Aplasia cutis associated with coarctation of the aorta: could this be an incomplete form of Adams?Oliver syndrome?

Abstract

cold urticaria 3,4 and is mentioned as a treatment to be considered in the most recent guidelines for the management of urticaria. 5 However, the risk of anaphylactic shock should be considered. This is confirmed by the data presented here where all patients treated with cold water baths experienced a distinct lowering of their temperature threshold. However, no patient was able to continue the treatment for more than several weeks or months due to a lack of motivation and sideeffects which apparently were rated higher than the impact of the disease on their lives.Cold baths remain an effective treatment of cold urticaria only for patients bothered by severe manifestation of their disease who are well informed about the procedure and risks and are prepared to comply with this time-consuming and subjectively unpleasant treatment. It may be performed as a single treatment or in combination with other therapeutic options.

Aplasia cutis congenita: approach to evaluation and management

“…Congenital heart defects have been reported in approximately 20% of AOS patients. Findings may include membranous subaortic stenosis, aortic stenosis, parachute mitral valve, ventricular septal defect, atrial septal defect, tetralogy of Fallot, coarctation of the aorta, bicuspid aortic valve, pulmonic stenosis, double outlet right ventricle, and pulmonary hypertension .…”

Aplasia cutis congenita associated with coarctation of the aorta: case report and review of the literature

“…It is often associated with a variety of abnormalities, such as omphalocele, 3 absence of distal limbs, 4 and craniofacial deformities 5 . In the literature, only a few case reports have described the association of ACC with cardiovascular malformation 6–9 …”

Cardiovascular malformations in Adams–Oliver syndrome

“…On one hand, literature review shows intra‐familial variability, including co‐segregation of classic and partial AOS in a few families [Santos et al, ; Lin et al, ]. Notably, some members displayed full blown features of AOS, while others had CVMs and aplasia cutis without limb defects [Bruel et al, ; Heras Mulero et al, ; Digilio et al, ]. On the other hand, concordant partial AOS can also segregate in the same family, as illustrated by a mother–son pair sharing aplasia cutis congenital and aortic coarctation with normal limbs [Dallapiccola et al, ; Digilio et al, ].…”