Aplasia Cutis Congenita (ACC): An Extremely Rare Case in Childhood

Aplasia cutis congenita (ACC) is a rare disease characterized by a local absence of skin and, in some cases, subcutaneous tissue. Most cases occur in the scalp, but the lesion can occur anywhere in the trunk and extremities. ACC is usually an isolated defect, but can also be associated with other abnormalities. Most reported cases are sporadic, with a few reports of family events. Neither pathogenesis nor etiology are clarified. In most cases, healing is spontaneous, and apart from keeping the lesion clean, no specific treatment is required. This report presents a case of ACC at the head and provides a brief review of the literature.

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Aplasia cutis congenita: Two cases of non-scalp lesions

Indian Journal of Plastic Surgery ◽

10.1055/s-0039-1699359 ◽

2009 ◽

Vol 42 (02) ◽

pp. 261-264 ◽

Cited By ~ 1

Author(s):

Tarek A. Abulezz ◽

Mahmoud A. Shalkamy

Keyword(s):

Specific Treatment ◽

Rare Condition ◽

Lower Limbs ◽

Familial Occurrence ◽

Review Of The Literature ◽

Aplasia Cutis Congenita ◽

Subcutaneous Tissues ◽

Scalp Lesions ◽

Aplasia Cutis

ABSTRACTAplasia cutis congenita (ACC) is a rare condition characterized by a localized absence of skin and in some cases, the subcutaneous tissues. The majority of cases occur in the scalp; however, the lesion may occur anywhere in the trunk and extremities. ACC is most often an isolated defect, but it can be associated with other anomalies. Most reported cases are sporadic with a few reports of familial occurrence. Neither the pathogenesis nor the aetiology is clarified. Healing is spontaneous in most cases, and apart from keeping the lesion clean, no specific treatment is required. In this report, two cases of non-scalp ACC occurring in the lower limbs are presented and a brief review of the literature is conducted.

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Belated Diagnosis of a Primary Bone Lymphoma of the Elbow: A Rare Case and Review of the Literature

Case Reports in Orthopedic Research ◽

10.1159/000509128 ◽

2020 ◽

pp. 100-107

Author(s):

Michele Boffano ◽

Nicola Ratto ◽

Martina Rezzoagli ◽

Andrea Conti ◽

Pietro Pellegrino ◽

...

Keyword(s):

Differential Diagnosis ◽

Rare Disease ◽

Rare Case ◽

Early Stage ◽

Bone Lesions ◽

Review Of The Literature ◽

Primary Bone Lymphoma ◽

Lytic Bone Lesions ◽

Primary Bone ◽

Bone Lymphoma

Primary non-Hodgkin bone lymphoma (PBL) is a rare disease that accounts for <2% of all lymphomas in adults. PBL can be monostotic or polyostotic, mainly causing destructive and lytic bone lesions frequently located in the femur, humerus, and pelvis. PBL is rarely considered a differential diagnosis of the osteolytic tumor. In addition, PBL is not uncommonly diagnosed with delay because patients do not experience symptoms nor show objective abnormalities in the early stage of disease. Here, we reported a 60-year-old woman with a PBL of the elbow.

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Type V Aplasia Cutis Congenita: Case Report, Review of the Literature, and Proposed Treatment Algorithm

Pediatric Dermatology ◽

10.1111/j.1525-1470.2012.01742.x ◽

2012 ◽

Vol 30 (6) ◽

pp. e208-e213 ◽

Cited By ~ 10

Author(s):

Daniel Morrow ◽

Robert Schelonka ◽

Alfons Krol ◽

Michael Davies ◽

Anna Kuang

Keyword(s):

Case Report ◽

Treatment Algorithm ◽

Review Of The Literature ◽

Aplasia Cutis Congenita ◽

Type V ◽

Aplasia Cutis

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A rare case report of aplasia cutis congenita of the scalp with triplet skin defects in non-syndromic newborn

