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Ichthyosis Follow-up

  • Author: Andrew A Dahl, MD, FACS; Chief Editor: Hampton Roy, Sr, MD  more...
 
Updated: Jul 07, 2016
 

Further Outpatient Care

The mainstay of ichthyosis therapy includes removal of surface scales, and application of a water barrier.

In disabling cases, oral retinoids may reduce cosmetic disfigurement, depression, and social isolation.

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Complications

Because skeletal hyperostosis and arthralgia may occur with long-term oral etretinate and isotretinoin use, this form of treatment is reserved only for those patients with very severe scaling and cosmetic deformity. The clinical adverse effects of oral liarozole are reminiscent of those with oral retinoids; therefore, precaution is warranted with long-term use. Topical dermatologic retinoid preparations are irritating to the conjunctival fornices; therefore, it should not be applied directly to the eye. Topical tazarotene 0.1% gel may be an effective alternative to oral retinoids, with a decreased risk of systemic complications.

In the hyperproliferative variants of ichthyosis, topical calcipotriene ointment has been shown to be beneficial. However, the use of calcipotriene in treating congenital hyperproliferative disorders is limited by the theoretical risk of hypercalcemia from absorption of the drug from the skin.

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Prognosis

The dryness of the eyes can be treated with artificial tears, ointments, bandage contact lenses, punctal occlusion, and possibly surgery, depending on the presence of abnormal lid closure or limbal stem cell deficiency.

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Patient Education

Patients must realize that this condition is chronic, and they will need long-term therapy. Without long-term therapy, the defective permeability barrier associated with ichthyosis can result in a chronic loss of water and calories, which may impair growth in children.

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Contributor Information and Disclosures
Author

Andrew A Dahl, MD, FACS Assistant Professor of Surgery (Ophthalmology), New York College of Medicine (NYCOM); Director of Residency Ophthalmology Training, The Institute for Family Health and Mid-Hudson Family Practice Residency Program; Staff Ophthalmologist, Telluride Medical Center

Andrew A Dahl, MD, FACS is a member of the following medical societies: American Academy of Ophthalmology, American College of Surgeons, American Intraocular Lens Society, American Medical Association, American Society of Cataract and Refractive Surgery, Contact Lens Association of Ophthalmologists, Medical Society of the State of New York, New York State Ophthalmological Society, Outpatient Ophthalmic Surgery Society

Disclosure: Nothing to disclose.

Specialty Editor Board

Francisco Talavera, PharmD, PhD Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Received salary from Medscape for employment. for: Medscape.

Steven R Feldman, MD, PhD Professor, Departments of Dermatology, Pathology and Public Health Sciences, and Molecular Medicine and Translational Science, Wake Forest Baptist Health; Director, Center for Dermatology Research, Director of Industry Relations, Department of Dermatology, Wake Forest University School of Medicine

Steven R Feldman, MD, PhD is a member of the following medical societies: American Academy of Dermatology, American Society of Dermatopathology, North Carolina Medical Society, Society for Investigative Dermatology

Disclosure: Received honoraria from Amgen for consulting; Received honoraria from Abbvie for consulting; Received honoraria from Galderma for speaking and teaching; Received consulting fee from Lilly for consulting; Received ownership interest from www.DrScore.com for management position; Received ownership interest from Causa Reseasrch for management position; Received grant/research funds from Janssen for consulting; Received honoraria from Pfizer for speaking and teaching; Received consulting fee from No.

Chief Editor

Hampton Roy, Sr, MD Associate Clinical Professor, Department of Ophthalmology, University of Arkansas for Medical Sciences

Hampton Roy, Sr, MD is a member of the following medical societies: American Academy of Ophthalmology, American College of Surgeons, Pan-American Association of Ophthalmology

Disclosure: Nothing to disclose.

Additional Contributors

Kenneth M Goins, MD Professor of Clinical Ophthalmology, Director of Cornea and External Diseases and Refractive Surgery, Department of Ophthalmology, University of Iowa Hospitals and Clinics; Medical Director of Iowa Lions Eye Bank

Kenneth M Goins, MD is a member of the following medical societies: American Society of Cataract and Refractive Surgery, Eye Bank Association of America, International Society of Refractive Surgery, Iowa Medical Society, American Academy of Ophthalmology, American Medical Association

Disclosure: Nothing to disclose.

Seth Adam Anderson Des Moines University College of Osteopathic Medicine

Disclosure: Nothing to disclose.

Matthew Rauen, MD Fellow in Corneal, External Diseases and Refractive Surgery, Department of Ophthalmology and Visual Sciences, University of Iowa Health Care

Matthew Rauen, MD is a member of the following medical societies: American Academy of Ophthalmology

Disclosure: Nothing to disclose.

Arash Taheri, MD Research Fellow, Center for Dermatology Research, Department of Dermatology, Wake Forest University School of Medicine

Disclosure: Nothing to disclose.

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This direct illumination slit lamp photograph discloses a reticular central corneal haze that is seen bilaterally. Visual acuity is 20/20 in both eyes. The patient's chief complaint is photophobia and dry scaly skin.
This slit beam illumination photograph of the cornea localizes the corneal opacity to the posterior stroma and the pre-Descemet membrane region. This type of corneal opacity is commonly present in X-linked recessive ichthyosis.
An orbital mass is shown on CT scan in a 38-year-old male with human immunodeficiency virus (HIV) who presented with a late onset generalized ichthyotic rash and proptosis.
 
 
 
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