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

Updated: Dec 18, 2017
  • Author: Laura F McGevna, MD; Chief Editor: Dirk M Elston, MD  more...
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Treatment

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 may be 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). [58]

Although topical tacrolimus or pimecrolimus was previously discouraged in the treatment of Netherton syndrome due to toxicity from absorption of the medication, [59] newer 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. [60] Further, larger studies are necessary to elucidate the safety and efficacy of pimecrolimus in Netherton syndrome patients. [60]

Psoralen plus ultraviolet light A (PUVA) and narrowband ultraviolet light B (NBUVB) have been shown to be beneficial in some case reports, [61, 62, 63] though patients treated with these modalities need to be monitored for the development of skin malignancy. [64]

Retinoids are generally not helpful, [65] although a few reports of improvement with a low dose have been published. [66, 67]

Infliximab was found to lead to sustained clinical improvement and clearance of ichthyosis linearis circumflexa in one study. [68]

The potential of treating Netherton syndrome patients with gene therapy [69, 70] and epidermal stem cell therapy [71] is currently being investigated.

A patient allergic to antibiotics showed significant improvement after treatment with several antistaphylococcal bacteriophage preparations. [72]

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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.

Consider consultation for pelvic examination for men and women with this condition.

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Long-Term Monitoring

Trichorrhexis invaginata and ichthyosis linearis circumflexa usually develop after age 2 years, but hair should be examined every 6 months because it rarely may occur earlier.

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