<\/span><\/h2>\nAplasia cutis congenita describes the congenital<\/span> absence of skin The most common form appears as a defect of the scalp at birth. Aplasia cutis is also a component of a series of genetic<\/span> syndromes<\/p>\n\n<\/span>Aplasia cutis<\/span><\/h5>\n<\/section>\n\n\n
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Aplasia cutis<\/p>\n
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Aplasia cutis<\/p>\n<\/p><\/div>\n<\/section>\n
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<\/span>How is aplasia cutis contracted and who is at risk?<\/span><\/h2>\nFactors that contribute to aplasia cutis include:<\/p>\n
\n- Genetics: Aplasia cutis can be seen in association with other congenital skin defects, such as organoids or epidermal<\/span> naevi<\/span>. Family<\/span> cases are reported with autosomal<\/span> dominant and autosomal recessive<\/span> heritage.<\/li>\n
- Nonsyndromic congenital aplasia cutis has been associated with a heterozygous<\/span> mutation<\/span> at BMS1 gene<\/span> (611448) in chromosome<\/span> 10q11. <\/li>\n
- Placental infection<\/span> during pregnancy, such as chickenpox (chicken pox) or herpes simplex\n<\/li>\n
- Teratogens (drugs or chemicals that cause birth defects, such as methimazole, carbimazole, misoprostol, cocaine, marijuana, and valproic acid<\/span>)<\/li>\n
- Skin development defect in the embryo \/ fetus.<\/li>\n
- \nAmniotic<\/span> bands due to early rupture of amniotic membranes<\/li>\n
- The death of a twin fetus (papyrus fetus).<\/li>\n<\/ul>\n
Aplasia cutis is rare and does not have a particular race or sex<\/span> you are at higher risk. <\/p>\n\n
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<\/span>What are the clinical characteristics of aplasia cutis?<\/span><\/h2>\nIn 70 to 80% of cases, aplasia cutis affects the scalp side<\/span> to the middle line<\/span>But injuries can also occur on the face, trunk, or extremities, sometimes symmetrically. <\/p>\n\n- Areas of skin loss or ulceration<\/span> they vary in size from 0.5 cm to 10 cm.<\/li>\n
- The flaws are notinflammatory<\/span> And they are fine demarcated<\/span>.<\/li>\n
- A plume of hair<\/span> Aplasia surrounding skin may denote underlying malformation<\/span> with defecT neural tube.<\/li>\n
- Superficial cutaneous aplasia involves only the epidermis<\/span> (upper layers of the skin). Superficial defects generally heal before the child is born, leaving a scar<\/span>.<\/li>\n
- Deeper defects can extend through the dermis<\/span>, subcutaneous<\/span> tissue, and rarely periosteum, skull, or dura.<\/li>\n
- Aplasia cutis may partially heal before delivery and appear hairless, atrophic<\/span>, membranous, parchment or fibrotic<\/span> scar.<\/li>\n
- Aplasia membranous cutis is a flat, white membrane that covers a defect in the skull. Distorted hair growth, known as a hair collar sign<\/span>, is a marker of an underlying cranial defect, such as encephalocele, meningocele, and brain tissue outside the skull.<\/li>\n
- A rare bullous<\/span> A variant of congenital aplasia cutis has been reported.<\/li>\n
- Some people with aplasia cutis also have congenital malformations of the heart, gastrointestinal, genitourinary (such as gastroschisis or <\/span>umbilical hernia)<\/span>and central nervous system (how <\/span>meningomyelocele or spinal dysraphism)<\/span>.<\/li>\n<\/ul>\n
<\/span>Classification of Aplasia Cutis<\/span><\/h2>\nThe Frieden classification system for aplasia cutis has 9 groups depending on the number and location of injuries and associated malformations.<\/p>\n
<\/span>Group 1<\/span><\/h3>\nAplasia cutis of the congenital scalp without other abnormalities.
