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UV-sensitive syndrome
ICD-10 Codes
Related ICD-10:
Description
UV-Sensitive Syndrome: A Condition Characterized by Sensitivity to Ultraviolet Rays
UV-sensitive syndrome, also known as UVSS, is a human DNA repair-deficiency disorder that affects an individual's sensitivity to ultraviolet (UV) rays in sunlight. This condition is characterized by mild clinical manifestations and is inherited in an autosomal recessive fashion.
Key Features of UV-Sensitive Syndrome:
- Sensitivity to UV Rays: Individuals with UV-sensitive syndrome are highly sensitive to UV rays, experiencing sunburn even with minimal sun exposure [3].
- Photosensitivity: This condition is marked by increased sensitivity of the skin to light, which may result in a rash upon exposure to the sun (photodermatosis) [4].
- Cutaneous Photosensitivity: UV-sensitive syndrome is a cutaneous condition that affects the skin's ability to tolerate UV radiation, leading to photosensitivity and solar lentigines [8].
- Mild Clinical Manifestations: Unlike other disorders with severe symptoms, UV-sensitive syndrome presents with mild clinical manifestations, including cutaneous photosensitivity and slight dyspigmentation [2].
Other Relevant Information:
- UV-sensitive syndrome is a rare photodermatosis characterized by cutaneous photosensitivity and slight dyspigmentation, without an increased risk of developing skin tumors [5].
- The condition is inherited in an autosomal recessive fashion, meaning that both parents must be carriers of the mutated gene for their offspring to develop the condition [8].
References:
[1] - UV-sensitive syndrome is a condition characterized by sensitivity to ultraviolet (UV) rays in sunlight. [2] - UV-sensitive syndrome (UV(S)S) is a human DNA repair-deficiency disorder with mild clinical manifestations. [3] - Individuals with UV-sensitive syndrome are highly sensitive to UV rays, experiencing sunburn even with minimal sun exposure. [4] - An increased sensitivity of the skin to light may result in a rash upon exposure to the sun (photodermatosis). [5] - A rare photodermatosis characterized by cutaneous photosensitivity and slight dyspigmentation, without an increased risk of developing skin tumors. [8] - UV-sensitive syndrome is a cutaneous condition inherited in an autosomal recessive fashion, characterized by photosensitivity and solar lentigines.
Additional Characteristics
- Photosensitivity
- Sensitivity to UV Rays
- Cutaneous Photosensitivity
- Mild Clinical Manifestations
- Rare photodermatosis characterized by cutaneous photosensitivity and slight dyspigmentation
- Inherited in an autosomal recessive fashion
Signs and Symptoms
Common Signs and Symptoms of UV-Sensitive Syndrome
UV-sensitive syndrome, also known as photosensitivity or photodermatosis, is a condition characterized by an abnormal sensitivity of the skin to light from the sun or artificial sources of ultraviolet (UV) radiation. The symptoms can vary depending on the individual, but here are some common signs and symptoms associated with UV-sensitive syndrome:
- Increased sensitivity of the skin to light: People with UV-sensitive syndrome may experience a rash or redness upon exposure to sunlight or other sources of UV radiation [3].
- Telangiectasia: Some individuals may have small clusters of enlarged blood vessels just under the skin, usually on the cheeks and nose [2].
- Itchy rash: A common symptom is an itchy red rash that forms on the skin after being in sunlight or other sources of UV radiation [7].
- Cutaneous photosensitivity: This condition causes an abnormal reaction of the skin to light, leading to symptoms such as a rash, redness, and itching [4][5].
- Freckling: Some people with UV-sensitive syndrome may experience increased freckling on their skin [8].
It's essential for individuals with UV-sensitive syndrome to take precautions when spending time outdoors or using artificial sources of UV radiation. This can include wearing protective clothing, applying sunscreen, and seeking shade to minimize exposure to UV light.
References: [1] Not applicable (no relevant information found in the context) [2] Context #2 [3] Context #6 [4] Context #4 [5] Context #5 [7] Context #7 [8] Context #8
Additional Symptoms
- Increased sensitivity of the skin to light
- Cutaneous photosensitivity
- rash
- Freckling
- Telangiectasia
Diagnostic Tests
Diagnostic Tests for UV-Sensitive Syndrome
UV-sensitive syndrome, also known as xeroderma pigmentosum (XP), is a rare genetic disorder that makes individuals extremely sensitive to ultraviolet (UV) light from the sun or other sources. While there are no specific tests to diagnose UV-sensitive syndrome, various diagnostic tests can help confirm the suspected clinical diagnosis.
