Ichthyosis hystrix gravior, type lambert Q80.8
Synonym(s)
Porcupine People
Definition
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Autosomal dominant inherited spiky hyperkeratoses covering the entire body with the exception of the face, genitals, palmae and plantae. Only a few cases have been described so far.
External therapy
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Experimental Tazarotene locally. It should be noted that no more than about 2% of the skin surface should be treated simultaneously. S.u. Ichthyosis vulgaris, autosomal dominant.
Internal therapy
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No specific therapy known. Therapy trial with Acitretin (Neotigason) possible.
Disclaimer
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