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  • Title: Adrenoleucodystrophy: dermatological findings and skin surface lipid study.
    Author: Papini M, Calandra P, Calvieri S, Laureti S, Casucci G.
    Journal: Dermatology; 1994; 188(1):25-7. PubMed ID: 8305751.
    Abstract:
    X-linked adrenoleucodystrophy (ALD) is a rare disorder of the very-long-chain fatty acid (VLCFA) metabolism. Cutaneous findings observed in a 29-year-old man included patchy non-cicatricial alopecia of the scalp, intense oily seborrhoea of the head, mild dry-scaling ichthyosis-like appearance of the trunk and legs and pseudo-acanthosis nigricans of the folds. Scanning electron-microscopic examination of the scalp hair showed trichorrhexis-nodosa-like fractures and several structural anomalies of the hair shaft. The skin surface lipid profile showed a marked increase in lignoceric acid (C24:0) which comprised over 60% of the fatty acid detectable in the fractions of triglycerides, diglycerides and free fatty acids. This behaviour of the VLCFA in the surface lipids corresponds to that observed in the plasma. If this finding were confirmed, the investigation of surface lipid composition could represent a useful non-invasive technique for the study of ALD.
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