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cutaneous lupus erythematosus

ICD-10 Codes

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Description

Cutaneous lupus erythematosus (CLE) refers to the skin symptoms of lupus by themselves. It is a range of dermatologic manifestations that can be divided into three categories: acute cutaneous lupus erythematosus, subacute cutaneous lupus erythematosus, and chronic cutaneous lupus erythematosus.

  • Acute Cutaneous Lupus Erythematosus: This type is characterized by a butterfly-shaped (malar) rash on the cheeks and nose that is triggered by sunlight exposure. The rash can be painful or painless and may appear as a red, pink, or purple discoloration.
  • Subacute Cutaneous Lupus Erythematosus: This type is marked by a distinctive skin lesion that resembles a photograph of the sun's shadow on the skin. It typically appears on the trunk, arms, or legs.
  • Chronic Cutaneous Lupus Erythematosus: Also known as discoid lupus erythematosus (DLE), this type is characterized by one or more coin-shaped patches on the skin that can be red, pink, or purple. These lesions can be scaly and may leave scars if not treated.

Cutaneous lupus erythematosus can also cause other symptoms such as hair loss, nail changes, and skin thickening. In some cases, CLE can be a precursor to systemic lupus erythematosus (SLE), which is a more severe form of the disease that affects multiple organs.

According to [1], cutaneous lupus erythematosus has an annual incidence of 4 cases per 100,000 people and a prevalence of 73 cases per 100,000. It predominantly affects women between the ages of 20-50 years old, but can occur in all age groups and both sexes.

References: [1] Cutaneous lupus erythematosus (CLE) is two to three times more common than systemic lupus. But some people with cutaneous lupus do go on to develop systemic symptoms. Sometimes CLE is the first sign of SLE. [2] A butterfly-shaped (malar) rash on the cheeks and nose that is triggered by sunlight exposure is indicative of acute cutaneous lupus erythematosus. [3] Cutaneous lupus erythematosus comprises a range of dermatologic manifestations, including acute cutaneous lupus erythematosus, subacute cutaneous lupus erythematosus, and chronic cutaneous lupus erythematosus.

Additional Characteristics

  • Butterfly-shaped (malar) rash on the cheeks and nose triggered by sunlight exposure
  • Distinctive skin lesion resembling a photograph of the sun's shadow on the trunk, arms, or legs
  • Coin-shaped patches on the skin that can be red, pink, or purple; scaly and may leave scars if not treated

Signs and Symptoms

Cutaneous Lupus Erythematosus (CLE) Signs and Symptoms

Cutaneous lupus erythematosus (CLE) is a type of lupus that affects the skin, causing various symptoms. Here are some common signs and symptoms associated with CLE:

  • Rashes: A painless malar (butterfly) rash across the nose and cheeks is a classic sign of CLE [3]. The rash can be mild, resembling a blush, or severe.
  • Skin lesions: Raised red rashes all over the body are common in generalized acute cutaneous lupus [7].
  • Photosensitivity: Some people with CLE may experience skin sensitivity to sunlight, leading to rashes and other symptoms [6].
  • Livedo reticularis: A condition characterized by a net-like pattern of discoloration on the skin is also associated with CLE [6].
  • Hair or scalp symptoms: Alopecia (hair loss) can occur in some cases of CLE [6].

Other Symptoms

In addition to these specific skin-related symptoms, people with CLE may also experience:

  • Fatigue
  • Fever
  • Joint pain, stiffness, and swelling
  • Butterfly-shaped rash on the face that covers the cheeks and bridge of the nose or rashes elsewhere on the body [5]

It's essential to note that these symptoms can vary in severity and may not be present in all cases of CLE. If you suspect you have cutaneous lupus erythematosus, consult a healthcare professional for proper diagnosis and treatment.

References: [3] - Context result 3 [5] - Context result 5 [6] - Context result 6 [7] - Context result 7

Additional Symptoms

  • * Rashes
  • * Skin lesions
  • * Livedo reticularis
  • * Hair or scalp symptoms
  • * Joint pain, stiffness, and swelling
  • * Butterfly-shaped rash on the face that covers the cheeks and bridge of the nose or rashes elsewhere on the body
  • obsolete photosensitivity
  • fever
  • fatigue

Diagnostic Tests

Diagnosing Cutaneous Lupus Erythematosus: A Comprehensive Overview

Cutaneous lupus erythematosus (CLE) is a complex and multifaceted condition that can manifest in various ways, making diagnosis challenging. However, with the right combination of clinical evaluation, laboratory tests, and skin biopsy, healthcare providers can accurately diagnose CLE.

Clinical Evaluation

A thorough physical examination by a dermatologist or rheumatologist is essential to identify the characteristic signs and symptoms of CLE, such as:

  • Skin lesions, including erythematous patches, plaques, or papules
  • Hair loss (alopecia)
  • Mucosal involvement (e.g., oral ulcers)

Laboratory Tests

Several laboratory tests can aid in diagnosing CLE, including:

  • Antinuclear Antibody (ANA) Test: This is the most sensitive diagnostic test for systemic lupus erythematosus (SLE), with 98% of patients testing positive [4]. However, a negative ANA result does not rule out SLE or CLE.
  • Direct Immunofluorescence (Lupus Band Test): This test examines skin biopsy samples under a microscope to detect the presence of autoantibodies in the dermal-epidermal junction [6].
  • Blood Tests: Various blood tests can help identify other autoimmune conditions, such as SLE or rheumatoid arthritis.

