Here, we present two cases of aplasia cutis congenita, which represent two different types of the disease. The first case presented as non-syndromic aplasia cutis congenita of scalp, and the other case presented as a part of Bart's syndrome. [mjdrdypu.org]

Dermis, if present, is thin and lacks skin appendages. The lesion is usually small, measuring roughly 0,5-3 cm in diameter [ 3 ]. [ijponline.biomedcentral.com]

Aplasia cutis congenita: Involvement of lower limbs and the feet: A rare presentation Department of Paediatrics and Neonatology, PGIMER and Dr. [ijpd.in]

There is minimal bleeding at the lesion sites, and no blisters are present. The rest of the skin has a normal appearance. Rubbing or applying pressure to the normal-appearing skin does not cause sloughing of skin or blister formation. [pedsinreview.aappublications.org]

Mental retardation is present in a few cases. Diagnosis Aplasia cutis congenita is a physical finding that has many causes. [encyclopedia.com]

Form a definitive diagnosis and create the best treatment plans possible using evidence-based medicine and astute clinical experiences from leading international authors—many new to this edition. [books.google.com]

You may also want to contact a university or tertiary medical center in your area, because these centers tend to see more complex cases and have the latest technology and treatments. [rarediseases.info.nih.gov]

The treatment strategies that are currently possible are conservative treatment, surgical treatment, or the two combined. In addition, initial treatment can be separated from late reconstructive treatments. [ncbi.nlm.nih.gov]

Long-term prognosis for Adams-Oliver syndrome is difficult to predict: it is not adequately described in medical literature due to a dearth of cases. [dermnetnz.org]

If the condition is associated with other abnormalities or malformations, the prognosis then depends on the nature and severity of the other condition(s). [3] Major complications of aplasia cutis congenita are rare, but can include hemorrhage, secondary [rarediseases.info.nih.gov]

Prognosis Less severe cases usually resolve within weeks to months, but alopecia persists. Underlying or associated defects may significantly affect mortality and morbidity. [orpha.net]

Prognosis The prognosis for aplasia cutis congenita (ACC) is usually excellent. If the defect is small, recovery is uneventful, with gradual epithelialization and formation of a hairless, atrophic scar over several weeks. [emedicine.medscape.com]

Etiology Etiology is manifold; genetic factors, teratogens, amniotic adhesion, vascular anomalies, pharmacological agents (e.g. methimazole, benzodiazepines), intrauterine trauma and infections have all been implicated. [orpha.net]

[…] has been clarified yet, due to multiple etiological factors involved in the diseases process, as like Genetic factors, intrauterine trauma, infection, compromised vasculature to the skin, medications and teratogens (methimazole, carbimazole, misoprostol [ijpd.in]

Hoyme HE, Higginbottom MC, Jones KL: Vascular etiology of disruptive structural defects in monozigotic twins. Pediatrics 1981;67: 288-291. [wellpath.uniovi.es]

[…] or butterfly pattern with or without involvement of proximal limbs. [3] [4] It is the most common congenital cicatricial alopecia, and is a congenital focal absence of epidermis with or without evidence of other layers of the skin. [5] [6] The exact etiology [en.wikipedia.org]

The phenotype suggests a spinal cord neuropathic etiology. [ read more ] 17701904 [nectarmutation.org]

Summary Epidemiology Incidence is approximately 1/10,000 births. Clinical description ACC is noticed immediately at birth and usually presents as a solitary lesion on or near the vertex. [orpha.net]

Epidemiology Frequency Aplasia cutis congenita (ACC) is an uncommon anomaly of newborns. [emedicine.medscape.com]

Etiology Pathophysiology The exact pathophysiology for ACC remains a mystery. However, various theories have been proposed, including intrauterine trauma, vascular compromise, teratogens such as infectious agents, and medications. [dermatologyadvisor.com]

Pathophysiology and treatment. Yale J Biol Med. 1993; 66 :143–155. [ PMC free article ] [ PubMed ] [ Google Scholar ] 9. Weedon D. Skin pathology. 1st ed. New York: Churchill-Livingstone; 1997. [ Google Scholar ] 10. Bridges AG, Mutasim DF. [ncbi.nlm.nih.gov]

Pathophysiology The exact pathophysiology of aplasia cutis congenita (ACC) is unclear. Proposed mechanisms include intrauterine trauma, vascular compromise, infection, and medications. [emedicine.medscape.com]

The authors of the current study selected a strip of split-thickness skin measuring 0.1 mm to prevent poor healing of the donor site. [woundsresearch.com]

It is hoped that this new knowledge will lead to prevention and treatment of genetic disorders in the future. Information on current clinical trials is posted on the Internet at www.clinicaltrials.gov. [rarediseases.org]

Repair using skin flaps and grafts is only recommended for larger defects, such as those extending down to the dura mater, to prevent any hemorrhage and infection. [orpha.net]

To prevent infection and promote healing, the defect was kept moist and covered at all times, and it was treated with surgical debridement when necessary. [ncbi.nlm.nih.gov]

Extensive bandaging of thelimbs was done to prevent articular contractures.Eighty percent of the lesions had healed withpapyraceous scarring by the twenty-second day oflife (Fig. 3). [documents.tips]