Morillo–Cucci–Passarge syndrome

Morillo-Cucci-Passarge Syndrome is a rare genetic disorder characterized by a combination of clinical features, including eyelid cysts, palmoplantar keratoderma, and hypodontia (missing teeth). The syndrome is inherited in an autosomal recessive manner, meaning that an individual must inherit two copies of the mutated gene, one from each parent, to be affected by the condition.

Symptoms and Diagnosis

The primary symptoms of Morillo-Cucci-Passarge Syndrome include:

• Eyelid Cysts: Small, benign growths that typically appear on the eyelids.
• Palmoplantar Keratoderma: A condition characterized by the thickening of the skin on the palms of the hands and the soles of the feet.
• Hypodontia: The developmental absence of one or more teeth, which can affect both primary (baby) and permanent teeth.

Diagnosis of Morillo-Cucci-Passarge Syndrome is based on clinical examination and the presence of the characteristic symptoms. Genetic testing may be used to confirm the diagnosis by identifying mutations in the responsible gene.

Genetics

The genetic basis of Morillo-Cucci-Passarge Syndrome is not fully understood, and the specific gene(s) associated with the condition have not been identified. However, its autosomal recessive inheritance pattern suggests that mutations in both copies of a gene are required for an individual to manifest the syndrome.

Management and Treatment

There is no cure for Morillo-Cucci-Passarge Syndrome, and treatment is symptomatic and supportive. Management strategies may include:

• Surgical removal of eyelid cysts if they cause discomfort or impair vision.
• Skin care regimens to manage palmoplantar keratoderma, including the use of emollients and keratolytic agents.
• Dental care to address hypodontia, which may involve the use of dental prostheses or implants to replace missing teeth.

Prognosis

The prognosis for individuals with Morillo-Cucci-Passarge Syndrome largely depends on the severity of the symptoms. While the condition does not typically affect life expectancy, it can impact quality of life. Regular follow-up with healthcare providers is important to manage symptoms effectively.

See Also

• Genetic disorder
• Autosomal recessive inheritance
• Keratoderma
• Hypodontia

Morillo–Cucci–Passarge syndrome Resources
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