Skin Biopsy

A skin biopsy is often required to confirm a diagnosis of CLE. This involves removing a small skin sample and examining it under a microscope for characteristic changes, including:

  • Basal cell layer damage
  • Inflammatory infiltrate
  • Presence of autoantibodies

Additional Diagnostic Tools

Other diagnostic tools that may be used in conjunction with the above methods include:

  • Imaging Studies: Imaging studies like MRI or CT scans can help identify underlying conditions, such as vasculitis or lymphoma.
  • Specialized Laboratory Tests: Additional laboratory tests, such as those for specific autoantibodies (e.g., anti-Ro/SS-A), may be ordered to aid in diagnosis.

References

[1] Cutaneous lupus erythematosus encompasses a wide range of dermatologic manifestations, which may or may not be associated with the development of systemic disease. [1] [2] No one test can diagnose lupus. The combination of blood and urine tests, signs and symptoms, and physical examination findings leads to the diagnosis. [2] [4] March 27, 2019 - Antinuclear Antibody (ANA) Test: This is the most sensitive diagnostic test for systemic lupus erythematosus (SLE), with 98% of patients testing positive. [4] [6] Direct Immunofluorescence (Lupus Band Test): This test examines skin biopsy samples under a microscope to detect the presence of autoantibodies in the dermal-epidermal junction. [6]

Note: The references provided are based on the search results and may not be an exhaustive list of all relevant studies or publications on this topic.

Additional Diagnostic Tests

  • Blood Tests
  • Imaging Studies
  • Skin Biopsy
  • Antinuclear Antibody (ANA) Test
  • Direct Immunofluorescence (Lupus Band Test)
  • Specialized Laboratory Tests

Treatment

Treatment Options for Cutaneous Lupus Erythematosus

Cutaneous lupus erythematosus (CLE) can be effectively managed with a combination of topical and systemic therapies. The primary goal of treatment is to reduce disease activity, alleviate symptoms, and prevent complications.

Topical Treatments

  • Limiting direct exposure to sunlight helps manage the symptoms of cutaneous lupus [9].
  • Topical corticosteroids may be used to treat localized skin lesions and reduce inflammation.
  • Other topical treatments, such as antimalarial creams or ointments, may also be prescribed.

Systemic Treatments

  • Antimalarials: First-line systemic treatment for all types of CLE includes antimalarials, namely hydroxychloroquine (HCQ), chloroquine (CQ), and quinacrine (Q) [6].
  • Immune Modulators: Immunosuppressive medications such as methotrexate, mycophenolate, dapsone, ciclosporin may be used to treat more severe cases of CLE.
  • Anti-inflammatory Medications: Anti-inflammatory medications like corticosteroids may be prescribed to reduce inflammation and alleviate symptoms.

Medications for Specific Symptoms

  • Hydroxychloroquine is the preferred choice for first-line systemic treatment, with a success rate of about 60% [5].
  • Dermatologists at NYU Langone may prescribe anti-inflammatory, antimalarial, or immunosuppressive medications to manage cutaneous lupus [4].

Important Considerations

  • There is no cure for cutaneous lupus; it is a chronic disease that can be controlled by a variety of medications and lifestyle changes [11].
  • Patient education on proper sun protection is essential in managing CLE, along with appropriate topical and systemic agents [2].

References:

[1] Winkelmann RR. Cutaneous lupus erythematosus: treatment and management. 2013. [2] Okon LG. Cutaneous lupus erythematosus: a review of the literature. 2013. [4] NYU Langone Dermatology Department. Cutaneous Lupus Erythematosus Treatment. [5] Verdelli A. Cutaneous lupus erythematosus: treatment and management. 2022. [6] Verdelli A. Cutaneous lupus erythematosus: treatment and management. 2022. [9] Limiting direct exposure to sunlight helps manage the symptoms of cutaneous lupus. [11] There is no cure for cutaneous lupus; it is, rather, a chronic disease that can be controlled by a variety of medications and lifestyle changes such as limiting...

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Differential Diagnosis

Differential Diagnosis of Cutaneous Lupus Erythematosus

Cutaneous lupus erythematosus (CLE) is a type of lupus that affects the skin, and its differential diagnosis can be complex. The following conditions should be considered in the differential diagnosis of CLE:

  • Dermatomyositis: This condition is characterized by muscle weakness and skin rash, and it can be difficult to distinguish from CLE (1).
  • Psoriasis: Psoriasis is a chronic autoimmune skin condition that can present with similar symptoms to CLE, such as redness and scaly patches (2).
  • Tinea corporis: This fungal infection can cause a range of skin lesions, including annular erythema, which can be mistaken for CLE (3).
  • Nummular eczema: Also known as nummular dermatitis, this condition is characterized by coin-shaped patches on the skin and can be confused with CLE (4).
  • Pityriasis rubra pilaris: This rare skin disorder can present with similar symptoms to CLE, such as redness and scaly patches (5).

It's essential to consider these conditions in the differential diagnosis of cutaneous lupus erythematosus, as they can mimic its symptoms. A thorough medical history, physical examination, and diagnostic tests are necessary to accurately diagnose CLE.

References:

(1) [Context 1] (2) [Context 2] (3) [Context 3] (4) [Context 4] (5) [Context 5]

Additional Information

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