Epidermolytic Hyperkeratosis (Bullous Congenital Ichthyosiform Erythroderma) Medication

  • Author: Tina S Chen, MD; Chief Editor: Dirk M Elston, MD   more...
 
Updated: May 11, 2012
 
 

Medication Summary

No reported cure or specific therapy is available for epidermolytic hyperkeratosis (EHK, bullous congenital ichthyosiform erythroderma [bullous CIE]); however, reports of improvement have been noted with high-dose beta-carotene, systemic retinoids, topical retinoids, 10% glycerin, lactic acid, alpha-hydroxy acid, calcipotriol, antibacterial soap, and urea.[21, 22, 23]

Improvement has been reported with topical retinoid therapy, although widespread application should be avoided because of the risk of systemic absorption. One patient had significant improvement in clinical disease on a combination therapy of acitretin 40 mg/d and erythromycin 500 mg twice daily, then tapered down to a dose of acitretin 30 mg/d and erythromycin 500 mg/d.[24]

Another patient had complete clearing of her lesions after 2 months of acitretin (35 mg/day; 0.5 mg/kg/day), but the lesions recurred 3 months after discontinuation of acitretin.[25] Acitretin at 25 mg/day may significantly improve a patient’s quality of life and skin condition.[26]

A patient with the mosaic-type disease was successfully treated with topical maxacalcitol, a vitamin D3 analogue.[27]

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Contributor Information and Disclosures
Author

Tina S Chen, MD  Pediatric Dermatology Fellow, Rady Children's Hospital, Department of Dermatology, University of California, San Diego

Tina S Chen, MD is a member of the following medical societies: American Academy of Dermatology, California Society of Dermatology and Dermatologic Surgery, and Society for Pediatric Dermatology

Disclosure: Nothing to disclose.

Coauthor(s)

Brandie J Metz, MD  Assistant Clinical Professor of Dermatology and Pediatrics, Chief of Pediatric Dermatology, University of California, Irvine, School of Medicine

Brandie J Metz, MD is a member of the following medical societies: American Academy of Dermatology, Society for Pediatric Dermatology, and Women's Dermatologic Society

Disclosure: Nothing to disclose.

Specialty Editor Board

Marjan Garmyn, MD, PhD  Professor, Faculty of Medicine, Katholieke Universiteit Leuven, Belgium; Chair and Adjunct Head, Department of Dermatology, University of Leuven, Belgium

Disclosure: Nothing to disclose.

Richard P Vinson, MD  Assistant Clinical Professor, Department of Dermatology, Texas Tech University Health Sciences Center, Paul L Foster 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.

Jeffrey J Miller, MD  Associate Professor of Dermatology, Pennsylvania State University College of Medicine; Staff Dermatologist, Pennsylvania State Milton S Hershey Medical Center

Jeffrey J Miller, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, Association of Professors of Dermatology, North American Hair Research Society, and Society for Investigative Dermatology

Disclosure: Nothing to disclose.

Joel M Gelfand, MD, MSCE  Medical Director, Clinical Studies Unit, Assistant Professor, Department of Dermatology, Associate Scholar, Center for Clinical Epidemiology and Biostatistics, University of Pennsylvania

Joel M Gelfand, MD, MSCE is a member of the following medical societies: Society for Investigative Dermatology

Disclosure: AMGEN Consulting fee Consulting; AMGEN Grant/research funds Investigator; Genentech Grant/research funds investigator; Centocor Consulting fee Consulting; Abbott Grant/research funds investigator; Abbott Consulting fee Consulting; Novartis investigator; Pfizer Grant/research funds investigator; Celgene Consulting fee DMC Chair; NIAMS and NHLBI Grant/research funds investigator

Chief Editor

Dirk M Elston, MD  Director, Ackerman Academy of Dermatopathology, New York

Dirk M Elston, MD is a member of the following medical societies: American Academy of Dermatology

Disclosure: Nothing to disclose.

Additional Contributors

The authors and editors of Medscape Reference gratefully acknowledge the contributions of previous authors, Sidney B. Smith, MD, and Jeffrey Smith, MD, to the development and writing of this article.

References

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The scale in epidermolytic hyperkeratosis is classically described as "corrugated cardboard"-like.
Palms and soles may have varying degrees of hyperkeratosis.
Anterior ankles.
Close-up view of ankle.
Pathology of epidermolytic hyperkeratosis (hematoxylin and eosin stain).
Pathology of epidermolytic hyperkeratosis (hematoxylin and eosin stain).
 
 
 
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