Incontinentia Pigmenti: A Case Report of a Complex Systemic Disease


  • Serena Gianfaldoni University G. Marconi of Rome, Dermatology and Venereology, Rome 00192
  • Georgi Tchernev Medical Institute of the Ministry of Interior, Dermatology, Venereology and Dermatologic Surgery; Onkoderma, Private Clinic for Dermatologic Surgery, Dermatology and Surgery, Sofia 1407
  • Uwe Wollina Krankenhaus Dresden-Friedrichstadt, Department of Dermatology and Venereology, Dresden, Sachsen
  • Torello Lotti Universitario di Ruolo, Dipartimento di Scienze Dermatologiche, Università degli Studi di Firenze, Facoltà di Medicina e Chirurgia, Dermatology, Via Vittoria Colonna 11, Rome 00186



Incontinentia Pigmenti, genodermatosis, NEMO, cutaneous manifestations, systemic disease


Incontinentia Pigmenti is an uncommon X-linked genodermatosis, caused by mutations in the NEMO gene. It is a systemic disease that involves tissue of ectodermic and mesodermic origin, including cutaneous tissue, teeth, eyes and the central nervous system, amongst other organs. The Authors report a rare case of Incontinentia Pigmenti in a female newborn.


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How to Cite

Gianfaldoni S, Tchernev G, Wollina U, Lotti T. Incontinentia Pigmenti: A Case Report of a Complex Systemic Disease. Open Access Maced J Med Sci [Internet]. 2017 Jul. 23 [cited 2023 Feb. 4];5(4):501-5. Available from:

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