eMedicine Specialties > Dermatology > Pediatric Diseases

Dermatopathia Pigmentosa Reticularis: Treatment & Medication

Author: Noah S Scheinfeld, MD, JD, FAAD, Assistant Clinical Professor, Department of Dermatology, Columbia University; Consulting Staff, Department of Dermatology, St Luke's Roosevelt Hospital Center, Beth Israel Medical Center, New York Eye and Ear Infirmary; Private Practice
Coauthor(s): Hong-Duo Chen, MD, PhD, Director, Immunodermatological Key Laboratory, China; Professor, Chairman, Department of Dermatology, Number 1 Hospital, China Medical University
Contributor Information and Disclosures

Updated: Jan 7, 2010

Treatment

Medical Care

No specific treatment exists for dermatopathia pigmentosa reticularis (DPR), except for symptomatic management of some of the associated conditions, such as palmoplantar hyperkeratosis. For hyperkeratosis, topical retinoic acids and keratolytics may be beneficial. Nonscarring blisters are generally transient and self-healing. Cold compress may suffice.

Activity

Advise patients with dermatopathia pigmentosa reticularis to avoid sun exposure, which may trigger blister formation.

More on Dermatopathia Pigmentosa Reticularis

Overview: Dermatopathia Pigmentosa Reticularis
Differential Diagnoses & Workup: Dermatopathia Pigmentosa Reticularis
Treatment & Medication: Dermatopathia Pigmentosa Reticularis
Follow-up: Dermatopathia Pigmentosa Reticularis
References

References

  1. Bu TS, Kim YK, Whang KU. A case of dermatopathia pigmentosa reticularis. J Dermatol. Apr 1997;24(4):266-9. [Medline].

  2. Dereure O. [Naegeli-Franceschetti-Jadassohn syndrome and dermatopathia pigmentosa reticularis. Two allelic ectodermal dysplasias related to mutations of dominant gene coding for keratin 14]. Ann Dermatol Venereol. Jun-Jul 2007;134(6-7):595. [Medline].

  3. Heimer WL 2nd, Brauner G, James WD. Dermatopathia pigmentosa reticularis: a report of a family demonstrating autosomal dominant inheritance. J Am Acad Dermatol. Feb 1992;26(2 Pt 2):298-301. [Medline].

  4. Itin PH, Lautenschlager S, Meyer R, Mevorah B, Rufli T. Natural history of the Naegeli-Franceschetti-Jadassohn syndrome and further delineation of its clinical manifestations. J Am Acad Dermatol. Jun 1993;28(6):942-50. [Medline].

  5. Sprecher E, Itin P, Whittock NV, et al. Refined mapping of Naegeli-Franceschetti- Jadassohn syndrome to a 6 cM interval on chromosome 17q11.2-q21 and investigation of candidate genes. J Invest Dermatol. Sep 2002;119(3):692-8. [Medline].

  6. Whittock NV, Coleman CM, McLean WH, et al. The gene for Naegeli-Franceschetti-Jadassohn syndrome maps to 17q21. J Invest Dermatol. Oct 2000;115(4):694-8. [Medline].

  7. Goh BK, Common JE, Gan WH, Kumarasinghe P. A case of dermatopathia pigmentosa reticularis with wiry scalp hair and digital fibromatosis resulting from a recurrent KRT14 mutation. Clin Exp Dermatol. Apr 2009;34(3):340-3. [Medline].

  8. Brar BK, Mehta V, Kubba A. Dermatopathia pigmentosa reticularis. Pediatr Dermatol. Sep-Oct 2007;24(5):566-70. [Medline].

  9. Lugassy J, Itin P, Ishida-Yamamoto A, et al. Naegeli-Franceschetti-Jadassohn syndrome and dermatopathia pigmentosa reticularis: two allelic ectodermal dysplasias caused by dominant mutations in KRT14. Am J Hum Genet. Oct 2006;79(4):724-30. [Medline].

  10. Tunca M, Koc E, Akar A, Erbil AH, Tastan HB. Early-onset gastric carcinoma in a man with dermatopathia pigmentosa reticularis. Int J Dermatol. Jun 2008;47(6):641-3. [Medline].

  11. Fulk CS. Primary disorders of hyperpigmentation. J Am Acad Dermatol. Jan 1984;10(1):1-16. [Medline].

  12. Maso MJ, Schwartz RA, Lambert WC. Dermatopathia pigmentosa reticularis. Arch Dermatol. Jul 1990;126(7):935-9. [Medline].

  13. Rycroft RJ, Calnan CD, Allenby CF. Dermatopathia pigmentosa reticularis. Clin Exp Dermatol. Mar 1977;2(1):39-44. [Medline].

  14. Schnur RE, Heymann WR. Reticulate hyperpigmentation. Semin Cutan Med Surg. Mar 1997;16(1):72-80. [Medline].

  15. van der Lugt L. Dermatopathia pigmentosa reticularis hyperkeratotica et mutilans. Dermatologica. 1970;140(5):294-302. [Medline].

Further Reading

Keywords

dermatopathia pigmentosa reticularis, DPR, Naegeli-Franceschetti-Jadassohn syndrome, NFJ syndrome, NFJS

Contributor Information and Disclosures

Author

Noah S Scheinfeld, MD, JD, FAAD, Assistant Clinical Professor, Department of Dermatology, Columbia University; Consulting Staff, Department of Dermatology, St Luke's Roosevelt Hospital Center, Beth Israel Medical Center, New York Eye and Ear Infirmary; Private Practice
Noah S Scheinfeld, MD, JD, FAAD is a member of the following medical societies: American Academy of Dermatology
Disclosure: Optigenex Consulting fee Independent contractor

Coauthor(s)

Hong-Duo Chen, MD, PhD, Director, Immunodermatological Key Laboratory, China; Professor, Chairman, Department of Dermatology, Number 1 Hospital, China Medical University
Disclosure: Nothing to disclose.

Medical Editor

Jacek C Szepietowski, MD, PhD, Professor, Vice-Head, Department of Dermatology, Venereology and Allergology, Wroclaw Medical University; Director of the Institute of Immunology and Experimental Therapy, Polish Academy of Sciences, Poland
Disclosure: Stiefel Salary Employment; Orfagen Consulting fee Consulting; Maruho Consulting fee Consulting; Astellas Consulting fee Consulting

Pharmacy Editor

Michael J Wells, MD, Associate Professor, Department of Dermatology, Texas Tech University Health Sciences Center
Michael J Wells, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American Medical Association, and Texas Medical Association
Disclosure: Nothing to disclose.

Managing Editor

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.

CME Editor

Catherine M Quirk, MD, Clinical Assistant Professor, Department of Dermatology, University of Pennsylvania
Catherine M Quirk, MD is a member of the following medical societies: Alpha Omega Alpha and American Academy of Dermatology
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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