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Hypomelanosis of Ito with ileal atresia
Author(s) -
Kaya Tamer Irfan,
Kokturk Aysin,
Nayci Ali,
Aksoyek Selim,
Dusmez Duygu
Publication year - 2001
Publication title -
international journal of dermatology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.677
H-Index - 93
eISSN - 1365-4632
pISSN - 0011-9059
DOI - 10.1046/j.1365-4362.2001.01281-2.x
Subject(s) - medicine , surgery , abdominal distension , asymptomatic , atresia
A 1‐year‐old girl was referred to our dermatology clinic by the Pediatric Surgery clinic for hypopigmented macular lesions over her trunk and proximal parts of the extremities. Physical examination revealed bilateral streak and whorl shaped hypopigmented macular lesions which had developed gradually since birth and were distributed along Blaschko's lines ( Fig. 1). Some of the lesions, particularly the ones located in the gluteal region, gave a ‘‘splashed water’' appearance ( Fig. 2). The majority of these macular lesions measured between 1–3 cm in diameter. They were asymptomatic. There was neither a history of preceding lesions nor a history of trauma at the sites of the lesions. The patient was otherwise healthy. 1Streak and whorl shaped hypopigmented macular lesions along Blaschko’s lines2The lesions located in the gluteal region gave a "splashed water’’ appearance The history of the patient revealed congenital ileal atresia. The patient was presented to the Pediatric Surgery clinic with abdominal distension, bilious vomiting, and lack of meconium passage at the second day after birth. Surgery was performed upon diagnosis of ileus and an ileal atresia (type II) was detected at the operation. The congenital defects of the small bowels were removed and the intestines were repaired with end to end anastomosis. The diagnosis of the congenital ileal atresia was confirmed by a histopathologic examination of the removed tissue specimens. The child was the product of a normal pregnancy, routine ultrasound was normal and there was no history of exposures to medications, infections or X‐rays during the pregnancy. She was the fourth child of nonconsanguineous parents. There was no family history of a congenital anomaly or similar cutaneous lesions. A biopsy specimen was obtained from a trunk lesion. Although areas of focal pigmentation were observed, histopathological examination of the biopsy specimen showed a decrease in the pigmentation of the basal cell layer. Pigment incontinence was not observed in the dermis. A full blood count, routine urinalysis, liver and renal function tests, serum electrolyte levels and erythrocyte sedimentation rate were within normal limits. Upon consideration of these findings we diagnosed the patient as suffering from hypomelanosis of Ito (HI) with ileal atresia.