Lupus Pernio Symptoms, Causes, and Treatments

Lupus pernio is a chronic skin condition characterized by purplish, swollen lesions that primarily affect the nose, cheeks, lips, and ears. It is a cutaneous manifestation of sarcoidosis, a systemic inflammatory disease that impacts multiple organs, particularly the lungs and lymph glands.

While often painless, these lesions can be distressing due to their appearance and may indicate underlying systemic involvement, requiring proper evaluation and management.

What are the Types of Lupus Pernio?

Lupus pernio is a chronic form of cutaneous sarcoidosis that mainly affects the face. While there are no distinct types, it can vary in severity:

Mild Lupus Pernio: Small, isolated lesions, usually on the nose or cheeks, with little skin thickening.
Moderate Lupus Pernio: Larger lesions affecting multiple areas, such as the lips and ears, sometimes causing discomfort.
Severe Lupus Pernio: Extensive, disfiguring lesions with deep skin thickening and scarring, often linked to lung involvement in sarcoidosis.

What are the Symptoms of Lupus Pernio?

Cutaneous Manifestations

Lupus pernio primarily manifests as purplish or violaceous nodules and plaques, often with a shiny surface. These lesions are most commonly found on the nose, cheeks, lips, and ears, but they can also occur on other parts of the body.

The nodules are usually non-tender, but they can sometimes cause discomfort due to their location and potential for ulceration.

Systemic Involvement

While lupus pernio itself is a cutaneous condition, its presence often suggests systemic sarcoidosis. Patients with lupus pernio may exhibit symptoms related to the involvement of other organs, such as:

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Respiratory symptoms: Cough, dyspnea, or chest pain due to lung involvement.
Ocular symptoms: Uveitis or other eye conditions.
Neurological symptoms: Neuropathy or central nervous system involvement.
Cardiac symptoms: Arrhythmias or heart failure.

What are the Causes of Lupus Pernio?

The exact cause of lupus pernio is unknown, but it is closely linked to sarcoidosis, an inflammatory disease that causes granulomas (clusters of immune cells) to form in various organs, including the skin.

Immune System Dysfunction: An overactive immune response leads to chronic inflammation and granuloma formation in the skin.
Genetic Predisposition: A family history of sarcoidosis may increase the risk.
Environmental Factors: Exposure to dust, mold, or chemicals may trigger an abnormal immune reaction.
Chronic Sarcoidosis: Lupus pernio is often seen in long-standing cases of sarcoidosis, particularly with lung involvement.

What are the Risk Factors for Lupus Pernio?

Several factors may increase the likelihood of developing lupus pernio, a chronic form of cutaneous sarcoidosis.

Sarcoidosis Diagnosis: Lupus pernio is strongly associated with systemic sarcoidosis, especially with lung involvement.
Age and Gender: It is more common in adults between 30-50 years old and tends to affect women more than men.
Ethnicity: Higher prevalence among African American, Caribbean, and Scandinavian populations.
Genetic Factors: A family history of sarcoidosis may increase susceptibility.
Environmental Triggers: Exposure to certain dust, mould, or chemicals may contribute to disease onset.
Chronic Inflammation: Individuals with persistent immune system activation are at higher risk.

Understanding these risk factors can help with early detection and management of lupus pernio.

What is the Diagnosis Process for Lupus Pernio?

Diagnosing lupus pernio involves a combination of clinical evaluation, imaging, and laboratory tests to confirm sarcoidosis and rule out other conditions.

Physical Examination: A doctor assesses skin lesions, typically found on the nose, cheeks, ears, and lips.
Skin Biopsy: A sample of the affected skin is analyzed under a microscope to confirm granulomas, a hallmark of sarcoidosis.
Chest X-ray or CT Scan: Since lupus pernio is associated with pulmonary sarcoidosis, imaging helps detect lung involvement.
Blood Tests: Elevated ACE (angiotensin-converting enzyme) levels and inflammatory markers may support the diagnosis.
Pulmonary Function Tests: If lung involvement is suspected, breathing tests assess respiratory function.

Early diagnosis helps in managing the condition effectively and preventing complications.

What are the Treatments of Lupus Pernio?

Medical Treatment

The management of lupus pernio involves treating both the cutaneous lesions and any systemic involvement. Corticosteroids are the mainstay of treatment for reducing inflammation and controlling symptoms.

Topical corticosteroids: Applied directly to the lesions to reduce swelling and Skin discoloration.
Systemic corticosteroids: Used for more severe cases or when there is significant systemic involvement.

Other medications that may be used include:

Antimalarials: Such as hydroxychloroquine, which can be effective for skin lesions.
Immunosuppressants: Methotrexate or azathioprine may be prescribed for patients who do not respond to corticosteroids.

Surgical and Procedural Interventions

In some cases, particularly when lesions are disfiguring, surgical interventions may be considered. Laser therapy or cryotherapy can be used to improve the appearance of the skin.

Lifestyle and Supportive Care

Patients with lupus pernio should be encouraged to follow a healthy lifestyle, including:

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Avoidance of triggers: Protecting skin from extreme temperatures and UV exposure.
Regular follow-ups: Monitoring for any changes in systemic symptoms or lesion appearance.
Psychological support: Counseling or support groups may be beneficial due to the cosmetic impact of the condition.

How can Lupus Pernio be Prevented?

Lupus pernio, a chronic skin condition linked to sarcoidosis, cannot always be prevented, but managing sarcoidosis through proper lupus pernio treatment

can help reduce the risk. Avoiding cold exposure, protecting the skin with moisturizers, and steering clear of irritants may prevent flare-ups.

Quitting smoking can also reduce inflammation, while regular medical check-ups allow for early detection and management to prevent severe cases.

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Frequently Asked Questions

Lupus pernio is a chronic skin manifestation of sarcoidosis, presenting as violaceous, indurated plaques on the nose, cheeks, ears, or lips. It is associated with systemic involvement, especially in the lungs and upper respiratory tract, and often indicates more severe disease.

Histologically, lupus pernio shows non-caseating granulomas composed of epithelioid histiocytes and multinucleated giant cells within the dermis. Chronic inflammation and fibrosis are common. Special stains help rule out infections like tuberculosis and fungal diseases.

Treatment includes systemic corticosteroids, methotrexate, or TNF inhibitors for severe cases. Topical or intralesional steroids can help mild lesions. Laser therapy or surgical intervention may be considered for refractory cases to reduce scarring and disfigurement.

Lupus pernio presents as chronic, indurated, purple plaques, primarily affecting the nose and cheeks, while rosacea causes transient erythema, telangiectasia, and pustules. Rosacea lacks granulomas histologically and is not linked to systemic sarcoidosis.

Both are cutaneous manifestations of sarcoidosis, but erythema nodosum presents as tender, red nodules on the lower legs and indicates an acute, self-limiting course. Lupus pernio signifies chronic disease and is linked to more severe systemic involvement.