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).

Asthma

Asthma is characterized by increased responsiveness of the tracheobronchial tree to multiple stimuli, leading to narrowing of the air passages with resultant dyspnea, cough, and wheezing.


Total: 2

                      


(per page)
PMID (PMCID)
23460164
FEMALE Adult
Asthma associated with incontinentia pigmenti and Fanconi anemia: variable airflow limitation without cellular bronchitis.
Dasgupta A, Sanaee MS, Bauer CMT, Botelho FM, Arnold DM, Stampfli MR, Nair P.
Chest. 2013;143(3):856-858.
We describe the clinical history of a 34-year-old woman with airway hyperresponsiveness and asthma who had a reduced ability to mount an inflammatory response due to two unrelated and rare genetic conditions: Fanconi anemia and incontinentia pigmenti.
23460164
FEMALE Adult
Asthma associated with incontinentia pigmenti and Fanconi anemia: variable airflow limitation without cellular bronchitis.
Dasgupta A, Sanaee MS, Bauer CMT, Botelho FM, Arnold DM, Stampfli MR, Nair P.
Chest. 2013;143(3):856-858.
Asthma associated with incontinentia pigmenti and Fanconi anemia: variable airflow limitation without cellular bronchitis.