Ichthyosis Linearis Circumflexa
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Ichthyosis linearis circumflexa is a distinctive skin condition of generalized
hyperkeratosis Hyperkeratosis is thickening of the stratum corneum (the outermost layer of the epidermis, or skin), often associated with the presence of an abnormal quantity of keratin,Kumar, Vinay; Fausto, Nelso; Abbas, Abul (2004) ''Robbins & Cotran Pathologi ...
and polycyclic and
serpiginous Serpiginous, first known to be used in the 15th century, is a term from Latin serpere to creep, usually referring to a creeping, snakelike or slowly progressive skin disease. It is used to describe the rash in cutaneous larvae migrans, erythema an ...
erythema Erythema (from the Greek , meaning red) is redness of the skin or mucous membranes, caused by hyperemia (increased blood flow) in superficial capillaries. It occurs with any skin injury, infection, or inflammation. Examples of erythema not asso ...
tous plaques with a characteristic, migratory, double-edged scale at the margins, and is the typical cutaneous manifestation of
Netherton's syndrome Netherton syndrome is a severe, autosomal recessive form of ichthyosis associated with mutations in the ''SPINK5'' gene. It is named after Earl W. Netherton (1910–1985), an American dermatologist who discovered it in 1958.Netherton, E. W. A u ...
.Freedberg, et al. (2003). ''Fitzpatrick's Dermatology in General Medicine''. (6th ed.). McGraw-Hill. .James, William; Berger, Timothy; Elston, Dirk (2005). ''Andrews' Diseases of the Skin: Clinical Dermatology''. (10th ed.). Saunders. .


See also

* Ichthyosis prematurity syndrome * List of cutaneous conditions


References

Genodermatoses {{Genodermatoses-stub