- Molecular Genetics Tests: These tests can identify mutations in the ERCC6 gene, which is associated with UV-sensitive syndrome. The following tests may be performed:
- Deletion/duplication analysis [1]
- Sequence analysis of the entire coding region [1]
- Targeted variant analysis [1]
These genetic tests can help confirm the diagnosis of UV-sensitive syndrome and rule out other conditions that may present similar symptoms.
- Clinical Diagnosis: The diagnosis of UV-sensitive syndrome is primarily made based on clinical manifestations, such as skin sensitivity to sunlight. A doctor may suspect a photosensitivity reaction when a rash appears only in areas exposed to sunlight [8].
While these diagnostic tests can help confirm the diagnosis of UV-sensitive syndrome, it's essential to note that laboratory testing is not always necessary for diagnosis.
References:
[1] Molecular Genetics Tests · Deletion/duplication analysis (1) · Sequence analysis of the entire coding region (1) · Targeted variant analysis (1)
[3] Jul 1, 2012 — UV-sensitive syndrome is a condition that is characterized by sensitivity to the ultraviolet. (UV) rays in sunlight.
[5] The diagnosis of UVSS is made clinically based on skin manifestations. Laboratory testing can be useful for confirming the suspected clinical diagnosis.
[6] Some people with UV-sensitive syndrome have small clusters of enlarged blood vessels just under the skin (telangiectasia), usually on the cheeks and nose.
[9] Some people with UV-sensitive syndrome have small clusters of enlarged blood vessels just under the skin (telangiectasia), usually on the cheeks and nose.
Additional Diagnostic Tests
- Molecular Genetics Tests
- Clinical Diagnosis
- duplication analysis [1]
- Sequence analysis of the entire coding region [1]
- Targeted variant analysis [1]
- [3] Jul 1, 2012 â UV-sensitive syndrome is a condition that is characterized by sensitivity to the ultraviolet. (UV) rays in sunlight.
- [5] The diagnosis of UVSS is made clinically based on skin manifestations. Laboratory testing can be useful for confirming the suspected clinical diagnosis.
- [6] Some people with UV-sensitive syndrome have small clusters of enlarged blood vessels just under the skin (telangiectasia), usually on the cheeks and nose.
- [9] Some people with UV-sensitive syndrome have small clusters of enlarged blood vessels just under the skin (telangiectasia), usually on the cheeks and nose.
Treatment
Treatment Options for UV-Sensitive Syndrome
UV-sensitive syndrome, also known as polymorphic light eruption (PMLE), is a condition that causes sensitivity to ultraviolet (UV) rays in sunlight. While there are no definitive cures for this condition, various treatment options can help manage its symptoms.
- Antihistamines: Antihistamines such as loratadine, fexofenadine, and cetirizine [1] can be used to alleviate itching and hives associated with PMLE.
- Corticosteroids: Corticosteroids like prednisone [2] may be prescribed to reduce inflammation and itching.
- Immunosuppressants: In severe cases, immunosuppressants such as azathioprine [3] or hydroxychloroquine [4][7] may be used to suppress the immune system's response to UV light.
Other Treatment Options
In addition to these medications, other treatment options for PMLE include:
- Topical corticosteroids: Topical corticosteroids or tacrolimus can be applied directly to affected skin areas [9].
- Phototherapy: Phototherapy, such as PUVA (psoralen and ultraviolet light) therapy, may be used in some cases [7].
Prevention is Key
While these treatment options can help manage symptoms, prevention remains the best course of action for individuals with UV-sensitive syndrome. This includes using sun protection measures, avoiding prolonged exposure to direct sunlight, and taking vitamin D supplements if necessary [8].
Recommended Medications
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Differential Diagnosis
Differential Diagnosis of UV-Sensitive Syndrome
UV-sensitive syndrome, also known as ultraviolet-sensitive syndrome, is a rare skin disorder characterized by heterogeneous phenotypic spectrum of skin freckling, telangiectasia, and acute sunburn. When diagnosing this condition, it's essential to consider other related disorders that may present similar symptoms.
Related Disorders:
- Xeroderma Pigmentosum (XP): A genetic disorder caused by defects in the nucleotide excision repair (NER) pathway, leading to increased sensitivity to ultraviolet (UV) radiation. [1]
- Cockayne Syndrome (CS): A rare genetic disorder characterized by premature aging, photosensitivity, and impaired DNA repair mechanisms. [2][3]
- Trichothiodystrophy (TTD): A rare genetic disorder affecting the NER pathway, leading to
Additional Differential Diagnoses
Additional Information
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