4 free views remaining today
peeling skin syndrome 3
Description
Peeling Skin Syndrome-3 (PSS3) Description
Peeling skin syndrome-3 (PSS3) is a rare condition characterized by the lifelong and continuous shedding of the stratum corneum, which is the outermost layer of the epidermis. This shedding occurs in superficial sheets, without any symptoms or discomfort [5].
The main feature of PSS3 is the asymptomatic peeling of the skin, which can be localized or generalized. The condition is considered a form of genodermatosis, a group of rare genetic disorders affecting the skin.
PSS3 is often associated with other conditions that affect the skin and its layers. It's essential to note that PSS3 is a rare disorder, and more research is needed to fully understand its characteristics and implications [9].
Key Features:
- Lifelong and continuous shedding of the stratum corneum
- Asymptomatic peeling of the skin
- Can be localized or generalized
- Considered a form of genodermatosis
References:
[5] - Description of Peeling Skin Syndrome-3 (PSS3) as characterized by asymptomatic lifelong and continuous shedding of the stratum corneum of the epidermis. [9] - Peeling Skin Syndrome (PSS) is a rare genodermatoses characterized by asymptomatic, localized or generalized, continuous exfoliation of the stratum corneum; it may be associated with other conditions that affect the skin and its layers.
Additional Characteristics
- Lifelong and continuous shedding of the stratum corneum
- Asymptomatic peeling of the skin
- Can be localized or generalized
- Considered a form of genodermatosis
Signs and Symptoms
Peeling Skin Syndrome 3 Signs and Symptoms
Peeling skin syndrome 3, also known as acral peeling skin syndrome, is a rare genetic condition that affects the skin on the hands and feet. The main symptom of this condition is painless peeling of the skin, which can start soon after birth or develop later in life [3].
The signs and symptoms of peeling skin syndrome 3 may include:
- Painless peeling of the skin on the hands and feet
- Superficial peeling of the skin, which can be temporary and not associated with scarring [1]
- The underlying skin may be temporarily red and itchy, but it typically heals without scarring
It's essential to note that peeling skin syndrome 3 is a rare condition, and its symptoms may vary from person to person. If you suspect that you or your child has this condition, consult with a healthcare professional for proper diagnosis and treatment.
References: [1] - The underlying skin may be temporarily red and itchy, but it typically heals without scarring. [3] - Oct 3, 2022 — The signs and symptoms of peeling skin usually appear soon after birth, but they may also develop later in life; The main symptom is painless ...
Additional Symptoms
- Painless peeling of the skin on the hands and feet
- Superficial peeling of the skin, which can be temporary and not associated with scarring
- The underlying skin may be temporarily red and itchy, but it typically heals without scarring
Diagnostic Tests
Diagnostic Tests for Peeling Skin Syndrome
Peeling skin syndrome (PSS) can be challenging to diagnose, but several diagnostic tests can help confirm the condition. Here are some of the diagnostic tests used to diagnose PSS:
- Skin Biopsy: A tiny sample of skin is sent to a lab and examined under a microscope. This test can help confirm the diagnosis by showing the characteristic changes in the skin's outermost layer (epidermis) [10].
- Molecular Genetics Testing: This test involves analyzing genes associated with PSS, such as CAST, CDSN, CHST8, CSTA, FLG2, SERPINB8, and TGM5 [3]. The Peeling skin syndrome NGS panel consists of these seven genes.
- Inter-laboratory Proficiency Testing: This test is performed for some tests to ensure accuracy and consistency in results.
It's worth noting that the diagnosis of PSS is mainly based on personal history and clinical presentation, including superficial skin peeling and blisters [9]. A combination of these diagnostic tests can help confirm the diagnosis and rule out other conditions.
