Dermatologic Manifestations of Proteus Syndrome Treatment & Management

Updated: Jun 26, 2018
  • Author: Megan Barry, MD; Chief Editor: Dirk M Elston, MD  more...
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Treatment

Medical Care

The aim of medical treatment is to minimize the physical and psychosocial consequences of Proteus syndrome; this requires a multidisciplinary approach. [33]

Antithrombotic prophylaxis should be considered if the patient is undergoing a surgical procedure because the patient's vascular malformations predispose them to deep venous thromboses and fatal pulmonary emboli. [34]

Cystic pulmonary lesions should be followed closely because they may progress to pneumonia, atelectasis and potentially pulmonary insufficiency. Pulmonary hamartomas can impair respiratory function. One report describes the successful reduction in hamartoma size with rapamycin therapy. [35]

Cerebriform connective-tissue nevi are of considerable concern for patients, especially when present on the plantar surfaces, making walking uncomfortable. Conservative medical treatment for cerebriform connective-tissue nevi includes (1) keeping the feet clean and dry, (2) regularly applying antibacterial lotion to reduce odor, (3) closely monitoring for ulceration and infection, and (4) using orthotic devices. Attempts at surgical excision have led to poor outcomes; therefore, surgery is not recommended. [18]

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Surgical Care

Preoperative evaluation should include assessment of the airway anatomy and pulmonary reserve because of the frequent presence of tonsillar hypertrophy and pulmonary cysts. [36]

Plastic surgeons and orthopedic surgeons can correct some skeletal defects. Epiphysiodesis can help correct asymmetric epiphyseal growth, and reduction osteotomy can be used to shorten long bones. Spinal fusion for overgrown vertebrae may prevent the development of severe kyphoscoliosis and the risk of pulmonary compromise. [10, 33]

Subcutaneous tumors should be removed in the early stages. [37]

Gonadal deformities (ovarian or testicular) should be managed aggressively because of the high incidence of neoplastic transformation. [36] The Medscape Testicular Cancer Resource Center and Ovarian Cancer Resource Center may be of interest.

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Consultations

Consult a dermatologist.

Consult a plastic surgeon for the correction of defects.

Consult an orthopedic surgeon for the correction of skeletal deformities.

Ophthalmologists and neurologists may be helpful because many ocular manifestations and CNS anomalies associated with seizures or developmental delays are reported in patients with Proteus syndrome.

Referral to psychologist or psychiatrist is important for patients and for their families. The disease creates an isolating social stigma associated with having a very rare, progressive, disfiguring condition. Symptoms of depression have been reported in 23% of parents of affected children. [38]

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