Aplasia cutis congenita is a rare disorder characterized by congenital absence of skin. Ilona J. Frieden classified ACC in 1986 into 9 groups on the basis of location of the lesions and associated congenital anomalies. The scalp is the most commonly involved area with lesser involvement of trunk and extremities. Frieden classified ACC with fetus papyraceus as type 5. This type presents as truncal ACC with symmetrical absence of skin in stellate or butterfly pattern with or without involvement of proximal limbs. 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.
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| - Aplasia cutis congenita (en)
- عدم تنسج الجلد الخلقي (ar)
- Aplasia cutis congenita (de)
- Aplasia cutis congénita (es)
- Aplasia congenita della cute (it)
- Aplasia cutis congenita (fr)
- Aplasia da cútis congênita (pt)
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| - عدم تنسج الجلد الخلقي هو عيب خلقي يتمثل في غياب الجلد في مواضع معينة من جسد المولود، وقد يكون جزءاً من متلازمة كمتلازمة باتو ومتلازمة جوهانسون-بليزارد ومتلازمة آدامز أوليفر ومتلازمة وولف - هيرشيرون. (ar)
- Die Aplasia cutis congenita (ACC) ist eine intrauterin erworbene oder angeborene Erkrankung, eine meist lokal begrenzte kongenitale Aplasie der Kutis, häufig am Kopf. Häufig finden sich weitere Auffälligkeiten an den Augen, an Hals, Nase oder Ohren oder der Gliedmaßen. Synonyme sind: Aplasia cutis verticis congenita (ACCV); englisch Aplasia cutis congenita, nonsyndromic; Congenital Defect of Skull and Scalp; Scalp defect, congenital Die Erkrankung wurde erstmals im Jahre 1767 durch M. Cordon beschrieben. (de)
- La aplasia cutis congénita es una enfermedad presente desde el momento del nacimiento, que se caracteriza por la ausencia de las 3 capas de la piel, en ocasiones solo puede estar ausente una sola capa en algunas zonas del organismo, generalmente afecta el cuero cabelludo provocando calvicie de variable extensión y con menos frecuencia al tronco y las extremidades. La primera descripción fue realizada por Cordon en 1767, su incidencia es muy baja, pues solo se presentan entre 1 y 3 casos por cada 10.000 nacimientos, puede manifestarse como entidad aislada o asociarse a otras anomalías o malformaciones. (es)
- L’aplasia cutis congenita, (ACC), (Pediatric Dermatology 3e édition), correspond à une absence congénitale et visible, dès la naissance, de zones de peau sur des surfaces petites ou étendues. La lésion, si elle guérit, donne une peau fine, plate et avec prolifération de fibroblastes. Une tendance familiale a été parfois rapportée. DD: Maladie des bandes amniotiques, hypoplasie dermique focale (fr)
- Per aplasia congenita della cute si intende, in campo medico, una rara forma specifica di aplasia di carattere genetico, accompagna spesso alcune sindromi, fra cui la sindrome di Adams-Oliver, la e la . (it)
- Aplasia da cútis congênita (ACC), é uma rara doença congênita, que caracteriza-se por formação incompleta da pele. Essa ausência pode estar localizada em qualquer parte do corpo, mas é mais comumente localizada na linha média da região das fontanelas, ao longo do seio sagital superior. Foi descrita pela primeira vez em 1767, sendo que em 1826 relatou-se o primeiro caso dessa lesão no couro cabeludo. (pt)
- Aplasia cutis congenita is a rare disorder characterized by congenital absence of skin. Ilona J. Frieden classified ACC in 1986 into 9 groups on the basis of location of the lesions and associated congenital anomalies. The scalp is the most commonly involved area with lesser involvement of trunk and extremities. Frieden classified ACC with fetus papyraceus as type 5. This type presents as truncal ACC with symmetrical absence of skin in stellate or butterfly pattern with or without involvement of proximal limbs. 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. (en)
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| - Aplasia cutis congenita (en)
