Lupus pernio – might be a reason for your uneven makeup

One among the most common forms of cutaneous sarcoidosis is Lupus pernio. Sarcoidosis is histologically characterized by a special type of inflammation called granuloma. It is a multisystem disorder.

This condition appears as plaques, purple or bluish-red nodules over the ears, nose, cheeks, forehead and lips. These nodules are chronic lesion of skin that is raised and hardened.

Lupus is originated from a Latin name for ‘wolf’ and pernio for ‘chilblain’. This name is a misnomer as this disease doesn’t have any features related to pernio or lupus. It was named so because it was thought to be in relation to lupus vulgaris a cutaneous tuberculosis.

This disease has alternate names like lupus pernio type, plaque or nodular sarcoidosis and sarcoidosis.

Erythema nodosum and Lupus pernio are cutaneous form of sarcoidosis. However, lupus pernio is suggested as a disease caused due to associated dilated cardiomyopathy, in particular with ventricular tachycardia or intraventricular conduction delay and heart block.

Victims of lupus pernio

Few populations are at higher risk to develop this disease. The annual occurrence differs between 1 to 64 out of 100,000.

• Age of onset – 45 to 65 years
• Twice common in women than men
• Most common and severe in African ethnic groups compared to Caucasians.
• There are Human Leukocytes Antigens or genetic associations.

The root cause of these bluish-red nodules

In spite of a thorough investigation, the exact cause for forms of cutaneous sarcoidosis and lupus pernio is not known. Sarcoidosis is identified as a chronic CMI (Cell-mediated Immunity) response to an unknown antigen. This makes the CD4 T-lymphocytes and activated macrophages to release cytokines that trigger the granulomas formation.

While some cases of cutaneous sarcoidosis might be due to an uncommon host reaction to one or more infective agents. Few infective agents are histoplasmosis, Mycobacterium paratuberculosis and other fungi.

It is not infectious. The reason it affects areas where the skin is cool is unknown.

Clinical features of this plague

Cosmetic damage is a frequent complaint. It rarely causes pain or itch and is usually not symptomatic.

These indolent reddish-purple lesions occur on cheeks, lips, digits, nose and forehead. They may ulcerate and then later heal with telangiectatic scars.

The Lesions on the skin may extend to the nasal septum and lead to crusting and nosebleed. Nasal rim lesions are associated with granulomatous inflammation of the upper respiratory tract.

Lupus pernio is  diagnosed by

It is recognized by the mentioned clinical appearance and history. But the diagnosis is delayed because of difficulty in establishing lupus pernio.

By examining the edge of skin lesion with glass slide pressure or diascopy. It shows appearance like apple-jelly typically explaining granulomatous inflammation.

Biopsy of the lymph nodes, lungs or skin reveals sarcoidal non-caseating granulomas and confirms the diagnosis.

Bluish-red nodules are 50% associated with sarcoidosis in upper respiratory and 75%  in the lungs. So, the following tests must be performed for monitoring and diagnosing

• Chest X-ray
• Lung function tests
• Blood tests – that shows hiked alkaline phosphatase, reduced lymphocyte count  angiotensin-converting enzyme (ACE), calcium and polyclonal (gamma globulin)
• Electrocardiogram (ECG) or echocardiogram

Alternate diagnosis can involve various types of cutaneous lupus erythematosus.

How can lupus pernio be treated?

The ultimate focus in the treatment of these bluish-red lesions is to prevent scarring on patient’s face and improve their appearance. The type of treatment depends on the severity of systemic symptoms and has differing success.

Options that include are:

• Intralesional steroid injections
• Systemic therapies that include hydroxychloroquine, methotrexate, biologic agents (adalimumab, infliximab)
• Topical corticosteroids
• Laser treatment – carbon dioxide lasers and pulsed dye)

Conclusion:

Since this is a condition that doesn’t have symptoms or a known cause, keep your facial skin hydrating and healthy. The only concern is Cosmetic disfigurement that brings these bluish-red nodes to light.