Parry–Romberg syndrome

Parry Romberg Syndrome 1

Parry–Romberg syndrome (PRS), also known as progressive hemifacial atrophy, is a rare disorder characterized by the progressive deterioration (atrophy) of the skin and soft tissues of half of the face. This condition typically affects the left side of the face, but it can also affect the right side or, in rare cases, both sides. The syndrome is named after Caleb Hillier Parry and Moritz Heinrich Romberg, who first described the condition in the 19th century.

Signs and Symptoms

The primary feature of Parry–Romberg syndrome is the slow, progressive atrophy of the skin and soft tissues on one side of the face. This atrophy can affect the muscles, fat, and bones, leading to a sunken appearance. Other symptoms may include:

• Facial pain or headaches
• Trigeminal neuralgia
• Seizures
• Enophthalmos (sunken eye)
• Alopecia (hair loss) on the affected side
• Hyperpigmentation or hypopigmentation of the skin

Causes

The exact cause of Parry–Romberg syndrome is unknown. Several theories have been proposed, including:

• Autoimmune disease
• Genetic factors
• Trauma
• Infection

Diagnosis

Diagnosis of Parry–Romberg syndrome is primarily clinical, based on the characteristic signs and symptoms. Imaging studies such as MRI or CT scan may be used to assess the extent of tissue atrophy and to rule out other conditions. Biopsy of affected tissues is rarely necessary but can be performed to exclude other diagnoses.

Treatment

There is no cure for Parry–Romberg syndrome, and treatment is primarily symptomatic. Options may include:

• Physical therapy to maintain muscle function
• Pain management for facial pain and headaches
• Anticonvulsants for seizures
• Reconstructive surgery to address cosmetic concerns

Prognosis

The progression of Parry–Romberg syndrome varies among individuals. In some cases, the atrophy may stabilize after a few years, while in others, it may continue to progress. The condition is not life-threatening, but it can have significant psychological and social impacts due to facial disfigurement.

Related Pages

• Autoimmune disease
• Trigeminal neuralgia
• Seizure
• Reconstructive surgery