Correspondence

415

PAPULAR JUVENILE XANTHOGRANULOMA EN PLAQUE IN AN INFANT To the Editor: We read with interest the report of a solitary juvenile xanthogranuloma (JXG) en plaque comprising a cluster of smaller lesions [Mowbray M, Schoeld OMV, 2007;24(6):6701]. We have recently seen a similar case developing at 3 weeks of age, with two isolated plaques; one was behind the ear, and the other on a shoulder. Both comprised multiple coalescing papular lesions, and were conrmed on biopsy to be JXG. Both plaques showed some attening within 4 months. Clayton et al (1) also recently described a similar case of an isolated giant plaque of JXG with superimposed nodules occurring congenitally. These further reports suggest that a plaque of coalescing individual lesions is a recognized but underreported variant of JXG. REFERENCE
1. Clayton TH, Mitra A, Holder J et al. Congenital plaque on the chest. Clin Exp Dermatol 2007;32:613614. JULIA GASS, M.R.C.P.C.H. NIGEL BURROWS, F.R.C.P.
Cambridgeshire, United Kingdom e-mail: julia.gass@addenbrookes.nhs.uk

ACRODERMATITIS ENTEROPATHICA-LIKE SYNDROME SECONDARY TO BRANCHED-CHAIN AMINO ACID DEFICIENCY IN INBORN ERRORS OF METABOLISM To the Editor: I read the article by Lane et al (1), which appeared in the September issue [2007; 24 (5)] of the journal with great interest. We recently treated a 4-year-old boy with maple syrup urine disease (MSUD) and acrodermatitis enteropathica-like syndrome secondary to iatrogenic branched-chain amino acid deciency. The patient was diagnosed at 9 months of age with MSUD (plasma leucine level was >1000 lmol L). His condition has been managed with dietary restriction of branched-chain amino acids (leucine, isoleucine, and valine). Two months before admission, a gastrostomy tube had been placed because of malnutrition caused by neurologic decits. At this time, as a result of inadequate oral intake, the dermatitis began with moist, erythematous lesions on his face and back, expanding to the neck, the dorsal aspects of the hands and feet, the forearms and lower legs, the axillary intertrigines, and the diaper region. He also had diarrhea and diuse hair loss. Laboratory

examinations found a lower plasma level of isoleucine (7.3 lmol L; targeted levels: 100400), and a normal level of leucine (328 lmol L; targeted levels: 200700) and valine (360 lmol L; targeted levels: 100400). Serum zinc concentrations were normal. Supplementation of a high-calorie diet with isoleucine led to a prompt improvement of the boys skin lesions. A week later, plasma branched-chain amino acid levels were found to be lower than before for all the three amino acids. This might have been related to the increased needs of a malnourished body in an anabolic stage. Two months afterward, plasma branched-chain amino acid levels reached target levels. Acrodermatitis enteropathica-like skin lesions seen in patients with inborn errors of metabolism generally arise as a result of protein-depletion nutrition (24). This problem is relatively common among children with aminoacidopathies during dietary restriction of specic amino acids. Especially in instances of intercurrent infections and prolonged starvation caused by surgical procedures, strict monitoring of plasma amino acid levels and serial measurements of body weight are highly important in terms of dietary management. A multidisciplinary approach accompanied by a strict follow-up can lead to the decrease in these types of complications in children with aminoacidopathies. Physicians need to be aware of the risk factors (e.g., infections, prolonged starvation, insucient calorie protein, isoleucine, valine intake during the rapid growth phase) that can trigger the development of these complications and they should be alert to the signs of acrodermatitis enteropathica-like syndrome. REFERENCES
1. Lane TN, Spraker MK, Parker SS. Propionic acidemia manifesting with low isoleucine generalized exfoliative dermatosis. Pediatr Dermatol 2007;24:508510. 2. De Raeve L, De Meirleir L, Ramet J et al. Acrodermatitis enteropathica-like cutaneous lesions in organic aciduria. J Pediatr 1994;124:416420. 3. Bodemer C, De Prost Y, Bachollet B et al. Cutaneous manifestations of methylmalonic and propionic acidaemia: a description based on 38 cases. Br J Dermatol 1994;131:9398. 4. Giacoia GP, Berry GT. Acrodermatitis enteropathica-like syndrome secondary to isoleucine deciency during treatment of maple syrup urine disease. Am J Dis Child 1993;147:954956. ZTU RK, M.D. YESIM O
Izmir, Turkey e-mail: yesimzaferozturk@gmail.com (or) yesimozturk@deu.edu.tr