Acrodermatitis acidemica with an eating disorder

ARTICLE

Auteur(s) : Shiro NIIYAMA, Ryoko OKIYAMA, Kazuya NAGAHASHI, Satoshi KANEKO, Shinsaku AIBA, Hideki MUKAI

Department of Dermatology, Yokohama Rosai Hospital, 3211 Kozukue, Kohoku-ku, Yokohama, Kanagawa, 222-0036 Japan.
<sniiyama@aol.com>

We experienced a patient who had an eruption typically derived from an eating disorder which was associated with decreases in the majority of amino acid fractions on hematological examination.

Case report

The patient, a Japanese male infant, was born after an uneventful pregnancy and delivery, as the first child of healthy unrelated parents. He had a good start, and examination showed no abnormalities.
At 30 days of age, his skin condition worsened, with erythema, superficial erosions, desquamation, and hyperkeratosis which started on the face and trunk (figure 1), expanding to the neck, extremities, axillary intertrigines and the diaper region. Superficial desquamation of his lips and perioral erosions were evident. The scalp hair was noted to be sparse and brittle, but trichorrhexis invaginata was not seen.
Histopathological examination revealed hyperorthokeratosis intermingled with parakeratosis, pallor of the upper part of the epidermis and focal subcorneal neutrophil invasion. The papillary dermis showed edema and an inflammatory infiltrate composed of lymphocytes and histiocytes.
Laboratory studies showed depressed serum levels of albumin (2.3 g/dL [normal range, 3.8 to 5.8 g/dL]) and a majority of amino acids. Serum zinc level was 78 μg/dL (normal range, 65 to 110 μg/dL) and fasting serum glucagon level was 93 pg/mL (normal range, 40 to 140 pg/mL). Skin cultures revealed Staphylococcus aureus and were negative for mycoses. No abnormalities were revealed by ultrasonography of the chest or abdomen or by CT. The chronic course was not influenced by any of the treatments attempted: antibiotics and topical corticosteroids. At present the patient has an eating disorder and an associated growth disorder. The pathogeneses are being investigated in detail.

Discussion

A periorificial dermatitis has previously been described in patients with branched-chain organic acid disorders [1, 2]. For this cutaneous disorder, Happle proposed the term «acrodermatitis acidemica» (AA), by analogy with acrodermatitis enteropathica (AE), for the periorificial manifestations observed in patients with the various forms of organic hyperacidaemia [3]. In some cases an amino acid deficiency was suspected to be the major factor leading to cutaneous lesions. This view is supported by the observation of Shipley and Pittelkow [4], who studied the influence of various nutritional components on the growth and differentiation of human keratinocytes cultured in serum-free media. Their investigations revealed that depletion of several essential amino acids in the basal medium arrested the growth of keratinocytes.
The skin lesions typically develop gradually over a few weeks, are especially painful and oozing. The erosive erythema shows an affinity to the diaper region, the neck folds, the periorificial areas (mouth, nose, ears, eyes, and perineum) and the extremities. Initially, vesiculobullous lesions become dry, hyperkeratotic, and psoriasiform in appearance. With the exception of cheilitis and glossitis, the mucous membranes are usually spared, but sparse, fine scalp hair appears.
Histopathological changes show pallor of the upper part of the epidermis with necrosis of keratinocytes. A diffuse parakeratosis is seen overlying the pale epidermal cells. Sometimes a subcorneal vesicle is present above the area of paleness of the epidermis.
Although the pathogenesis of the eruption in our present patient remains unknown, the eruption is considered to have been caused by hypoalbuminemia associated with the eating disorder in this patient. At present, with ongoing detailed clinical examinations, further studies on the etiology of the eruption may be undertaken. Understanding the pathophysiological factors of the cutaneous eruptions in these disorders may help elucidate fundamental metabolic relationships in the epidermis. n

1. Niiyama S, Koelker S, Degen I, Hoffmann GF, Happle R, Hoffmann R. Acrodermatitis acidemica secondary to malnutrition in glutaric aciduria type I. Eur J Dermatol 2001; 11: 244-6.

2. Koopman RJJ, Happle R. Cutaneous manifestations of methylmalonic acidemia. Arch Dermatol Res 1990; 282: 272-3.

3. Happle R. Neurocutanous diseases. In: Freedberg IM, Eisen AZ, Wolff K, Austen KF, Goldsmith LA, Katz SI, Fitzpatrick TB, eds. Dermatology in General Medicine, 5^th ed. New York: McGraw-Hill, 1999: 2131-48.

4. Shipley GD, Pittelkow MR. Control of growth and differentiation in vitro of human keratinocytes cultured in serum-free medium. Arch Dermatol 1987; 123: 1541-4.