Trichorrhexis Invaginata (Netherton Syndrome or Bamboo Hair) Treatment & Management

  • Author: Tina S Chen, MD; Chief Editor: Dirk M Elston, MD   more...
 
Updated: Aug 17, 2011
 

Medical Care

Management is conservative. Emollients, keratolytics, and antibiotics are the mainstay of treatment in Netherton syndrome. White, soft paraffin and liquid paraffin in a ratio of 50:50 have been used as a topical treatment.

  • Management essentially relies on the treatment of cutaneous infections and other complications.
  • Topical steroids of moderate potency are helpful, but reports have described complications such as aminoaciduria and pituitary adrenal axis suppression, especially in patients with widespread erythroderma, and thus should be used with caution.
  • In one case, split body treatment with 12% lactic acid lotion surprisingly cleared the eruption on the treated side (control side emollient).[41]
  • Although topical tacrolimus or pimecrolimus was previously discouraged in the treatment of Netherton syndrome due to toxicity from absorption of the medication,[42] new evidence suggests that pimecrolimus 1% cream applied twice daily may be well tolerated and lead to improvement of skin involvement, with low, safe levels absorbed systemically.[43] Further, larger studies are necessary to elucidate the safety and efficacy of pimecrolimus in Netherton syndrome patients.[43]
  • One Japanese woman's condition has been reported to respond to psoralen plus ultraviolet light A (PUVA).[44]
  • Retinoids are generally not helpful,[45] although a few reports of improvement with a low dose have been published.[46, 47]
  • Infliximab was found to lead to sustained clinical improvement and clearance of ichthyosis linearis circumflexa in one study[48]
  • The potential of treating Netherton syndrome patients with gene therapy[49, 50] and epidermal stem cell therapy[51] is currently being studied.
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Consultations

  • Consult a pediatrician for systemic infections, hypernatremic dehydration, and failure to thrive.
  • Consult a gastroenterologist for failure to thrive, diarrhea, and villous atrophy of the small bowel.
  • Consult a geneticist for genetic studies and counseling.
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Contributor Information and Disclosures
Author

Tina S Chen, MD  Pediatric Dermatology Fellow, Rady Children's Hospital, Department of Dermatology, University of California, San Diego

Tina S Chen, MD is a member of the following medical societies: American Academy of Dermatology, California Society of Dermatology and Dermatologic Surgery, and Society for Pediatric Dermatology

Disclosure: Nothing to disclose.

Specialty Editor Board

James W Patterson, MD  Professor of Pathology and Dermatology, Director of Dermatopathology, University of Virginia Medical Center

James W Patterson, MD is a member of the following medical societies: American Academy of Dermatology, American College of Physicians, American Society of Dermatopathology, Royal Society of Medicine, Society for Investigative Dermatology, and United States and Canadian Academy of Pathology

Disclosure: Nothing to disclose.

David F Butler, MD  Professor of Dermatology, Texas A&M University College of Medicine; Chair, Department of Dermatology, Director, Dermatology Residency Training Program, Scott and White Clinic, Northside Clinic

David F Butler, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American Medical Association, American Society for Dermatologic Surgery, American Society for MOHS Surgery, Association of Military Dermatologists, and Phi Beta Kappa

Disclosure: Nothing to disclose.

Van Perry, MD  Assistant Professor, Department of Medicine, Division of Dermatology, University of Texas School of Medicine at San Antonio

Van Perry, MD is a member of the following medical societies: American Academy of Dermatology and American Society for Laser Medicine and Surgery

Disclosure: Nothing to disclose.

Glen H Crawford, MD  Assistant Clinical Professor, Department of Dermatology, University of Pennsylvania School of Medicine; Chief, Division of Dermatology, The Pennsylvania Hospital

Glen H Crawford, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American Medical Association, Phi Beta Kappa, and Society of USAF Flight Surgeons

Disclosure: Nothing to disclose.

Chief Editor

Dirk M Elston, MD  Director, Ackerman Academy of Dermatopathology, New York

Dirk M Elston, MD is a member of the following medical societies: American Academy of Dermatology

Disclosure: Nothing to disclose.

Acknowledgments

The author and editors of Medscape Reference gratefully acknowledge the contributions of previous authors, Mohsin Ali, MBBS, FRCP, MRCP, and David T Robles, MD, PhD, to the development and writing of this article.

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