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Images in Clinical Practice

Indian Pediatrics 2003; 40:265-266

Ichthyosis Bullosa of Siemens


A 2-year-old male child was brought with patchy scaly lesions developing following blistering since one year of age. The lesions progressively became dark brown and hyperkeratotic but blistering became a rare event. Minor trivial trauma of footwear was still causing blisters over the feet. The mother was afflicted by the same disorder, however, other sibling was normal. The child had bilateral symmetrical dark brown ichthyotic scaling, localized mainly to body folds where it demonstrated typical rippled pattern (Fig. 1) and over the abdomen (Fig. 2) and acral sites, where circumscribed areas of focal peeling of skin were seen. The scalp, face, palms and soles were spared. There were no abnormalities of hair, nails and mucous membranes. A skin biopsy was suggestive of epidermolytic hyperkeratosis.

Fig. 1. Axilla showing dark brown hyperkeratosis and rippled pattern. Fig. 2. Circumscribed dark gray hyperkeratosis in flexural sites and localized peeling of the skin over the abdomen

The clinical and histological features suggested the diagnosis of ichthyosis bullosa of Siemens and the patient was treated with keratolytics topically.

Ichthyosis bullosa of Siemens (IBS) is a rare autosomal dominant disorder, distinct from bullous congenital ichthyosiform ery-throderma (BCIE) or epidermolytic hyper-keratosis. Circumscribed dark gray hyper-keratosis in flexural sites and localized peeling of the skin are its characteristic features. This latter feature, due to intracorneal blistering has been termed as ‘Mauserung’ or ‘Moulting effect’. Overall, the clinical features are less marked than those of BCIE with relatively mild hyperkeratosis usually limited to the skin flexures. Though, hyperkeratotic component of ichthyosis responds to systemic etretinate but blistering is the real problem, which continue to occur. Acitretin therapy for children with inherited keratinization disorders has been found to be safe and effective, provided the minimal effective dose is maintained and side effects are carefully monitored. Topically kerato-lytics and emollients do help such patients.

Devinder Mohan Thappa,
B Jeevankumar,

Department of Dermatology and STD,
Jawaharlal Institute of Postgraduate Medical
Education and Research (JIPMER),
Pondicherry 605 006, India.




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