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Year : 2015  |  Volume : 1  |  Issue : 1  |  Page : 35-36

Excellent response to oral corticosteroids in netherton's syndrome

1 Department of Dermatology, Jawaharlal Nehru Medical College, Wardha, Maharashtra, India
2 Consultant Dermatologist, Nagpur, Maharashtra, India

Date of Web Publication1-Dec-2015

Correspondence Address:
Nitin Lade
Department of Dermatology, Jawaharlal Nehru Medical College, Sawangi (Meghe), Wardha, Maharashtra
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/WKMP-0110.170748

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How to cite this article:
Lade N, Saoji V. Excellent response to oral corticosteroids in netherton's syndrome. Indian J Drugs Dermatol 2015;1:35-6

How to cite this URL:
Lade N, Saoji V. Excellent response to oral corticosteroids in netherton's syndrome. Indian J Drugs Dermatol [serial online] 2015 [cited 2023 Jun 1];1:35-6. Available from: https://www.ijdd.in/text.asp?2015/1/1/35/170748


Netherton's syndrome (NS) is an autosomal recessive disorder characterized by triad of ichthyosis linearis circumflexa (ILC), trichorrhexis invaginata (TI), and atopic dermatitis.[1] Regular use of emollient is the backbone of treatment, but significant improvement with topical tacrolimus and pimecrolimus has been reported.[1],[2] The use of corticosteroids for the treatment of this disorder of keratinization has not been frequently reported.

An 18-month-old male child, moderately built, born by nonconsanguineous marriage, referred from Pediatric Department for reddish, itchy, and scaly lesions all over the body since 1-year. Lesions had continuous waxing and waning course, in between the skin becomes normal. There was a history of sparseness of scalp and eyebrow hairs, and of redness of cheek since birth. There was no history of atopy, recurrent infections, or erythroderma. On cutaneous examination, there were multiple erythematous, annular or polycyclic scaly patches, with double edged scales (ILC) at the periphery of the lesions [Figure 1]a present over the trunk, proximal part of extremities and face. Scalp and eyebrows hairs were sparse, rough, brittle, and lusterless with traumatic alopecia on scalp. There was no nail or mucosal involvement. On investigation, complete hemogram, liver, and kidney function tests were normal. Immunoglobulin E (IgE) level was 38.6 IU/ml (normal). Light microscopy of eyebrow hair revealed TI (bamboo hair) [Figure 2].
Figure 1: (a) Skin lesions typical of Netherton's syndrome prior to the treatment; (b) excellent response to oral corticosteroids in Netherton's syndrome.

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Figure 2: Trichorrhexis invaginata or “Bamboo hair” in our patient of Netherton's syndrome.

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Before referring to us, a pediatrician had given 5 mg of syrup prednisolone (0.5 mg/kg) once a day for 2 days considering it as eczema. We added white soft paraffin for twice daily application. Oral steroids were completely tapered in the next 8 days without any side effects. The patient got complete relief from itch and skin lesions after starting oral steroid [Figure 1]b, but there was no improvement in hair. After discontinuation of oral corticosteroids, the patient was observed for the next 4 weeks and showed no recurrence. The patient could not be followed up later.

Mutation in serine protease inhibitor Kazal type 5 gene has been identified in NS patients.[3] ILC is characteristic, but the TI is diagnostic.[1] Furio and Hovnanian postulated severe form of NS in which erythroderma and pustular lesions are seen, also the family history of atopy and raised IgE level [4] are common which was not noted in this case. In NS lesions of ILC are that studies in mouse models and in NS patients have established that unopposed kallikrein 5 activity triggers stratum corneum detachment and activates proteinase-activated receptor 2 signaling, leading to the autonomous production of pro-allergic and pro-inflammatory mediators.[5]

For similar reasons, anti-inflammatory or immunosuppressive drugs has been tried in the treatment of severe NS. Maatouk et al. reported successful use of narrow-band phototherapy in the treatment of NS.[6]

The incidental oral steroids to our patient had given him complete relief from itch and skin lesions. Remission after discontinuation of tapering dosages of corticosteroids lasted for 4 weeks. As patient did not come for follow-up after 4 weeks, the long-term effect of short dosages of corticosteroids could not be ascertained. To the best of our knowledge, the use of oral corticosteroids for treatment of NS has not been reported. The use of corticosteroids for treatment of NS has a potential of significant corticosteroids side effects but use in NS can be limited for short duration of the time and only in severe cases of NS. Halverstam et al. reported a case of Cushing' syndrome (CS) after 1-year of application of 1% hydrocortisone ointment for NS.[7]

We suggest that oral corticosteroids or topical corticosteroids may be tried for symptomatic relief from itching, eczematization, and ichthyosis in NS. Considering this observation, even in acute erythroderma which is accompanied by severe inflammatory changes, a short course of low dose oral steroids may be tried. Long-term follow-up to look for safety and efficacy of CS in NS is required.

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There are no conflicts of interest.

  References Top

Judge MR, McLean WH, Munro CS. Disorders of keratinization. In: Burn T, Breathnach S, Cox N, Griffiths C, editors. Rook's Text of Dermatology. 8th ed. Oxford: Wiley Blackwell; 2010. p. 19.37-19.41.  Back to cited text no. 1
Wehr RF, Hickman J, Krochmal L. Effective treatment of Netherton's syndrome with 12% lactate lotion. J Am Acad Dermatol 1988;19(1 Pt 1):140-2.  Back to cited text no. 2
Chavanas S, Bodemer C, Rochat A, Hamel-Teillac D, Ali M, Irvine AD, et al. Mutations in SPINK5, encoding a serine protease inhibitor, cause Netherton syndrome. Nat Genet 2000;25:141-2.  Back to cited text no. 3
Greene SL, Muller SA. Netherton's syndrome. Report of a case and review of the literature. J Am Acad Dermatol 1985;13(2 Pt 2):329-37.  Back to cited text no. 4
Furio L, Hovnanian A. Netherton syndrome: Defective kallikrein inhibition in the skin leads to skin inflammation and allergy. Biol Chem 2014;395:945-58.  Back to cited text no. 5
Maatouk I, Moutran R, Tomb R. Narrowband ultraviolet B phototherapy associated with improvement in Netherton syndrome. Clin Exp Dermatol 2012;37:364-6.  Back to cited text no. 6
Halverstam CP, Vachharajani A, Mallory SB. Cushing syndrome from percutaneous absorption of 1% hydrocortisone ointment in Netherton syndrome. Pediatr Dermatol 2007;24:42-5.  Back to cited text no. 7


  [Figure 1], [Figure 2]


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