eMedicine Specialties > Dermatology > Pediatric Diseases

Ichthyosis, X-Linked: Treatment & Medication

Author: Camila K Janniger, MD, Clinical Professor of Dermatology, Clinical Associate Professor of Pediatrics, Chief of Pediatric Dermatology, New Jersey Medical School
Coauthor(s): Robert A Schwartz, MD, MPH, Professor and Head, Dermatology, Professor of Pathology, Pediatrics, Medicine, and Preventive Medicine and Community Health, UMDNJ-New Jersey Medical School
Contributor Information and Disclosures

Updated: May 29, 2009

Treatment

Medical Care

  • Topical keratolytics, emollients, and hydrating agents are used to reduce scaling associated with X-linked ichthyosis (XLI).
  • Topical isotretinoin may be beneficial.
  • In a small study, the topical receptor-selective retinoid tazarotene was efficacious.
  • Patients often choose to use no therapy, although appearance-conscious adolescents and young adults may be eager and willing to treat themselves.

Surgical Care

In cases with cryptorchidism, consider surgical intervention if spontaneous descent has not occurred by age 1 year.

Consultations

  • An ophthalmologist may detect corneal opacities.
  • An obstetrician should be involved for higher risk delivery in future pregnancies.

Medication

The goal of pharmacotherapy is the reduction of retained scales.

Keratolytics

Alpha hydroxy acids are effective in the treatment of X-linked ichthyosis.


Ammonium lactate (Lac-Hydrin)

Contains lactic acid, an alpha hydroxy acid that has keratolytic action thus facilitating release of comedones. Available in 12% and 5% strengths. The 12% form may cause irritation on the face. Causes disadhesion of corneocytes.

Adult

Apply bid to affected areas

Pediatric

Not established

Pregnancy

C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus

Precautions

May cause stinging and burning at application site; avoid contact with eyes, lips, or mucous membranes; concurrent use of sunscreen recommended for sun-exposed areas also treated with these products

More on Ichthyosis, X-Linked

Overview: Ichthyosis, X-Linked
Differential Diagnoses & Workup: Ichthyosis, X-Linked
Treatment & Medication: Ichthyosis, X-Linked
Follow-up: Ichthyosis, X-Linked
Multimedia: Ichthyosis, X-Linked
References
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References

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  14. Schlotawa L, Steinfeld R, von Figura K, Dierks T, Gartner J. Molecular analysis of SUMF1 mutations: stability and residual activity of mutant formylglycine-generating enzyme determine disease severity in multiple sulfatase deficiency. Hum Mutat. Jan 2008;29(1):205. [Medline].

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  34. Zalel Y, Kedar I, Tepper R, et al. Differential diagnosis and management of very low second trimester maternal serum unconjugated estriol levels, with special emphasis on the diagnosis of X-linked ichthyosis. Obstet Gynecol Surv. Mar 1996;51(3):200-3. [Medline].

  35. Zettersten E, Man MQ, Sato J, et al. Recessive x-linked ichthyosis: role of cholesterol-sulfate accumulation in the barrier abnormality. J Invest Dermatol. Nov 1998;111(5):784-90. [Medline].

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Further Reading

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Keywords

ichthyosis nigricans, X-linked ichthyosis, enzyme deficiency, XLI

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Contributor Information and Disclosures

Author

Camila K Janniger, MD, Clinical Professor of Dermatology, Clinical Associate Professor of Pediatrics, Chief of Pediatric Dermatology, New Jersey Medical School
Camila K Janniger, MD is a member of the following medical societies: American Academy of Dermatology
Disclosure: Nothing to disclose.

Coauthor(s)

Robert A Schwartz, MD, MPH, Professor and Head, Dermatology, Professor of Pathology, Pediatrics, Medicine, and Preventive Medicine and Community Health, UMDNJ-New Jersey Medical School
Robert A Schwartz, MD, MPH is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American College of Physicians, and Sigma Xi
Disclosure: Nothing to disclose.

Medical Editor

Bernice R Krafchik, MBChB, FRCPC, Professor Emeritus, Department of Pediatrics, Section of Dermatology, University of Toronto
Bernice R Krafchik, MBChB, FRCPC is a member of the following medical societies: American Academy of Dermatology, American Dermatological Association, Canadian Medical Association, College of Physicians and Surgeons of Ontario, Royal College of Physicians and Surgeons of Canada, and Society for Pediatric Dermatology
Disclosure: Nothing to disclose.

Pharmacy Editor

Richard P Vinson, MD, Assistant Clinical Professor, Department of Dermatology, Texas Tech University School of Medicine; Consulting Staff, Mountain View Dermatology, PA
Richard P Vinson, MD is a member of the following medical societies: American Academy of Dermatology, Association of Military Dermatologists, Texas Dermatological Society, and Texas Medical Association
Disclosure: Nothing to disclose.

Managing Editor

Van Perry, MD, Assistant Professor, Department of Medicine, Division of Dermatology, University of Texas Health Science Center
Van Perry, MD is a member of the following medical societies: American Academy of Dermatology and American Society for Laser Medicine and Surgery
Disclosure: Nothing to disclose.

CME Editor

Glen H Crawford, MD, Assistant Clinical Professor, Department of Dermatology, University of Pennsylvania School of Medicine; Chief, Division of Dermatology, The Pennsylvania Hospital
Glen H Crawford, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American Medical Association, Phi Beta Kappa, and Society of USAF Flight Surgeons
Disclosure: Nothing to disclose.

Chief Editor

Dirk M Elston, MD, Director, Department of Dermatology, Geisinger Medical Center
Dirk M Elston, MD is a member of the following medical societies: American Academy of Dermatology
Disclosure: Nothing to disclose.

 
 
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