Chilblain lupus

What are Chilblains?

Chilblains, also known as perniosis or primary pernio, are small, painful red/purple bumps on the skin that develop after exposure to cold, damp conditions [3]. They usually appear on fingers, toes, ears, nose, and other areas of the body exposed to cold temperatures.

Causes and Risk Factors

Chilblains occur when tiny blood vessels in the skin become inflamed due to prolonged exposure to cold air. This inflammation can lead to redness, itching, pain, and sometimes blisters [13]. The condition is more common in young to middle-aged adults, with a female predominance [1].

Symptoms

The symptoms of chilblains typically include:

• Small, painful red/purple bumps on the skin
• Itching or burning sensation
• Redness and swelling of the affected area
• Painful blisters (in some cases)

Prevention and Treatment

To prevent chilblains, it's essential to limit exposure to cold temperatures, dress warmly, and cover exposed skin [11]. If you do get chilblains, keeping the affected skin warm and dry can help ease symptoms. In most cases, chilblains clear up on their own within 2-3 weeks, especially if the weather gets warmer.

Complications

While rare, repeated episodes of chilblains may lead to complications such as:

• Long-term health problems
• Skin ulcers or sores

However, these complications are relatively uncommon and usually occur in people with underlying medical conditions [4].

References:

[1] - Chilblains can affect all age groups, but is most common in young to middle-aged adults, with a female predominance.

[3] - Chilblains are small, painful red/purple bumps on the skin that develop after exposure to cold, damp conditions. Usually found on the fingers, toes, ears, nose ...

[11] - Chilblains can be prevented by limiting your time in the cold, dressing warmly and covering exposed skin.

[13] - Chilblains are itchy, red swollen tender areas of skin that develop on the exposed skin of fingers, toes or rims of ears after exposure to cold at temperatures ...

Note: The above information is based on the provided context and search results.

Chilblain Lupus Signs and Symptoms

Chilblain lupus, also known as chilblain LE or cold-induced lupus, is a rare form of cutaneous lupus erythematosus. The condition is characterized by the development of painful red or purple sores and discolored patches of skin after exposure to cold temperatures.

Common Symptoms:

• Painful Red or Purple Sores: Chilblain lupus causes small, painful sores (chilblains) that form or worsen after exposure to cold. These sores can be red or purple in color and may appear on the fingers, toes, ears, nose, and other areas of the body.
• Discolored Patches of Skin: People with chilblain lupus often experience discolored patches of skin that are red, purple, or violaceous in color. These patches can be itchy or painful to the touch.
• Swollen Patches of Skin: Chilblains can cause swollen patches of skin that appear shiny due to the swelling.

Other Symptoms:

• Itching and Pain: The sores and patches caused by chilblain lupus can be itchy and painful, especially when exposed to cold temperatures.
• Swollen Fingers and Toes: In some cases, people with chilblain lupus may experience swollen fingers and toes due to the inflammation caused by the condition.

Duration of Symptoms:

• Temporary or Chronic: Chilblains can be temporary, lasting only a few weeks, or chronic, persisting for months or even years if left untreated.

These symptoms are often cited in medical literature as [1] (People with chilblain lupus get painful red or purple sores and discolored patches of skin. These small sores (chilblains) form or worsen after exposure to cold ...), [2] (Chilblain LE begins as red or dusky purple patches, papules, and plaques that are initiated or exacerbated by exposure to cold and moisture in a cool climate.), [3] (A rare, chronic form of cutaneous SLE characterised by mildly tender, pruritic, erythematous lesions that typically involves acral surfaces.), [4] (A rare, chronic cutaneous lupus erythematosus disease characterized by red or violaceous, initially pruritic (evolving to painful) papules and plaques.), [5] (The patient usually comes with symptoms of purple plaques or nodules and oedematous skin, mainly around the acral regions of the body. Histologic features are ...), [6] (Chilblains cause swollen patches of skin that appear red or, on some occasions, blue. Due to the swelling, they may look shiny.), [7] (Chilblains are itchy, swollen and painful lumps or patches of skin on your fingers and toes, which form due to cold.), [8] (People suffering from chilblain lupus experience painful red or purple lesions and discolored regions of skin. Cold temperatures cause these little sores (...).

Diagnostic Tests for Chilblain Lupus

Chilblain lupus, also known as chilblain lupus erythematosus, is a rare skin condition that can be challenging to diagnose. However, several diagnostic tests can help confirm the presence of this condition.

• Clinical Presentation and Histopathological Examination: A diagnosis of chilblain lupus may be made based on clinical presentation and histopathological examination or direct immunofluorescence study [2].
• Laboratory Tests: The following laboratory tests may be needed to diagnose chilblain lupus:
  • Complete Blood Count (CBC) with differential
  • Liver function tests
  • Rheumatoid factor test
  • Cryoglobulin test
  • Direct immunofluorescence studies [6]
• Skin Biopsy: A skin biopsy may be performed to confirm the diagnosis of chilblain lupus. During a skin biopsy, a small sample of skin tissue is taken from the affected area and examined under a microscope [8].
• Immunologic Abnormalities: Most patients with chilblain LE have immunologic abnormalities, including polyclonal hypergammaglobulinemia, elevated rheumatoid factor, and/or cryoglobulins [7].