International Surgery Journal ◽

10.18203/2349-2902.isj20210468 ◽

2021 ◽

Vol 8 (3) ◽

pp. 997

Author(s):

Naim Sulaiman Abuzarifa ◽

Wan Azman Wan Sulaiman

Keyword(s):

Case Report ◽

Gestational Age ◽

Rare Case ◽

Surgical Intervention ◽

Skin Condition ◽

Skin Defect ◽

Aplasia Cutis Congenita ◽

Skin Defects ◽

Rare Case Report ◽

Aplasia Cutis

Aplasia cutis congenita is an uncommon localized or widespread congenital skin condition characterized by the absence of the skin, and occasionally underlying tissues occurs in about one every 10.000 birth mostly in the scalp as single or more than one lesion and sometimes occurs in extremities and trunk with an uncertain cause and can be associated with numerous syndromes or can be sporadic which is diagnosed clinically and usually conservatively managed but sometimes surgical intervention needed. In this literature, we present nonsyndromic newborn Malay girl normally delivered with 35 weeks gestational age with triplet skin raw areas at the vertex of the scalp with well-demarcated defect round in shape measured about 0.5 in radius covered with a noninflammatory, necrotic patch. To our knowledge, many works of literatures presented Aplasia cutis congenita at the scalp with solitary single or occasionally more than one skin defect. In contrast, in our case, we present an infrequent rare case of triplet skin defect of aplasia cutis congenita for nonsyndromic newborns.

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Aplasia Cutis Congenita. Case report and literature review

JBNC - JORNAL BRASILEIRO DE NEUROCIRURGIA ◽

10.22290/jbnc.v30i3.1846 ◽

2019 ◽

Vol 30 (3) ◽

pp. 267-272

Author(s):

Moysés Isaac Cohen ◽

Jefferson Travessa Ribeiro ◽

Cleinaldo De Almeida Costa ◽

Fernando Luís Westphal

Keyword(s):

Subcutaneous Tissue ◽

Bone Lesion ◽

Treatment Strategies ◽

Rare Condition ◽

Aplasia Cutis Congenita ◽

Skull Defects ◽

Increased Risk ◽

Csf Leakage ◽

Multiple Treatments ◽

Aplasia Cutis

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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Bullous aplasia cutis congenita: A rare presentation of a rare disease

Dermatology Online Journal ◽

10.5070/d3262047428 ◽

2020 ◽

Vol 26 (2) ◽

Author(s):

Lauren Watchmaker ◽

Jacqueline Watchmaker

Keyword(s):

Rare Disease ◽

Rare Presentation ◽

Aplasia Cutis Congenita ◽

Aplasia Cutis

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A CASE REPORT OF APLASIA CUTIS CONGENITA TYPE VI: BART'S SYNDROME

International Journal of Advanced Research ◽

10.21474/ijar01/13901 ◽

2021 ◽

Vol 9 (12) ◽

pp. 251-254

Author(s):

Ahmed Mead ◽

◽

Yordanos Alem ◽

Omar Adam Sheikh ◽

Layla Ibrahim Hussein ◽

...

Keyword(s):

Case Report ◽

Rare Case ◽

Genetic Disorder ◽

Skin Layer ◽

Topical Antibiotic ◽

Aplasia Cutis Congenita ◽

Rare Genetic Disorder ◽

Skin Formation ◽

Aplasia Cutis

Aplasia cutis congenita type VI (Barts syndrome), is a rare genetic disorder characterized by congenital localized absence of skin, formation of blisters (epidermolysisbullosa), and nail abnormalities. In this report, we present a rare case of aplasia cutis congenita type VI (Barts syndrome) in a newborn malebaby with the absence of a skin layer over the anterior right leg, slightly below the patella (kneecap) and around the ankle joint.On the second day, the affected areas developed blisters characterizing epidermolysisbullosa.Laboratory investigationswere all normal. The patients wound was managed conservatively with dressing and topical antibiotic ointments.

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