<\/span><\/p>\n<\/span>Group 2<\/span><\/h3>\nAplasia cutis of the congenital scalp with abnormalities in the extremities: includes Adams-Oliver syndrome<\/span> <\/span><\/p>\n<\/span>Group 3<\/span><\/h3>\nAplasia cutis of the congenital scalp with solitary epidermal or sebaceous<\/span> (organoid) nevus<\/span> or an epidermal nevus syndrome<\/p>\n<\/span>Group 4<\/span><\/h3>\nAplasia congenital skin that covers deep embryological malformations<\/p>\n
<\/span>Team 5<\/span><\/h3>\nAplasia congenital stellate cutis of the trunk or limb associated with the papiraceous fetus or placental infarction
<\/sup><\/p>\n<\/span>Group 6<\/span><\/h3>\nEpidermolysis bullosa with congenital absence of skin (congenital aplasia cutis of the lower limb associated with epidermolysis bullosa (Bart syndrome)<\/p>\n
<\/span>Group 7<\/span><\/h3>\nAplasia congenital cutis de limb without epidermolysis bullosa
<\/sup><\/p>\n<\/span>Group 8<\/span><\/h3>\nAplasia cutis congenita due to teratogens such as intrauterine<\/span> herpes simplex or chickenpox infection or medicines like methimazole or carbimazole<\/p>\n<\/span>Group 9<\/span><\/h3>\nAplasia cutis congenita associated with malformation syndromes, including trisomy 13 with large membranous defect (Patau syndrome), Wolf-Hirschhorn syndrome (deletion of the short arm of chromosome 4) with defects of the scalp in the midline, Setleis syndrome with aplasia bitemporal congenital skin and abnormal eyelashes; Johanson-Blizzard syndrome with stellate scalp defects; focal<\/span> dermal<\/span> hypoplasia (Goltz syndrome); and others.<\/p>\n<\/span>What are the complications of aplasia cutis?<\/span><\/h2>\nComplications of aplasia cutis rarely occur, but may include:<\/p>\n
\n- \nArterial<\/span> bleeding<\/li>\n
- Secondary wound infection\n<\/li>\n
- Sagittal breast<\/span> thrombosis<\/span>.<\/li>\n
- Brain infection that leads to death.<\/li>\n<\/ul>\n
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<\/span>What treatments are available for Aplasia Cutis?<\/span><\/h2>\nSmall areas of aplasia cutis generally heal spontaneously over time, forming a hairless scar. To avoid infection, mild cleanses and mild ointments can be used. If an infection occurs, antibiotics can be used.<\/p>\n
Larger injuries or multiple defects of the scalp may require surgical repair; sometimes a skin or bone graft may be necessary. Tissue expanders can be used.<\/p>\n<\/section>","protected":false},"excerpt":{"rendered":"
Que es aplasia cutis congenita? La aplasia cutis cong\u00e9nita describe la cong\u00e9nito ausencia de piel La forma m\u00e1s com\u00fan se presenta como un defecto del cuero cabelludo al nacer. La…<\/p>","protected":false},"author":8,"featured_media":0,"menu_order":0,"comment_status":"closed","ping_status":"closed","template":"","format":"standard","meta":{"footnotes":""},"categories":[204],"tags":[],"class_list":["post-7013","wiki","type-wiki","status-publish","format-standard","category-glosario-definiciones"],"_links":{"self":[{"href":"https:\/\/doctorhoogstra.com\/en\/wp-json\/wp\/v2\/wiki\/7013","targetHints":{"allow":["GET"]}}],"collection":[{"href":"https:\/\/doctorhoogstra.com\/en\/wp-json\/wp\/v2\/wiki"}],"about":[{"href":"https:\/\/doctorhoogstra.com\/en\/wp-json\/wp\/v2\/types\/wiki"}],"author":[{"embeddable":true,"href":"https:\/\/doctorhoogstra.com\/en\/wp-json\/wp\/v2\/users\/8"}],"replies":[{"embeddable":true,"href":"https:\/\/doctorhoogstra.com\/en\/wp-json\/wp\/v2\/comments?post=7013"}],"version-history":[{"count":0,"href":"https:\/\/doctorhoogstra.com\/en\/wp-json\/wp\/v2\/wiki\/7013\/revisions"}],"wp:attachment":[{"href":"https:\/\/doctorhoogstra.com\/en\/wp-json\/wp\/v2\/media?parent=7013"}],"wp:term":[{"taxonomy":"category","embeddable":true,"href":"https:\/\/doctorhoogstra.com\/en\/wp-json\/wp\/v2\/categories?post=7013"},{"taxonomy":"post_tag","embeddable":true,"href":"https:\/\/doctorhoogstra.com\/en\/wp-json\/wp\/v2\/tags?post=7013"}],"curies":[{"name":"wp","href":"https:\/\/api.w.org\/{rel}","templated":true}]}}