Lupus Pernio – A skin disfiguring condition that targets women

Sarcoidosis is a common health disorder of unknown origins that mainly affects the lungs and other organs like skin, eyes, neck, and heart. It is characterized by the growth of small groups of inflammatory cells in the affected part of your body. The disease manifests itself in various forms, one of them being lupus pernio.

On average, around 25 percent of people who suffer from sarcoidosis develop lupus pernio on the skin of various organs. The condition is observed more often in females than in males.

Lupus Pernio – What Is It?

Lupus pernio affects orally. It refers to the hardened, bluish-red or violet eruptions that commonly occur on the surface of cheeks, chin, nose, and forehead. It was initially thought to be related to lupus vulgaris, another disorder characterised by skin lesions which is a type of skin tuberculosis.

It is most commonly observed among people in the age group of 45-65 years. Though its exact cause remains unknown, it has been shown to be associated with the affected person’s genetics.

Lupus Pernio Pathology Outlines

A microscope, Credits: Pixabay

The most prominent histopathology feature of lupus pernio is the presence of epithelioid cells with various large cells called Langerhans that is commonly found in many diseases. It is generally found in people of Africa and does not abate with usual therapies. Its effect varies from person to person and lasts up to many years.

Features of lupus pernio

Apart from affecting the skin, there are no other symptoms of this disorder. It exhibits the following features:

Stubborn reddish-purple plaques appearing on nose, cheeks, lips, and ears.
Its facial lesion is particular to sarcoidosis.
The lesions may turn into ulcers or sores on the skin. They can leave scars
The lesions may spread up to the area between the two nostrils.
The lesions begin to appear slowly but progress rapidly. They may permeate to the cartilage of the area affected causing skin disfigurement.

Differential diagnosis of lupus pernio

Lupus pernio can be confused with other forms of malignant and benign tumours like lymphoma. Its differential diagnosis also includes skin infections like leprosy and skin tuberculosis, particularly, lupus vulgaris.

Diagnosis of Lupus Pernio

Caption: Doctor with patient, Credits: Pixabay

It is diagnosed on the basis of the clinical features described above. It is associated with sarcoidosis which affects the respiratory tract. A physical examination of the patient is done after which the lesion is examined. A skin biopsy is needed to fully confirm the diagnosis of lupus pernio.

Other important tests for the diagnosis include:

Chest x-ray
Pulmonary (lungs) function tests
Other radiological tests

Treatment of Lupus Pernio

Caption: Cosmetic camouflage, Credits: Pixabay

Lupus pernio needs to be treated immediately as it progresses very fast and can cause considerable skin disfigurement. It can lead to negative mental, social, and psychological consequences. The treatment is done according to the severity of the disease.

The typical line of treatment followed include:

Topical treatments like corticosteroid therapy
Intralesional steroid injections if the affected skin area is small.
Laser treatment
Antimalarial drugs like hydroxychloroquine
Skin reconstructive surgery is conducted for lesions that severely disfigure the skin.

Additionally, patients can take the help of cosmetic camouflage (creams, liquids, concealers) to hide the lesions on the skin surface.

There is no known origin as well as treatment of lupus pernio. Since it spreads very fast, it’s advised that patients consult the doctor soon. As it’s a form of sarcoidosis, the respiratory health of the patients is also at risk. They should regularly go for lung and cardiovascular health checkups.