A Sporadic Case of Ichthyosis Curth Macklin: Rare Presentation of a Rare Disease.

Indian J Dermatol 2015 Sep-Oct;60(5):522

Department of Dermatology, SMS Medical College, Jaipur, Rajasthan, India.

Ichthyosis hystrix is a rare autosomal dominant genodermatosis, characterized by persistent spiny hyperkeratotic scales which cover a significant part of the skin surface. Based on the pattern of distribution, five clinical variants namely Brocq type, Lambert type, Curth-Macklin type, Rheydt type and Bδfverstedt type have been described. We report the case of an 11-year-old male child with spiny, hyperkeratotic scales all over the body since birth with sparing of scalp and central part of the face. Palmoplantar keratoderma was also present. These clinical features are suggestive of Ichthyosis Curth Macklin, which has been typically described in families. However, family history was negative in our patient. The case is being reported on account of rarity of the disease, that too with a very rare sporadic presentation.

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Source
http://dx.doi.org/10.4103/0019-5154.164439DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4601452PMC
November 2015
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