The authors reported a case of Netherton's syndrome. This patient presented cutaneous lesions of ichthyosis linearis circumflexa, tricorrexis invaginata and atopy signs associated with cystinuria. Cutaneous and hair lesions were treated with etretinate.

1. Barrera M, Bosch R, Mendiola M, Frieyro M, Castillo R, Fernández A, et al. Reactivación de la sífilis en Málaga. Actas Dermosifiliogr. 2006;97:323-6. 2. Dourmishev LA, Dourmishev AL. Syphilis: uncommon presentations in adults. Clin Dermatol. 2005;23:555-64. 3. Baniandrés Rodríguez O, Nieto Perea O, Moya Alonso L, Carrillo Gijón R, Harto Castaño A. Nodular secondary syphilis in a HIV patient ...

© 2017 Indian Dermatology Online Journal | Published by Wolters Kluwer Medknow Trichorrhexis nodosa (TN) is a well‐known entity which affects hair shafts. Clinically, it presents as minute nodular concretions along the hair shaft. This is caused by the loss of cuticle and cortical fibers. On light microscopy, tiny nodules appear as “thrust paint brushes” as if two brushes are thrust into each o...

We report a case of Noonan syndrome with loose anagen hair (NS/LAH), a rare variant of Noonan syndrome, with associated trichorrhexis nodosa and trichoptilosis. The SHOC2 mutation may be responsible for these additional hair shaft defects, revealing the importance of microscopic examination of hairs in these patients.

Netherton syndrome (NS) is a rare autosomal reces-sive genodermatosis caused by SPINK-5 mutations. The SPINK-5 gene encodes the serine protease inhibitor LEKTI and is located on chromosome 5q32. Unopposed degradation of corneodesmosomes is the basis for a severely impaired skin barrier function in patients with NS. Effective treatments for patients with NS are limited. Some success has been ach...

Netherton’s syndrome Cyclosporine Dr. A.A. Ramelet, 2, place Benjamin-Constant, CH-1003 Lausanne (Switzerland), Tel. 021 312 60 60, Fax 021 320 40 90 In 1958, Netherton [IJ reported a unique case of trichorrhexis nodosa ‘bamboo hairs’. Netherton’s syndrome (NS) consists of the triad of ichthyosiform dermatosis, multiple defects of the hair shaft (trichorrhexis invaginata) and an atopic diathesi...