title: Netherton syndrome: successful use of topical tacrolimus and pimecrolimus in four siblings.
abstract: Netherton's syndrome (NS) is a rare autosomal recessive disease comprised of ichthyosis in the form of ichthyosis linearis circumflexa, hair shaft defects and atopic manifestations with an elevated IgE level. Various therapeutic options have been used in NS with variable success. Tacrolimus and pimecrolimus belong to the family of calcineurin inhibitors. They bind cytoplasmic proteins and the resulting complex binds calcineurin, inhibiting its ability to dephosphorylate the nuclear factor of activated T cells, thus suppressing gene transcription. There have been conflicting reports of the usefulness of tacrolimus in NS patients, with systemic absorption being the main adverse outcome. Here we report four Saudi siblings (two boys and two girls) with NS who were treated with topical tacrolimus and pimecrolimus with good control of their skin disease without any toxic effect. To our knowledge, this is the second report of the use of topical pimecrolimus in NS in the English literature.
authors.affiliation: Division of Dermatology, Department of Medicine, King Fahad National Guard Hospital, King Abdulaziz Medical City-Riyadh, Saudi Arabia.
authors.forename: Ghada
authors.initials: G
authors.lastname: Bin Saif
authors.forename: Sultan
authors.initials: S
authors.lastname: Al-Khenaizan
mesh_terms: D000287:Administration, Topical; D002648:Child; D002675:Child, Preschool; D003879:Dermatologic Agents / Q000008:administration & dosage*; D004359:Drug Therapy, Combination; D005260:Female; D006197:Hair / Q000002:abnormalities*; D006801:Humans; D007057:Ichthyosis / Q000188:drug therapy* / Q000235:genetics; D007166:Immunosuppressive Agents / Q000008:administration & dosage*; D007223:Infant; D008297:Male; D012074:Remission Induction; D013577:Syndrome; D016559:Tacrolimus / Q000008:administration & dosage* / Q000031:analogs & derivatives*
chemical_list: D003879:Dermatologic Agents; D007166:Immunosuppressive Agents; C117268:pimecrolimus; D016559:Tacrolimus