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| - Aplasia cutis congenita (en)
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| - Aplasia cutis congenital is autosomal dominant (en)
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| - عدم تنسج الجلد الخلقي هو عيب خلقي يتمثل في غياب الجلد في مواضع معينة من جسد المولود، وقد يكون جزءاً من متلازمة كمتلازمة باتو ومتلازمة جوهانسون-بليزارد ومتلازمة آدامز أوليفر ومتلازمة وولف - هيرشيرون. (ar)
- Aplasia cutis congenita is a rare disorder characterized by congenital absence of skin. Ilona J. Frieden classified ACC in 1986 into 9 groups on the basis of location of the lesions and associated congenital anomalies. The scalp is the most commonly involved area with lesser involvement of trunk and extremities. Frieden classified ACC with fetus papyraceus as type 5. This type presents as truncal ACC with symmetrical absence of skin in stellate or butterfly pattern with or without involvement of proximal limbs. 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. The exact etiology of ACC is still unclear but intrauterine infection by varicella or herpes virus, drugs such as methimazole, misoprostol, valproate, cocaine, marijuana etc., fetus papyraceus, feto-fetal transfusion, vascular coagulation defects, amniotic membrane adherence, abnormal elastic fiber biomechanical forces and trauma are implicated. It can be associated with Johanson–Blizzard syndrome, Adams–Oliver syndrome, trisomy 13, and Wolf–Hirschhorn syndrome.It can also seen with exposure to methimazole and carbimazole in utero. This dermatological manifestation has been linked to Peptidase D haploinsufficiency and a deletion in Chromosome 19. (en)
- Die Aplasia cutis congenita (ACC) ist eine intrauterin erworbene oder angeborene Erkrankung, eine meist lokal begrenzte kongenitale Aplasie der Kutis, häufig am Kopf. Häufig finden sich weitere Auffälligkeiten an den Augen, an Hals, Nase oder Ohren oder der Gliedmaßen. Synonyme sind: Aplasia cutis verticis congenita (ACCV); englisch Aplasia cutis congenita, nonsyndromic; Congenital Defect of Skull and Scalp; Scalp defect, congenital Die Erkrankung wurde erstmals im Jahre 1767 durch M. Cordon beschrieben. (de)
- La aplasia cutis congénita es una enfermedad presente desde el momento del nacimiento, que se caracteriza por la ausencia de las 3 capas de la piel, en ocasiones solo puede estar ausente una sola capa en algunas zonas del organismo, generalmente afecta el cuero cabelludo provocando calvicie de variable extensión y con menos frecuencia al tronco y las extremidades. La primera descripción fue realizada por Cordon en 1767, su incidencia es muy baja, pues solo se presentan entre 1 y 3 casos por cada 10.000 nacimientos, puede manifestarse como entidad aislada o asociarse a otras anomalías o malformaciones. (es)
- L’aplasia cutis congenita, (ACC), (Pediatric Dermatology 3e édition), correspond à une absence congénitale et visible, dès la naissance, de zones de peau sur des surfaces petites ou étendues. La lésion, si elle guérit, donne une peau fine, plate et avec prolifération de fibroblastes. Une tendance familiale a été parfois rapportée. DD: Maladie des bandes amniotiques, hypoplasie dermique focale (fr)
- Per aplasia congenita della cute si intende, in campo medico, una rara forma specifica di aplasia di carattere genetico, accompagna spesso alcune sindromi, fra cui la sindrome di Adams-Oliver, la e la . (it)
- Aplasia da cútis congênita (ACC), é uma rara doença congênita, que caracteriza-se por formação incompleta da pele. Essa ausência pode estar localizada em qualquer parte do corpo, mas é mais comumente localizada na linha média da região das fontanelas, ao longo do seio sagital superior. Foi descrita pela primeira vez em 1767, sendo que em 1826 relatou-se o primeiro caso dessa lesão no couro cabeludo. (pt)
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