Additional Diagnostic Tests
- Skin Biopsy
- Molecular Genetics Testing
- Inter-laboratory Proficiency Testing
Treatment
Treatment Options for Peeling Skin Syndrome
Peeling skin syndrome (PSS) can be challenging to treat, and there is currently no cure available. However, various treatment modalities have been explored to manage the symptoms.
- Topical treatments: Ointments such as petroleum jelly may provide relief from the symptoms of PSS [7]. Topical corticosteroids and keratolytic agents can also be used to help soften and remove dead skin cells [8].
- Systemic medications: Methotrexate, isotretinoin, and other systemic medications have been tried in some cases, but their effectiveness is not well established [8].
- Phototherapy: Exposure to specific wavelengths of light may help alleviate symptoms in some individuals.
- Antibiotic treatment: In cases where PSS is caused by an underlying infection, antibiotic medication may be prescribed to treat the infection.
It's essential to note that these treatments are often used on a case-by-case basis and may not be effective for everyone. Additionally, there is currently no approved treatment or cure for peeling skin syndrome [9].
References: [3] Stevens-Johnson syndrome and toxic epidermal necrolysis are commonly caused by medications or infections. Typical symptoms for both disorders include peeling ... [7] Sep 9, 2020 — Although there is currently no cure for APSS, people may find relief from the symptoms with the use of ointments, such as petroleum jelly. This ... [8] by F Rajab · 2023 — Various treatment modalities, including methotrexate, isotretinoin, topical keratolytic agents, topical corticosteroids, and phototherapy, have ... [9] Aug 6, 2024 — Peeling Skin Syndrome is a rare autosomal disease with no approved treatment or cure. Initial clinical site and pediatric patient identified ...
Recommended Medications
- antibiotics
- petroleum jelly
- topical corticosteroids
- keratolytic agents
- isotretinoin
- Isotretinoin
- methotrexate
- Methotrexate
💊 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
The differential diagnosis for peeling skin syndrome (PSS) includes several conditions that can present with similar symptoms. According to the provided context, some of these differential diagnoses are:
- Localised epidermolysis bullosa simplex [3]
- Oudtshoorn disease (syn. keratolytic winter erythema)
- Acral peeling skin syndrome (APSS) is a subtype of PSS
- PLACK syndrome
- Staphylococcal scalded skin syndrome
It's worth noting that the differential diagnosis for PSS can vary depending on the specific subtype and presentation of the disease. A thorough medical evaluation and diagnostic workup are necessary to accurately diagnose PSS and rule out other conditions.
References: [3] Oudtshoorn disease (syn. keratolytic winter erythema) is mentioned as a differential diagnosis for PSS in [3]. [10] Localised epidermolysis bullosa simplex, PLACK syndrome, staphylococcal scalded skin syndrome are also mentioned as differential diagnoses in [10].
Additional Differential Diagnoses
- Staphylococcal scalded skin syndrome
- Oudtshoorn disease (syn. keratolytic winter erythema)
- Acral peeling skin syndrome (APSS)
- epidermolysis bullosa simplex
- PLACK syndrome
Additional Information
- owl#annotatedSource
- t349788
- oboInOwl#hasOBONamespace
- disease_ontology
- oboInOwl#id
- DOID:0070522
- core#notation
- DOID:0070522
- oboInOwl#hasDbXref
- MIM:616265
- core#exactMatch
- MIM:616265
- core#broadMatch
- ORDO:263548
- IAO_0000115
- A peeling skin syndrome that has_material_basis_in homozygous mutation in the CHST8 gene on chromosome 19q13.11.
- rdf-schema#label
- peeling skin syndrome 3
- oboInOwl#hasBroadSynonym
- peeling skin syndrome type A
- oboInOwl#hasExactSynonym
- PSS3
- rdf-schema#subClassOf
- http://purl.obolibrary.org/obo/DOID_0060283
- 22-rdf-syntax-ns#type
- http://www.w3.org/2002/07/owl#Class
Medical Disclaimer: The information provided on this website is for general informational and educational purposes only.
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.