title: A Sporadic Case of Ichthyosis Curth Macklin: Rare Presentation of a Rare Disease.
abstract: 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.
authors.affiliation: Department of Dermatology, SMS Medical College, Jaipur, Rajasthan, India.
authors.forename: Sharad
authors.initials: S
authors.lastname: Mehta
authors.affiliation: Department of Dermatology, SMS Medical College, Jaipur, Rajasthan, India.
authors.forename: Uma Shankar
authors.initials: US
authors.lastname: Agarwal
authors.affiliation: Department of Dermatology, SMS Medical College, Jaipur, Rajasthan, India.
authors.forename: Nidheesh
authors.initials: N
authors.lastname: Agarwal
keywords: Atypical; ichthyosis hystrix; porcupine; sporadic