It's essential to note that diagnosing chilblain lupus can be a complex process, and a combination of clinical presentation, laboratory tests, and histopathological examination may be necessary to confirm the diagnosis.

References: [1] Not applicable [2] S Patel · 2013 · Cited by 29 — Chilblain lupus erythematosus may be diagnosed based on clinical presentation and histopathological examination or direct immunofluorescence study. [3] Not applicable [4] MSM Ali · 2023 — Diagnosing a patient requires two major and at least one minor criterion. Patients with chilblain lupus erythematosus may also display hypergammaglobulinaemia, ... [5] Not applicable [6] Histological examination and direct immunofluorescence studies reveal nonspecific inflammatory lupus erythematosus changes while results of cryoglobulin and ... [7] Jan 12, 2022 — Most patients with chilblain LE have immunologic abnormalities, including polyclonal hypergammaglobulinemia, elevated rheumatoid factor, and/or ... [8] Dermatologists can treat chilblain lupus. Dermatologists typically employ a test known as a skin biopsy. During a skin biopsy, the dermatologist takes a small ...

Treatment Options for Chilblain Lupus Erythematosus (CHLE)

Chilblain lupus erythematosus (CHLE) is a rare and complex autoimmune condition that requires careful management. The primary goal of treatment is to alleviate symptoms, prevent disease progression, and improve quality of life.

Medications Used in CHLE Treatment

Several medications have been found to be effective in treating CHLE:

• Corticosteroids: Topical corticosteroids are often used as a first-line treatment for mild and localized symptoms. Systemic corticosteroids may also be prescribed for more severe cases.
• Immunomodulators: These medications, such as mycophenolate, can help reduce inflammation and modulate the immune system's response.
• Calcium-channel blockers: These medications have been shown to improve cutaneous lesions in some patients with CHLE.
• Anifrolumab: This medication has been used successfully in treating two patients with recalcitrant CHLE, as described in a recent study [1][2].
• Baricitinib: Another study found that baricitinib, 4 mg once daily for 3 months, improved cutaneous lesions in patients with CHLE [3].

Other Treatment Options

In addition to medications, other treatment options may be considered on a case-by-case basis:

• Topical steroids
• Systemic calcium channel blockers
• Systemic steroids
• Mycophenolate

It's essential to note that each patient with CHLE is unique, and the most effective treatment plan will depend on individual factors. A healthcare professional should be consulted for personalized guidance.

References:

[1] MJ Woodbury (2024) - Successful treatment of 2 patients with recalcitrant CHLE using anifrolumab [Context #2]

[2] MJ Woodbury (2024) - Drug Administration-approved for adult patients with moderate-to-severe SLE; however, emerging data suggest it may be a viable option for CLE as well. [Context #7]

[3] W Damsky (2019) - Improved cutaneous lesions after treatment with baricitinib in patients with CHLE [Context #4]

Differential Diagnosis of Chilblain Lupus Erythematosus

Chilblain lupus erythematosus (CLE) is a rare form of chronic cutaneous lupus erythematosus, characterized by purple plaques/nodules and oedematous skin mainly around the acral regions of the body, which are most exposed to the cold. The differential diagnosis for CLE includes several other skin conditions that can be confused with chilblain LE.

Other Skin Conditions to Consider:

• Perniosis (ordinary chilblains): a condition caused by exposure to cold temperatures, leading to redness and swelling of the skin.
• Acrocyanosis: a condition characterized by discoloration and coldness of the hands and feet.
• Raynaud phenomenon: a condition that affects blood flow to the fingers and toes, causing them to turn white or blue in response to cold temperatures.
• Lupus pernio: a cutaneous manifestation of sarcoidosis, which can present with similar symptoms to CLE.
• Livedo reticularis: a condition characterized by a net-like discoloration of the skin, often on the legs.
• Dermatomyositis: an inflammatory disease that affects the muscles and skin, leading to symptoms such as skin rashes and muscle weakness.
• Cold panniculitis: a condition caused by cold temperatures, leading to inflammation of the fat layer under the skin.
• Cold urticaria: a condition characterized by hives or itchy skin in response to cold temperatures.

Laboratory Tests:

Several laboratory tests can be performed to help diagnose CLE and rule out other conditions. These include:

• Blood tests to check for immunologic abnormalities, such as polyclonal hypergammaglobulinemia, elevated rheumatoid factor, and/or cryoglobulins.
• Histopathological examination of skin biopsies to show interface dermatitis with keratinocytic cell death.

Key Points:

• Chilblain lupus erythematosus is a rare form of chronic cutaneous lupus erythematosus.
• The differential diagnosis for CLE includes several other skin conditions that can be confused with chilblain LE.
• Laboratory tests, such as blood tests and histopathological examination, can help diagnose CLE and rule out other conditions.

References:

• [1] Arch Dermatol. 1996;132:459–462. doi: 10.1001/archderm.1996.03890280123017.
• [2] Perniosis (ordinary chilblains): a condition caused by exposure to cold temperatures, leading to redness and swelling of the skin.
• [3] Raynaud phenomenon: a condition that affects blood flow to the fingers and toes, causing them to turn white or blue in response to cold temperatures.