Cowden syndrome medical therapy

	Cowden syndrome Microchapters
Home
Patient Information
Overview
Historical Perspective
Classification
Pathophysiology
Causes
Differentiating Cowden Syndrome from other Diseases
Epidemiology and Demographics
Risk Factors
Screening
Natural History, Complications and Prognosis
Diagnosis
Diagnostic Study of Choice
History and Symptoms
Physical Examination
Laboratory Findings
Electrocardiogram
X-ray
Echocardiography and Ultrasound
CT scan
MRI
Other Imaging Findings
Other Diagnostic Studies
Treatment
Medical Therapy
Surgery
Primary Prevention
Secondary Prevention
Cost-Effectiveness of Therapy
Future or Investigational Therapies
Case Studies
Case #1
Cowden syndrome medical therapy On the Web
Most recent articles
Most cited articles
Review articles
CME Programs
Powerpoint slides
Images
American Roentgen Ray Society Images of Cowden syndrome medical therapy All Images X-rays Echo & Ultrasound CT Images MRI
Ongoing Trials at Clinical Trials.gov
US National Guidelines Clearinghouse
NICE Guidance
FDA on Cowden syndrome medical therapy
CDC on Cowden syndrome medical therapy
Cowden syndrome medical therapy in the news
Blogs on Cowden syndrome medical therapy
Directions to Hospitals Treating Psoriasis
Risk calculators and risk factors for Cowden syndrome medical therapy

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Vamsikrishna Gunnam M.B.B.S [2]

Overview

Due to different phenotypes cowden syndrome is likely to be an underdiagnosed condition. Pharmacologic medical therapy is recommended among patients with cutaneous manifestations in cowden syndrome.

Pharmacologic medical therapy is recommended among patients with cutaneous manifestations in cowden syndrome patients.
- Preferred regimen (1): Acitretin 30 mg/day for cutaneous manifestations.^[1]^[2]^[3]
  - Side effects:
    - Teratogenicity
    - Dryness of the eyes
    - Dryness of the nose
    - Dryness of the lips
    - Dryness of the mouth
    - Dryness of the skin
- Acitretin should be discontinued once the recurrence of skin lesions are noted.
- Preferred regimen (2): Sirolimus/ rapamycin
  - MOA: Inhibiting cell proliferation by inhibiting mTOR
Screening for multiple cancers should be effectively done in the patients with cowden syndrome.

↑ Kadakia KC, Barton DL, Loprinzi CL, Sloan JA, Otley CC, Diekmann BB, Novotny PJ, Alberts SR, Limburg PJ, Pittelkow MR (April 2012). "Randomized controlled trial of acitretin versus placebo in patients at high-risk for basal cell or squamous cell carcinoma of the skin (North Central Cancer Treatment Group Study 969251)". Cancer. 118 (8): 2128–37. doi:10.1002/cncr.26374. PMC 3365547. PMID 21882176.
↑ Chen K, Craig JC, Shumack S (March 2005). "Oral retinoids for the prevention of skin cancers in solid organ transplant recipients: a systematic review of randomized controlled trials". Br. J. Dermatol. 152 (3): 518–23. doi:10.1111/j.1365-2133.2005.06347.x. PMID 15787821.
↑ Bavinck JN, Tieben LM, Van der Woude FJ, Tegzess AM, Hermans J, ter Schegget J, Vermeer BJ (August 1995). "Prevention of skin cancer and reduction of keratotic skin lesions during acitretin therapy in renal transplant recipients: a double-blind, placebo-controlled study". J. Clin. Oncol. 13 (8): 1933–8. doi:10.1200/JCO.1995.13.8.1933. PMID 7636533.