Granulosis rubra nasi

Granulosis Rubra Nasi is a rare familial disease of the nose, first described by Carl Freiherr von Rokitansky in 1860. It is characterized by hyperhidrosis of the nose, leading to the formation of small, dark red nodules and vesicles, and is often associated with a high degree of nasal hyperhidrosis.

Etiology

The exact cause of Granulosis Rubra Nasi is unknown. However, it is believed to be an autosomal dominant condition with variable penetrance. This means that an affected person has a 50% chance of passing the disorder on to each of their children. The condition is more common in males than in females.

Symptoms

The primary symptom of Granulosis Rubra Nasi is the presence of small, dark red nodules and vesicles on the nose. These are often accompanied by excessive sweating (hyperhidrosis) of the nose. Other symptoms may include a burning sensation, itching, and occasional bleeding from the nodules. The condition typically begins in childhood and may persist into adulthood.

Diagnosis

Diagnosis of Granulosis Rubra Nasi is primarily based on the clinical presentation of the symptoms. A dermatologist may perform a skin biopsy to confirm the diagnosis and rule out other conditions. Other diagnostic tests may include a sweat test to measure the amount of sweat production, and genetic testing to identify the presence of the autosomal dominant gene.

Treatment

There is currently no cure for Granulosis Rubra Nasi. Treatment is primarily aimed at managing the symptoms and may include the use of topical corticosteroids to reduce inflammation and itching. In severe cases, surgical removal of the affected skin may be considered.

Prognosis

The prognosis for individuals with Granulosis Rubra Nasi is generally good. While the condition can cause discomfort and cosmetic concerns, it does not typically affect overall health or lifespan. However, the persistent nature of the condition can have a significant impact on quality of life, and individuals may require ongoing treatment to manage symptoms.

See also

• Hyperhidrosis
• Dermatology
• Genetic disorder