Incontinentia pigmenti

Incontinentia pigmenti (IP) is a rare X-linked dominant multi-systemic ectodermal dysplasia usually lethal in males and presenting neonatally in females with a bullous rash along Blashko's lines (BL) followed by verrucous plaques evolving over time to hyperpigmented swirling patterns. It is further characterized by teeth abnormalities, alopecia, nail dystrophy and affects occasionally the retina and the central nervous system (CNS).

Unusual infection

A type of infection that is regarded as a sign of a pathological susceptibility to infection. There are five general subtypes. (i) Opportunistic infection, meaning infection by a pathogen that is not normally able to cause infection in a healthy host (e.g., pneumonia by Pneumocystisjirovecii or CMV); (ii) Unusual location (focus) of an infection (e.g., an aspergillus brain abscess); (iii) a protracted course or lack of adequate response to treatment (e.g., chronic rhinosinusitis); (iv) Unusual severity or intensity of an infection; and (v) unusual recurrence of infections.


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PMID (PMCID)
3902309
FEMALE Child
Actinomycosis meningitis in a girl with incontinentia pigmenti.
Diamantopoulos N, Bergman I, Kaplan S.
Clin Pediatr (Phila). 1985;24(11):651-4.
Although all tests of immunologic function were normal in this patient, her history of recurrent and unusual infections is consistent with previous suggestions of an undefined immunodeficiency state associated with incontinentia pigmenti.