Skip to main content Skip to main navigation menu Skip to site footer

Incontinentia pigmenti in neonate: a case report

  • Putu Gde Hari Wangsa ,
  • Luh Made Mas Rusyati ,
  • I Gusti Ayu Agung Dwi Karmila ,
  • Ni Luh Putu Ratih Vibriyanti Karna ,
  • Ketut Wida Komalasari ,
  • Made Hermina Laksmi ,


Background: Incontinentia pigmenti (IP) is a rare X-linked dominant inherited genodermatosis that occurs almost in females and is usually accompanied by other ectodermal tissue diseases such as the central nervous system, eyes, hair, nails, teeth and skeletal system. This case report aims to enhance understanding of incontinentia pigmenti and provide appropriate treatment to patients and proper education to families.

Case report: A 3-days-od baby girl was consulted by the Pediatric department with complaints of blisters and erythematous rashes on almost her entire body with the Blaschko line distribution. The nails on the right and left big toes appeared inward. No history of fever and seizure. No family had similar complaints. There were no eye and nerve abnormalities involved. She treated with hydrocortisone 1% cream every 12 hours on erythematous papules and vesicles, open compresses with 0.9% NaCl every 8 hours for 10-15 minutes on lesions with yellowish crusts. Thus, the patient diagnosed with incontinentia pigmenti.

Conclusion: The diagnosis of incontinentia pigmenti is based on history and physical examination. This case is very rare, so the family needs to understand the course of this disease. Appropriate management and education can prevent secondary infection.


  1. Poziomczyk CS, Maria FDS, Freitas AM, Fiegenbaum M, Bau AEK, Recuero JK, et al. Incontinentia pigmenti. An Bras Dermatol. 2014;89(1):26–36.
  2. Berlin AL, Paller AS, Chan LS. Incontinentia pigmenti: A review and update on the molecular basis of pathophysiology. J Am Acad Dermatol. 2002;47(2):169–90.
  3. Carney R. Incontinentia pigmenti a world statistical analysis. Arch Dermatol. 1976;112:535–42.
  4. Anonim. Register Poliklinik Kulit dan Kelamin Divisi Dermatologi Anak Rumah Sakit Umum Pusat Sanglah Januari 2019 - Januari 2021. RSUP Sanglah Denpasar; 2021.
  5. Nieman EL, Grange DK. Ectodermal Dysplasia. In: Goldsmith L, Katz S, Gilchrest B, Paller A, Leffel D, Wolff K, editors. Fitzpatrick’s Dermatology in General Medicine 9th ed. New York: Mc Graw Hill Education; 2019. p. 2386–2338.
  6. Pereira M a C, Mesquita L a DF, Budel AR, Cabral CSP, Feltrim ADS. X-linked incontinentia pigmenti or Bloch-Sulzberger syndrome: a case report. An Bras Dermatol. 1928;85(3):372–5.
  7. van Leeuwen R, Wintzen M, van Praag M. Incontinentia pigmenti: an extensive second episode of a “first-stage” vesicobullous eruption. Pediatr Dermatol. 2000;17(1):70–2.
  8. De Argila D, Rivera R, Guerra A, Iglesias L. Incontinentia pigmenti: a case with an unusual course. Pediatr Dermatol. 1993;13:434–5.
  9. Bodak N, Hadj-Rabia S, Hamel-Teillac D, de Prost Y, Bodemer C. Late Recurrence of Inflammatory First-Stage Lesions in Incontinentia Pigmenti. Arch Dermatol. 2003;139(2):5–8.
  10. Hadj-Rabia S, Froidevaux D, Bodak N, Hamel-Teillac D, Smahi A, Touil Y, et al. Clinical study of 40 cases of incontinentia pigmenti. Arch Dermatol. 2003;139(9):1163–70.
  11. Montes CM, Maize JC, Guerry-Force ML. Incontinentia pigmenti with painful subungual tumors: A two-generation study. J Am Acad Dermatol. 2004;50(2 Suppl.):45–52.
  12. Shotts N, Emery E. Bloch-Sulzberger syndrome (incontinentia pigmenti). J Med Genet. 1966;3:148–52.
  13. Mini? S, Trpinac D, Obradovi? M. Incontinentia pigmenti diagnostic criteria update. Clin Genet. 2014;85(6):536–42.
  14. Kaya TI, Tursen U, Ikizoglu G. Therapeutic use of topical corticosteroids in the vesiculobullous lesions of incontinentia pigmenti. Clin Exp Dermatol. 2009;34(8):e611–3.
  15. Sybert V. Guide to Information for Families with Inherited Skin Disorders. Pediatr Dermatol. 1990;7(3):214–7.

How to Cite

Wangsa, P. G. H., Rusyati, L. M. M., Karmila, I. G. A. A. D., Karna, N. L. P. R. V., Komalasari, K. W., & Laksmi, M. H. (2022). Incontinentia pigmenti in neonate: a case report. Intisari Sains Medis, 13(1), 221–223.




Search Panel

Putu Gde Hari Wangsa
Google Scholar
ISM Journal

Luh Made Mas Rusyati
Google Scholar
ISM Journal

I Gusti Ayu Agung Dwi Karmila
Google Scholar
ISM Journal

Ni Luh Putu Ratih Vibriyanti Karna
Google Scholar
ISM Journal

Ketut Wida Komalasari
Google Scholar
ISM Journal

Made Hermina Laksmi
Google Scholar
ISM Journal