Systemic aplasia cutis congenita: A case report and review of the literature

Zhou, Juan; Zheng, Li; Tao, Wenqi

doi:10.1016/j.prp.2009.12.011

Cited by 12 publications

(8 citation statements)

References 19 publications

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“…Almost 500 cases of ACC have been reported in the literature . Suggested risk factors, which were not found in this case, include amniotic membranes, viral infections (herpes simplex virus, varicella zoster virus), intrauterine trauma or vascular ischemia , and drugs (cocaine, which leads to placental vasoconstriction; methimazole; misoprostol; valproic acid) .…”

Section: Discussionmentioning

confidence: 77%

“…Aplasia cutis congenita (ACC) is a rare disorder characterized by a focal defect of the skin at birth . It could result from disrupted development or degeneration of skin in utero.…”

mentioning

confidence: 99%

“…It could result from disrupted development or degeneration of skin in utero. In 85% of cases, ACC presents as a solitary lesion of the scalp vertex . ACC can be isolated or associated with other congenital anomalies.…”

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confidence: 99%

“…ACC can be isolated or associated with other congenital anomalies. It can involve underlying structures such as muscles and bones, often extending to the dura mater when the scalp is involved . In 1986 Frieden classified ACC into nine groups according to location and associated anomalies .…”

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confidence: 99%

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Fetus Papyraceus: Congenital Pulmonary Anomalies Associated with Congenital Aplasia Cutis on the Surviving Twin

Louise

Maruani

Hubert

et al. 2013

Pediatric Dermatology

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Aplasia cutis congenita (ACC) can be associated with fetus papyraceus. We report here the first case of ACC linked to fetus papyraceus with pulmonary anomalies. At birth, the patient presented with skin lesions of the trunk consisting of well-defined, symmetrically distributed, bilateral atrophic ulcerations. Physical examination was otherwise normal. Persistent bronchospasm occurred at the age of 7 months; computed tomography images showed small bilateral pulmonary bullae. At the age of 5 years, skin and pulmonary lesions had not extended. Although the mechanisms of ACC linked to fetus papyraceus are unclear, vascular ischemia is strongly suggested, and could explain the bilateral and symmetric congenital skin and lung aplasia.

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Section: Discussionmentioning

confidence: 77%

“…Aplasia cutis congenita (ACC) is a rare disorder characterized by a focal defect of the skin at birth . It could result from disrupted development or degeneration of skin in utero.…”

mentioning

confidence: 99%

mentioning

confidence: 99%

mentioning

confidence: 99%

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Fetus Papyraceus: Congenital Pulmonary Anomalies Associated with Congenital Aplasia Cutis on the Surviving Twin

Louise

Maruani

Hubert

et al. 2013

Pediatric Dermatology

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“…The most common lesion location is the scalp (70%);[ 17 41 43 ] however, any skin site can be affected and there is also a rare universally fatal subcategory affecting the majority of the dermis. [ 51 59 ] In 1986, a classification system was devised by Frieden consisting of nine main ACC types based on the number, the location of the lesions, and the presence or absence of associated deformities,[ 13 ] which is shown in Table 1 . [ 7 ]…”

Section: Introductionmentioning

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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2018

Atlas Der Pädiatrischen Dermatologie

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Systemic aplasia cutis congenita: A case report and review of the literature

Cited by 12 publications

References 19 publications

Fetus Papyraceus: Congenital Pulmonary Anomalies Associated with Congenital Aplasia Cutis on the Surviving Twin

Fetus Papyraceus: Congenital Pulmonary Anomalies Associated with Congenital Aplasia Cutis on the Surviving Twin

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

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