Aplasia cutis congenita

Steinbacher, Johannes; Rath, T.; Tzou, Chieh‐Han John

doi:10.1055/s-0042-103155

Cited by 3 publications

(3 citation statements)

References 29 publications

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“…Aplasia cutis congenita (ACC) is a rare congenital skin malformation that presents as a localized skin defect . It can manifest as a cicatrical scar, ulcer, or focal skin defect, and should be differentiated from encephalocele, dermoid cyst, neonatal herpes, focal dermal hypoplasia, amniotic band syndrome, and nevus sebaceous .…”

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confidence: 99%

Aplasia cutis congenita in Korea: Single center experience and literature review

Yang

Kim

et al. 2020

Pediatrics International

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Background Aplasia cutis congenita (ACC) is a rare congenital malformation characterized by a localized absence of skin. which most commonly affects the scalp. We performed the present study to elucidate the basic clinical data regarding ACC in Korea, including demographics, clinical features, radiological and therapeutic results. Methods Fifty‐nine patients (70 lesions) with ACC (35 from our department and 24 from a Koreamed database search) were enrolled. We assessed demographics, family and obstetrical histories, clinical features (multiplicity, subtype, size, shape, hair collar sign, location, and Frieden's classification), and radiologic and therapeutic results. Results The mean age of patients was 2.62 years, with a male‐to‐female ratio of 1.03. A minority of patients had a family history (three patients), birth trauma (one patient), maternal drug use (two patients), or human immunodeficiency virus infection (one patient) during pregnancy, and fetus papyraceus of placental infarcts (two patients). Six patients (6/59, 10.17%) had multiple lesions. Scarring was the most common manifestation (39/70, 55.71%). The scalp was the most commonly affected site (50 cases, 71.43%). Thirty‐nine patients (66.10%) met Frieden's type I classification (scalp ACC without multiple anomalies). Radiological investigations were performed in 30 patients (30/59, 50.85%) with abnormal findings in eight patients. Twenty‐five patients (42.37%) were managed conservatively, and 17 patients (28.81%) were treated with local wound care. Conclusions This is the first and largest study assessing the basic clinical data of ACC in Korea. The results of the present study could be useful for pediatricians and dermatologists who routinely manage ACC.

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confidence: 99%

Aplasia cutis congenita in Korea: Single center experience and literature review

Yang

Kim

et al. 2020

Pediatrics International

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“…[ 48 56 58 ] There are multiple reports of specialized adherent wound dressings that are thought to offer increased wound healing rates,[ 3 4 23 ] as well as recent reports of novel dressing materials such as fatty gauzes. [ 49 ] However, none of the more specialized dressing materials have proven to be significantly superior, and therefore, it is accepted to treat ACC with standard traditional wound care. [ 30 ] Although conservative treatment has traditionally been the default strategy for normal and small-sized lesions, lately there have been reports of extensive ACC lesions managed through the conservative route with excellent healing results,[ 14 37 ] and this shows a tendency of recent literature in favor of the conservative approach.…”

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confidence: 99%

Aplasia cutis congenita: Two case reports and discussion of the literature

Blionas¹,

Giakoumettis²,

Antoniades³

et al. 2017

Surg Neurol Int

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Background:Aplasia cutis congenita (ACC) is a part of a heterogeneous group of conditions characterized by the congenital absence of epidermis, dermis, and in some cases, subcutaneous tissues or bone usually involving the scalp vertex. There is an estimated incidence of 3 in 10,000 births resulting in a total number of 500 reported cases to date. The lesions may occur on every body surface although localized scalp lesions form the most frequent pattern (70%). Complete aplasia involving bone defects occurs in approximately 20% of cases. ACC can occur as an isolated defect or can be associated with a number of other congenital anomalies such as limb anomalies or embryologic malformations. In patients with large scalp and skull defects, there is increased risk of infection and bleeding along with increased mortality and therefore prompt and effective management is advised.Case Description:We describe two cases of ACC, involving a 4 × 3 cm defect managed conservatively and a larger 10 × 5 cm defect managed surgically with the use of a temporo-occipital scalp flap. Both cases had an excellent outcome.Conclusions:Multiple treatment regimens exist for ACC, but there is no consensus on treatment strategies. Conservative treatment has been described and advocated, but many authors have emphasized the disadvantages of this treatment modality. Decision between conservative and surgical management must be individualized according to lesion size and location.

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“…This includes physiological saline solution, continuous saline drips, betadine solution, bacitracin ointment and silver sulfadiazine dressings 28 . Today we have lots of specialized adherent wound dressings that offer increased wound healing rates [29][30][31] and fatty gauzes 32 . None of these materials proved to be better than the other 12 .…”

Section: Reviewmentioning

confidence: 99%

Aplasia Cutis Congenita. Case report and literature review

Cohen¹,

Ribeiro²,

Costa³

et al. 2019

jbnc

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Aplasia Cutis Congenita (ACC) is a rare condition characterized by congenital absence of epidermis, dermis and, in some cases, like this one, subcutaneous tissue and bone. It usually involves the scalp vertex. The estimated incidence is 3 in 10,000 births resulting in a total number of 500 reported cases till nowadays. The lesion can occur on everybody surface, but scalp is the most affected region (70% of the cases). In about 20% of cases there is bone lesion. ACC can occur as an isolated defect or can be associated with a number of other congenitally anomalies such as limb anomalies or embryologic malformations. In patients with large scalp and skull defects, there is increased risk of infection and bleeding along with increased mortality and therefore prompt and effective management is advised. Case presentation: We describe a child with ACC involving almost all skull, where it could be seen the brain only with arachnoid membrane. At presentation there was no Cerebral Spinal Fluid (CSF) leakage. And it was managed surgically. The child was operated on one time. It was covered with non-absorbable matrix and the skin was approached. Conclusion: There are multiple treatments for this condition. But there is no consensus on treatment strategies. Conservative treatment can be tried, especially when there is no bone lesion. The treatment must be individualized for each case.

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Aplasia cutis congenita

Abstract: Conservative treatment with fatty gauze represents a reliable therapeutic option in case the disease solely affects the skin.

Cited by 3 publications

References 29 publications

Aplasia cutis congenita in Korea: Single center experience and literature review

Aplasia cutis congenita in Korea: Single center experience and literature review

Aplasia cutis congenita: Two case reports and discussion of the literature

Aplasia Cutis Congenita. Case report and literature review

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