fulltext(8)
TRANSCRIPT
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 clusterof smaller lesions [Mowbray M, Schofield OMV,2007;24(6):670–1]. We have recently seen a similar casedeveloping at 3 weeks of age, with two isolated plaques;onewasbehind the ear, and theother ona shoulder.Bothcomprised multiple coalescing papular lesions, and wereconfirmed on biopsy to be JXG. Both plaques showedsome flattening within 4 months. Clayton et al (1) alsorecently described a similar case of an isolated giantplaque of JXG with superimposed nodules occurringcongenitally. These further reports suggest that a plaqueof coalescing individual lesions is a recognized but under-reported variant of JXG.
REFERENCE
1. Clayton TH,Mitra A, Holder J et al. Congenital plaque onthe chest. Clin Exp Dermatol 2007;32:613–614.
JULIA GASS, M.R.C.P.C.H.NIGEL BURROWS, F.R.C.P.Cambridgeshire, United Kingdome-mail: [email protected]
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 withgreat interest. We recently treated a 4-year-old boy withmaple syrup urine disease (MSUD) and acrodermatitisenteropathica-like syndrome secondary to iatrogenicbranched-chain amino acid deficiency. The patient wasdiagnosed at 9 months of age with MSUD (plasmaleucine level was >1000 lmol ⁄L). His condition hasbeenmanagedwith dietary restriction of branched-chainamino acids (leucine, isoleucine, and valine). Twomonths before admission, a gastrostomy tube had beenplaced because of malnutrition caused by neurologicdeficits.At this time, as a result of inadequate oral intake,the dermatitis beganwithmoist, erythematous lesions onhis face and back, expanding to the neck, the dorsalaspects of the hands and feet, the forearms and lowerlegs, the axillary intertrigines, and the diaper region. Healso had diarrhea and diffuse hair loss. Laboratory
examinations found a lower plasma level of isoleucine(7.3 lmol ⁄L; targeted levels: 100–400), and a normallevel of leucine (328 lmol ⁄L; targeted levels: 200–700)and valine (360 lmol ⁄L; targeted levels: 100–400).Serum zinc concentrations were normal. Supplementa-tion of a high-calorie diet with isoleucine led to a promptimprovement of the boy’s skin lesions. A week later,plasma branched-chain amino acid levels were found tobe lower than before for all the three amino acids. Thismight have been related to the increased needs of amalnourished body in an anabolic stage. Two monthsafterward, plasma branched-chain amino acid levelsreached target levels.
Acrodermatitis enteropathica-like skin lesions seen inpatients with inborn errors of metabolism generally ariseas a result of protein-depletion nutrition (2–4). Thisproblem is relatively common among children withaminoacidopathies during dietary restriction of specificamino acids. Especially in instances of intercurrentinfections and prolonged starvation caused by surgicalprocedures, strictmonitoringofplasmaaminoacid levelsand serial measurements of body weight are highlyimportant in terms of dietary management. A multi-disciplinary approach accompanied by a strict follow-upcan lead to the decrease in these types of complications inchildren with aminoacidopathies. Physicians need to beaware of the risk factors (e.g., infections, prolongedstarvation, insufficient calorie ⁄protein, isoleucine, valineintake during the rapid growthphase) that can trigger thedevelopment of these complications and they should bealert to the signs of acrodermatitis enteropathica-likesyndrome.
REFERENCES
1. Lane TN, Spraker MK, Parker SS. Propionic acidemiamanifesting with low isoleucine generalized exfoliativedermatosis. Pediatr Dermatol 2007;24:508–510.
2. De Raeve L, De Meirleir L, Ramet J et al. Acrodermatitisenteropathica-like cutaneous lesions in organic aciduria.J Pediatr 1994;124:416–420.
3. Bodemer C, De Prost Y, Bachollet B et al. Cutaneousmanifestations of methylmalonic and propionic acidaemia:a description based on 38 cases. Br J Dermatol1994;131:93–98.
4. Giacoia GP, Berry GT. Acrodermatitis enteropathica-likesyndrome secondary to isoleucine deficiency during treat-ment of maple syrup urine disease. Am J Dis Child1993;147:954–956.
YESIM OZTURK, M.D.Izmir, Turkeye-mail: [email protected] (or)[email protected]
Correspondence 415