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Dermatopathia Pigmentosa Reticularis Follow-up

  • Author: Noah S Scheinfeld, JD, MD, FAAD; Chief Editor: Dirk M Elston, MD  more...
 
Updated: Jan 19, 2016
 

Deterrence/Prevention

Advise patients with dermatopathia pigmentosa reticularis (DPR) to avoid sun exposure and minor trauma, which may trigger blister formation.

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Complications

No serious complications are known for dermatopathia pigmentosa reticularis. Some conditions mentioned in the literature are probably coincidental rather than complications.

Early-onset gastric carcinoma in a male with dermatopathia pigmentosa reticularis was noted by Tunca et al in 2008.[21]

Goh et al reported a case of dermatopathia pigmentosa reticularis caused by a recurrent KRT14 mutation in which the patient had digital fibromatosis and wiry scalp hair.[15]

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Prognosis

The hyperpigmentation associated with dermatopathia pigmentosa reticularis persists throughout life, showing no tendency of spontaneous fading.

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Contributor Information and Disclosures
Author

Noah S Scheinfeld, JD, MD, FAAD Assistant Clinical Professor, Department of Dermatology, Weil Cornell Medical College; Consulting Staff, Department of Dermatology, St Luke's Roosevelt Hospital Center, Beth Israel Medical Center, New York Eye and Ear Infirmary; Assistant Attending Dermatologist, New York Presbyterian Hospital; Assistant Attending Dermatologist, Lenox Hill Hospital, North Shore-LIJ Health System; Private Practice

Noah S Scheinfeld, JD, MD, FAAD is a member of the following medical societies: American Academy of Dermatology

Disclosure: Serve(d) as a speaker or a member of a speakers bureau for: Abbvie<br/>Received income in an amount equal to or greater than $250 from: Optigenex<br/>Received salary from Optigenex for employment.

Coauthor(s)

Hong-Duo Chen, MD Director, Immunodermatological Key Laboratory; Professor, Department of Dermatology, Number 1 Hospital, China Medical University, China

Disclosure: Nothing to disclose.

Specialty Editor Board

Michael J Wells, MD, FAAD Associate Professor, Department of Dermatology, Texas Tech University Health Sciences Center, Paul L Foster School of Medicine

Michael J Wells, MD, FAAD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American Medical Association, Texas Medical Association

Disclosure: Nothing to disclose.

Robert A Schwartz, MD, MPH Professor and Head of Dermatology, Professor of Pathology, Pediatrics, Medicine, and Preventive Medicine and Community Health, Rutgers New Jersey Medical School; Visiting Professor, Rutgers University School of Public Affairs and Administration

Robert A Schwartz, MD, MPH is a member of the following medical societies: Alpha Omega Alpha, New York Academy of Medicine, American Academy of Dermatology, American College of Physicians, Sigma Xi

Disclosure: Nothing to disclose.

Chief Editor

Dirk M Elston, MD Professor and Chairman, Department of Dermatology and Dermatologic Surgery, Medical University of South Carolina College of Medicine

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

Disclosure: Nothing to disclose.

Additional Contributors

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: Received consulting fee from Orfagen for consulting; Received consulting fee from Maruho for consulting; Received consulting fee from Astellas for consulting; Received consulting fee from Abbott for consulting; Received consulting fee from Leo Pharma for consulting; Received consulting fee from Biogenoma for consulting; Received honoraria from Janssen for speaking and teaching; Received honoraria from Medac for speaking and teaching; Received consulting fee from Dignity Sciences for consulting; .

References
  1. Bu TS, Kim YK, Whang KU. A case of dermatopathia pigmentosa reticularis. J Dermatol. 1997 Apr. 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. 2007 Jun-Jul. 134(6-7):595. [Medline].

  3. Oldak M, Kowalewski C, Maksym RB, et al. Novel keratin 14 hotspot mutation in Dowling-Meara type of epidermolysis bullosa simplex: strategy to avoid KRT14 pseudogene amplification by a simple approach. J Dermatol Sci. 2010 Jan. 57(1):69-70. [Medline].

  4. Liovic M, D'Alessandro M, Tomic-Canic M, Bolshakov VN, Coats SE, Lane EB. Severe keratin 5 and 14 mutations induce down-regulation of junction proteins in keratinocytes. Exp Cell Res. 2009 Oct 15. 315(17):2995-3003. [Medline].

  5. van Steensel MA, Lemmink HH. A missense mutation in KRT14 causing a dermatopathia pigmentosa reticularis/Naegeli-Franceschetti-Jadassohn phenotype. J Eur Acad Dermatol Venereol. 2010 Feb 17. [Medline].

  6. van Steensel MA, Lemmink HH. A missense mutation in KRT14 causing a dermatopathia pigmentosa reticularis/Naegeli-Franceschetti-Jadassohn phenotype. J Eur Acad Dermatol Venereol. 2010 Sep. 24(9):1116-7. [Medline].

  7. Shah BJ, Jagati AK, Gupta NP, Dhamale SS. Naegeli-Franceschetti-Jadassohn syndrome: A rare case. Indian Dermatol Online J. 2015 Nov-Dec. 6 (6):403-6. [Medline].

  8. Titeux M, Décha A, Pironon N, Tonasso L, et al. A new case of keratin 14 functional knockout causes severe recessive EBS and questions the haploinsufficiency model of Naegeli-Franceschetti-Jadassohn syndrome. J Invest Dermatol. 2011 Oct. 131(10):2131-3. [Medline].

  9. Cai BH, Hsu PC, Hsin IL, et al. p53 acts as a co-repressor to regulate keratin 14 expression during epidermal cell differentiation. PLoS One. 2012. 7(7):e41742. [Medline]. [Full Text].

  10. Cai BH, Chao CF, Lu MH, Lin HC, Chen JY. A half-site of the p53-binding site on the keratin 14 promoter is specifically activated by p63. J Biochem. 2012 Jul. 152(1):99-110. [Medline].

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

  12. 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. 1993 Jun. 28(6):942-50. [Medline].

  13. 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. 2002 Sep. 119(3):692-8. [Medline].

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

  15. 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. 2009 Apr. 34(3):340-3. [Medline].

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

  17. Shanker V, Gupta M. Dermatopathia pigmentosa reticularis: A rare reticulate pigmentary disorder. Indian Dermatol Online J. 2013 Jan. 4(1):40-2. [Medline]. [Full Text].

  18. Gallouj S, Benchat L, Sekal S, Harmouch T, Amarti A, Mernissi FZ. Dermatopathia pigmentosa reticularis. Ann Dermatol Venereol. 2014 Apr. 141(4):322-4. [Medline].

  19. Belligni EF, Dokal I, Hennekam RC. Prenatal and postnatal growth retardation, microcephaly, developmental delay, and pigmentation abnormalities: Naegeli syndrome, dyskeratosis congenita, poikiloderma Clericuzio type, or separate entity?. Eur J Med Genet. 2011 May-Jun. 54(3):231-5. [Medline].

  20. 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. 2006 Oct. 79(4):724-30. [Medline].

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

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