Netherton’s syndrome

Contraindications Hypersensitivity to pimecrolimus or any component of the formulation, Netherton s syndrome (potential for increased systemic absorption), application to active cutaneous viral infections. 

A major benefit of topical tacrolimus compared with topical glucocorticoids is that tacrolimus does not cause skin atrophy and therefore can be used safely in locations such as the face and intertriginous areas. Common side effects at the site of application are transient erythema, burning, and pruritus, which tend to improve with continued treatment. Systemic absorption generally is very low and decreases with resolution of the dermatitis. However, topical tacrolimus should be used with extreme caution in patients with Netherton s syndrome because these patients have been shown to develop elevated blood levels of the drug after topical application. It is recommended that patients using tacrolimus use sunscreen and avoid excessive UV exposure. 

Ichthyosis linearis cirumflexa Cornel) will not be reported here. Frenk and Mevorah as well as Thorne and co-workers have shown that unknown substances are formed in the border of growing lesions where parakeratosis is present and keratohyalin granules are missing We found this process not only to be limited locally but to be restricted to short periods of abnormal differentiation (unpublished observations). In Netherton s syndrome, this type of ichthyosis is combined with trichorrhexis invaginata, the ultrastructure of which was described by Orfanos et aiy. ... 

Frenk, E. and Mevorah, B. (1972). Ichthyosis linearis circumflexa Cornel with trichorrhexis invaginata (Netherton s syndrome). An ultrastructural study of skin changes. Arch. Dermatol Forsch., 245,42... 

Netherton s syndrome is an inherited, autosomal recessive condition, but almost only females are affected . It starts as a transient erythroderma and subsequently develops migratory and fixed cutaneous lesions (similar to those of Erythrokeratodermia variabilis), a characteristic trichorrhexis invaginata on the scalp, eyebrows and eyelashes, and atopy. Palms, soles and nails are normal. The ingestion of nuts causes the exudation of skin lesions. Histologically, hyperkeratosis with parakeratosis, discontinuous stratum granu-losum, acanthosis and prominent rete ridges are present. Mononuclear exocytosis and infiammatory infiltrate in the upper dermis are also observed. 

The patients with X-linked ichthyosis, Netherton s syndrome, nevus comedonicus, or psoriasis either worsened or failed to improve. 

Chavanas S, Bodemer C, Rochat A, et al. Mutations in SPINK5, encoding a serine protease inhibitor, cause Netherton syndrome. Nat Genet 2000 25 141-142. 

Chavanas S, Bodemer C, Rochat A, Hamel-Teillac D, Ali M, Irvine AD, Bonafe JL, Wilkinson J, Taieb A, Barrandon Y, Harper JI, de Prost Y, Hovnanian A. Mutations in SPINK5, encoding a serine protease inhibitor, cause Netherton syndrome. Nat. Genet. 2000 25 141-142. 

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