Lupus Pernio: Causes, Symptoms, Diagnosis and Treatment

Definition:

The medical condition known as lupus pernio actually has nothing to do with lupus or pernio, since it is a type of cutaneous sarcoidosis .

It is also known as Besnier-Boeck-Schaumann disease , which is considered rare to occur.

One of the skin characteristics of Sarcoidosis is lupus pernio, which can be manifested purple or red, mainly affecting the nose, ears, cheeks, lips and sometimes the respiratory system.

What causes the Lupus Pernio

We still do not have clear knowledge about what causes or produces lupus pernio; However, the only thing that has been established is that it is an autoimmune diseasethat affects mostly people between twenty and forty years of age, predominantly in the black race, especially in women.

Some of the symptoms include:

  • Plaques purple or red.
  • They are painless; However, if the skin breaks, it can cause pain.
  • They tend not to ulcerate.
  • Sometimes granulomatous nodules are present in the nose.
  • Granulomatous infiltrations in the bone tissue.
  • Fever.
  • Weightloss.
  • Cough sometimes.

The differential diagnosis includes:

Lymphoma cutis, leishmaniasis, basal cell carcinoma, cutaneous lupus and wegener’s granulomatosis, among others.

How is it diagnosed

Carrying out an initial evaluation through a good clinical history. Conduct thorough examinations that include:

  • Tuberculin test.
  • Pulmonary function test.
  • X-rays of the chest (usually always comes out abnormal).
  • Liver and kidney function tests.
  • Ophthalmological examination.
  • An electrocardiogram
  • Biopsy. In order to have a definitive diagnosis.

Treatment for Lupus Pernio

Be supplied corticosteroid orally as prednisolone (in patients with major injury healing)

Supply of cytotoxic drugs such as methotrexate, as it seems to be the most appropriate. Allopurinol has also proven to be effective. In rare cases they require plastic surgery because they can be disfiguring in some patients.