The treatment of Netherton Syndrome (NS) is directed towards the specific symptoms that are apparent in each individual. The treatment of NS is symptomatic, often difficult, and should be personalized to the patient's specific needs.
The recommendations include the regular use of emollients and moisturizing creams and lotions. Other topical agents should be used with caution because the skin in NS may allow ingredients from some topically applied medications to be absorbed into the blood, which may pose a danger to the child.
Topical keratolytic agents, such as urea or lactic acid derivatives may be limited by skin irritation and should generally be reserved for older children. The baseline treatment also includes oral antihistamines, which can help to control the itchy, eczematous component, and topical or systemic antibiotics as needed. To treat the scaling of the scalp, mild dandruff shampoos and topical steroids might be helpful.
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Oral and topical steroids are beneficial in reducing inflammation and the eczematous component of the disease. However, the well-documented side effects of long-term steroid use need to be considered. Oral retinoids have been used with varying success, leading to a dramatic improvement in some patients and severe worsening of the disease in others. Current treatment options are limited to topical treatment with mild moisturizers containing petrolatum or lanolin and/or a skin barrier repair formula containing ceramides or cholesterol.
Topical calcineurin inhibitors (pimecrolimus and tacrolimus) have been shown to be helpful for individuals with NS, must be used sparingly because they are absorbed easily through the abnormal stratum corneum, and widespread use can lead to dangerously high blood levels. Topical steroids are also readily absorbed and should be used in low potency forms to limited areas of the body for short periods of time.
Psoralen plus ultraviolet light A (PUVA) and narrowband ultraviolet light B (NBUVB) have been shown to be beneficial in some case reports, though patients treated with these modalities need to be monitored for the development of skin malignancy.
More advanced therapy for treatment-refractory cases includes intravenous immunoglobulin, which has shown efficacy in severe cases of NS, although this is a costly treatment and requires monthly infusions, potentially making it impractical for this chronic condition.
Infliximab (anti-TNFα) infusions have also been reported to cause a significant improvement in cutaneous inflammation associated with NS, although further studies are still required.
Kallikrein inhibitors represent a new potential therapy for NS, with Tan et al.demonstrating a reduction in human kallikrein proteolytic activity within transgenic mice.
In NS, kallikrein inhibition is typically absent, and thus the inhibitor properties of this treatment may prevent or minimize the inflammatory cutaneous features that are typically present. This study also identified minimal cytotoxic activity of some kallikrein inhibitors against healthy keratinocytes, suggesting that it may be safe for human use, pending further studies.
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