Clinical study of 20 patients with incontinentia pigmenti

Abstract

These findings reinforce the heterogeneity of dermatologic findings and the numerous extracutaneous manifestations requiring a multidisciplinary approach. The follow-up of patients with IP is important in the detection of serious associated diseases. The relationships between these disorders and IP raise the need for additional longitudinal studies with longterm monitoring of these patients. The management of IP in clinical practice may benefit from early efforts to detect associated diseases.

Questionnaire survey on association between preeclampsia and incontinentia pigmenti

“…Schematic representation of relationship between preeclampsia, incontinentia pigmenti and NEMO/IKBKG gene. : * : This article; Ref …”

“…Defects in leukocyte chemotaxis, lymphocyte defects and defective immune tolerance have been reported in association with IP . Inflammatory and autoimmune diseases such as SLE, Behçet's disease，still's disease overlapping with macrophage‐activation syndrome (MAS), myasthenia gravis, atypical enterocolitis, and Wilms’ tumor have been reported in patients with IP …”

Orthodontic Treatment for Bloch-Sulzberger Syndrome in Patient with Cleft Lip and Palate

“…This condition has an Case Report doi:10.2209/tdcpublication.2016-0051 X-linked-dominant pattern of inheritance, and is extremely rare in men 11) . It usually develops as a result of a mutation that deactivates the IKKγ (IκB kinase γ) gene (also referred to as the NEMO, or NFκB essential modulator gene), which is located on region 28 of the long arm of the X chromosome (Xq28) 15) .…”

“…Rashes develop frequently all over the body, followed by grayish-brown pigmented spots in the same areas. Thereafter, the pigmented spots gradually disappear, and the prognosis is good 11) . Tooth abnormalities include hypodontia, delayed eruption, pegshaped teeth, impacted teeth, morphological anomalies, and malocclusion 6,8) .…”

“…Tooth abnormalities include hypodontia, delayed eruption, pegshaped teeth, impacted teeth, morphological anomalies, and malocclusion 6,8) . In addition to reports on skin symptoms associated with this syndrome 11) , a number of studies have also described associated dental features 2,4,6,7,9,13) and corresponding orthodontic treatment 1,3,10,14,16) . Reports on orthodontic treatment for this syndrome when accompanied by cleft lip and palate are particularly limited, however, and, to our knowledge, there are none on the subsequent long-term course of such treatment.…”