IAO_0000115
- A palmoplantar keratosis characterized by keratoses with a raindrop pattern on the palmoplantar surface, skin lesions which may involve the whole of the palmoplantar surface, or may be more restricted in their distribution.
- oboInOwl#hasRelatedSynonym
- punctate keratosis palmoplantaris
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