Elastase-inhibiting activity in scaling skin disorders.

Elastase inhibiting activity (EIA) has been observed in normal skin as a response to surface trauma, immediately following the intra-epidermal accumulation of polymorphonuclear leukocytes (PMN). In order to elucidate the relation between EIA and inflammation, the inhibiting activity was assessed in skin samples of scaling dermatoses (a) without significant inflammation: erythrodermic autosomal recessive lamellar ichthyosis (EARLI), non-erythrodermic autosomal recessive lamellar ichthyosis (NEARLI), X-linked recessive ichthyosis (XLRI) and X-linked dominant chondrodysplasia punctata (XLD-CDP); (b) with predominantly mononuclear cell infiltration: atopic dermatitis; (c) with mixed infiltration of PMN and mononuclear cells: psoriasis and Netherton syndrome. All skin disorders investigated showed an increased EIA as compared with normal skin. Scales from psoriatic lesions, EARLI and Netherton syndrome showed a statistically significant increase in EIA above that observed in other monogenic disorders of keratinization NEARLI, XLRI XLD-CDP and above atopic dermatitis. EIA proved to be an indicator for abnormal keratinization with a marked expression when a mixed infiltrate is present in the skin.