Biotin Deficiency in an Infant Fed with Amino Acid Formula

Biotin deficiency is rarely encountered in an infant on weaning from breast and formula feeding. It is characterized by alopecia and scaly, erythematous dermatitis distributed around the body orifices. We report a 5‐month‐old Japanese infant with typical skin lesions who had been diagnosed as a neon...

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Bibliographic Details
Published in:Journal of dermatology 2005-04, Vol.32 (4), p.256-261
Main Authors: Fujimoto, Wataru, Inaoki, Makoto, Fukui, Toru, Inoue, Yoshito, Kuhara, Tomiko
Format: Article
Language:English
Subjects:
alopecia
biotin
Biotin - administration & dosage
Biotin - blood
Biotin - deficiency
Biotin - urine
Deficiency Diseases - diagnosis
Deficiency Diseases - drug therapy
Deficiency Diseases - pathology
Dietary Supplements
Erythema - diagnosis
Erythema - drug therapy
Erythema - pathology
Facial Dermatoses - diagnosis
Facial Dermatoses - drug therapy
Facial Dermatoses - pathology
formula
Humans
Infant
Infant Formula
Male
organic aciduria
periorificial dermatitis
Peritoneum
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Summary:Biotin deficiency is rarely encountered in an infant on weaning from breast and formula feeding. It is characterized by alopecia and scaly, erythematous dermatitis distributed around the body orifices. We report a 5‐month‐old Japanese infant with typical skin lesions who had been diagnosed as a neonate with dyspepsia and fed only an amino acid formula. Serum and urine levels of biotin were below the normal range, but zinc and biotinidase were within normal range. Urinary excretion of 3‐methylcrotonylglycine, 3‐hydroxyisovaleric acid, and methylcitric acid was significantly elevated. Daily oral supplementation with 1 mg of biotin resulted in dramatic improvement of the periorificial dermatitis and hair growth together with a complete disappearance of the organic aciduria. Our case shows that the characteristic skin manifestations are the most important clue to the diagnosis of biotin deficiency and demonstrated that urinary excretion of biotin and organic aciduria, rather than the serum concentration of biotin, are the sensitive indicators for evaluating the patient's status of biotin deficiency.
ISSN:0385-2407
1346-8138
DOI:10.1111/j.1346-8138.2005.tb00758.x