Lupus Pernio

What is lupus pernio?

Lupus pernio is one of the most common forms of cutaneous sarcoidosis Sarcoidosis is a multisystem disorder characterized histologically by granulomas (a specific type of inflammation)

Lupus pernio appears as bluish-red or violaceous nodules and plates on the nose, cheeks and ears.

The name is derived from the Latin name for 'wolf' (lupus) and for 'chilblains' (pernio). This is because Besnier (1889) thought it was related to lupus vulgaris (skin tuberculosis). Lupus pernio is also called nodular or license plate sarcoidosis and sarcoidosis, lupus pernio type.

Who gets lupus pernio?

Some populations are at increased risk of developing lupus pernio. the annual incidence ranges from 1 to 64 per 100,000.

• The age of onset is usually between 45 and 65 years.
• Lupus pernio is twice as common in women as in men.
• Lupus pernio is more common and more severe in African ethnic groups than in Caucasians.
• exist genetic/ /HLA associations.

What Causes Lupus Pernio?

Despite intense research, the cause of lupus pernio and other forms of skin sarcoidosis is unknown. Sarcoidosis is recognized as chronic cell-mediated immune response to an unknown antigen, in which activated macrophages and CD4 T-lymphocytes launching cytokines that trigger the formation of granulomas.

At least some cases of cutaneous sarcoidosis may be due to an unusual host reaction to one or more infectious agents, such as Mycobacterium paratuberculosis, histoplasmosis, and other fungi. Lupus pernio itself is not infectious.

It is not clear why lupus pernio affects places where the skin is cold.

What are the clinical features of lupus pernio?

Lupus pernio is usually asymptomatic, and it rarely causes itching or pain. Cosmetic disfigurement is the most common complaint.

It is presented as indolent reddish-purple plaques and nodules on the nose, cheeks, lips, ears, and digits. Lesions can ulcerate and then heal with telangiectatic scars.

Lesions on the nasal border are associated with granulomatous inflammation of the upper respiratory tract. Skin lesions can spread to the nose. pulp leading to nosebleeds and crust.

How is the diagnosis of lupus pernio made?

Lupus pernio can be recognized by its typical clinical appearance and history, but the diagnosis can be difficult to establish and delayed.

Examination of the edge of the skin. injury using transmitted light, or pressure with a glass slide, shows an apple jelly appearance typical of granulomatous inflammation.

Biopsy of the skin lymph nodes or lungs confirms the diagnosis by revealing non-caseating sarcoidal granulomas.

As lupus pernio is associated with sarcoidosis in the lungs (75% of patients) and upper respiratory tract (50%), the following tests should also be performed at the time of diagnosis and for monitoring:

• Lung function tests
• Chest x-ray
• Electrocardiogram (ECG) or echocardiogram
• Blood tests, which may show elevated angiotensin conversion enzyme (ACE), alkaline phosphatase, calcium, gamma globulin (polyclonal) and reduced lymphocyte tell

It is important to exclude tuberculosis. The Tuberculin Skin Test and the QuantiFERON® Gold Blood Test are negative for sarcoidosis.

the differential diagnosis It can include several types of cutaneous lupus erythematosus.

What is the treatment for lupus pernio?

The goal of lupus pernio treatment is to improve the patient's appearance and prevent scarring. The treatment chosen may depend on the severity of systemic symptoms

The treatment has different successes. Options include:

• Current corticosteroids
• Intralesional steroid injections
• To be treatment (pulsed dye and carbon dioxide laser)
• Systemic therapies, including systemic corticosteroids, hydroxychloroquine, methotrexate, and biologic agents (infliximab, adalimumab)

Cosmetic camouflage can be used to disguise skin lesions.

What is the outlook for lupus pernio?

Lupus pernio tends to persist for several years and rarely resolves spontaneously. Lung and heart function should be monitored.