The inherited congenital ichthyoses are a heterogeneous group of disorders marked by the common finding of scaly skin. Clinically they are characterised by a wide range of severity, from isolated skin involvement to multi-organ disease.
Acquired ichthyosis develops later in life and can be associated with multiple underlying conditions or medications.
The work-up, diagnosis, and treatment vary based on the type of ichthyosis that is suspected by the treating physician.
Ichthyosis is a descriptive term for dry, scaly skin that in some cases may resemble the scales on a fish (ichthys is the Greek root for fish). The inherited ichthyoses are a group of diseases that feature scaling skin as part of their clinical presentation. The underlying defect, mode of inheritance, character, and extent of scaling differ among the various types. Because of these differences, work-up and treatment varies depending on the clinical presentation. Ichthyosis can also be acquired as a result of underlying diseases or use of certain medications.
History and exam
- hx of atopy
- hx of lymphoma, diabetes, systemic lupus erythematosus
- use of clofazimine, lipid-lowering agents, or butyrophenone
- alopecia and ectropion in lamellar ichthyosis
- cryptorchidism and corneal opacities
- deafness, mental retardation, limb defects, epilepsy, short stature
Assistant Professor of Dermatology and Pediatrics
BC declares that she is a member of the Foundation for Ichthyosis and Related Skin Types Advisory Board. BC is an author of a reference cited in this monograph.
Dr Brittany Craiglow would like to gratefully acknowledge Dr Timothy Patton, a previous contributor to this monograph. TP declares that he has no competing interests.
Department of Pathology and Laboratory Medicine
University of California
DC declares that he has no competing interests.
Department of Dermatology
Erasme University Hospital
TS declares that he has no competing interests.
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