Extensive Type V Aplasia Cutis Congenita Without Fetus Papyraceus or Placental Infarction: A Rare Case
Raden Mohamad Rendy Ariezal Effendi, Lita Nuraeni, Inne Arline Diana, Srie Prihianti Gondokaryono, Hendra Gunawan, Reiva Farah Dwiyana Department of Dermatology and Venereology, Faculty of Medicine Universitas Padjadjaran - Dr. Hasan Sadikin Hospital, Bandung, IndonesiaCorrespondence: Raden Mohamad...
| Main Authors: | , , , , , |
|---|---|
| Format: | Article |
| Language: | English |
| Published: |
Dove Medical Press
2021-10-01
|
| Series: | Clinical, Cosmetic and Investigational Dermatology |
| Subjects: | |
| Online Access: | https://www.dovepress.com/extensive-type-v-aplasia-cutis-congenita-without-fetus-papyraceus-or-p-peer-reviewed-fulltext-article-CCID |
| _version_ | 1818899047657766912 |
|---|---|
| author | Effendi RMRA Nuraeni L Diana IA Gondokaryono SP Gunawan H Dwiyana RF |
| author_facet | Effendi RMRA Nuraeni L Diana IA Gondokaryono SP Gunawan H Dwiyana RF |
| author_sort | Effendi RMRA |
| collection | DOAJ |
| description | Raden Mohamad Rendy Ariezal Effendi, Lita Nuraeni, Inne Arline Diana, Srie Prihianti Gondokaryono, Hendra Gunawan, Reiva Farah Dwiyana Department of Dermatology and Venereology, Faculty of Medicine Universitas Padjadjaran - Dr. Hasan Sadikin Hospital, Bandung, IndonesiaCorrespondence: Raden Mohamad Rendy Ariezal EffendiDepartment of Dermatology and Venereology, Faculty of Medicine, Universitas Padjadjaran - Dr. Hasan Sadikin Hospital, Jl. Pasteur 38, Bandung, 40161, West Java, IndonesiaEmail rendy.ariezal.effendi@unpad.ac.idAbstract: Aplasia cutis congenita (ACC) is a congenital disorder characterized by the absence of epidermis, dermis, and sometimes subcutaneous tissue and bone. There are nine types of ACC based on the number and location of the lesions as well as the presence or absence of associated deformities, with type I ACC being the most common. Type V affects the trunk with a characteristic pattern resembling the “H” letter of the alphabet, generally accompanied by fetus papyraceus (death of one of the twin fetuses) or placental infarction. Type V ACC without fetus papyraceus and placental infarction is a rare case. We reported a case of type V ACC in a 3-day-old baby girl, with clinical manifestation of extensive ulcers on the scalp, back, buttocks, and both lower extremities, in an “H”-shaped pattern, with history of placenta accreta, but no fetus papyraceus or placental infarction was found. The patient received dialkyl carbamoyl chloride hydrophobic swab with hydrogel (Sorbact® gel dressing-BSN Medical) and hydrocolloid wound dressing with good response, as indicated by the wounds becoming dried up, leaving an atrophic scar. Complete epithelialization was achieved in 72 days. The cause of type V ACC remains not completely understood. The diagnosis of ACC can be established based on clinical manifestations. Further examination is required if extracutaneous involvement is suspected. Early identification of the etiology of the lesions and appropriate investigation and treatment, including conservative wound management, are needed so that aplastic lesions can heal successfully, although cosmetic repair may be required at a later stage.Keywords: aplasia cutis congenita, fetus papyraceus, placenta accreta |
| first_indexed | 2024-12-19T19:41:45Z |
| format | Article |
| id | doaj.art-a3b044fed31e4df78bae6e1a7fcfdaa4 |
| institution | Directory Open Access Journal |
| issn | 1178-7015 |
| language | English |
| last_indexed | 2024-12-19T19:41:45Z |
| publishDate | 2021-10-01 |
| publisher | Dove Medical Press |
| record_format | Article |
| series | Clinical, Cosmetic and Investigational Dermatology |
| spelling | doaj.art-a3b044fed31e4df78bae6e1a7fcfdaa42022-12-21T20:08:14ZengDove Medical PressClinical, Cosmetic and Investigational Dermatology1178-70152021-10-01Volume 141413141869436Extensive Type V Aplasia Cutis Congenita Without Fetus Papyraceus or Placental Infarction: A Rare CaseEffendi RMRANuraeni LDiana IAGondokaryono SPGunawan HDwiyana RFRaden Mohamad Rendy Ariezal Effendi, Lita Nuraeni, Inne Arline Diana, Srie Prihianti Gondokaryono, Hendra Gunawan, Reiva Farah Dwiyana Department of Dermatology and Venereology, Faculty of Medicine Universitas Padjadjaran - Dr. Hasan Sadikin Hospital, Bandung, IndonesiaCorrespondence: Raden Mohamad Rendy Ariezal EffendiDepartment of Dermatology and Venereology, Faculty of Medicine, Universitas Padjadjaran - Dr. Hasan Sadikin Hospital, Jl. Pasteur 38, Bandung, 40161, West Java, IndonesiaEmail rendy.ariezal.effendi@unpad.ac.idAbstract: Aplasia cutis congenita (ACC) is a congenital disorder characterized by the absence of epidermis, dermis, and sometimes subcutaneous tissue and bone. There are nine types of ACC based on the number and location of the lesions as well as the presence or absence of associated deformities, with type I ACC being the most common. Type V affects the trunk with a characteristic pattern resembling the “H” letter of the alphabet, generally accompanied by fetus papyraceus (death of one of the twin fetuses) or placental infarction. Type V ACC without fetus papyraceus and placental infarction is a rare case. We reported a case of type V ACC in a 3-day-old baby girl, with clinical manifestation of extensive ulcers on the scalp, back, buttocks, and both lower extremities, in an “H”-shaped pattern, with history of placenta accreta, but no fetus papyraceus or placental infarction was found. The patient received dialkyl carbamoyl chloride hydrophobic swab with hydrogel (Sorbact® gel dressing-BSN Medical) and hydrocolloid wound dressing with good response, as indicated by the wounds becoming dried up, leaving an atrophic scar. Complete epithelialization was achieved in 72 days. The cause of type V ACC remains not completely understood. The diagnosis of ACC can be established based on clinical manifestations. Further examination is required if extracutaneous involvement is suspected. Early identification of the etiology of the lesions and appropriate investigation and treatment, including conservative wound management, are needed so that aplastic lesions can heal successfully, although cosmetic repair may be required at a later stage.Keywords: aplasia cutis congenita, fetus papyraceus, placenta accretahttps://www.dovepress.com/extensive-type-v-aplasia-cutis-congenita-without-fetus-papyraceus-or-p-peer-reviewed-fulltext-article-CCIDaplasia cutis congenitafetus papyraceusplacenta accreta |
| spellingShingle | Effendi RMRA Nuraeni L Diana IA Gondokaryono SP Gunawan H Dwiyana RF Extensive Type V Aplasia Cutis Congenita Without Fetus Papyraceus or Placental Infarction: A Rare Case Clinical, Cosmetic and Investigational Dermatology aplasia cutis congenita fetus papyraceus placenta accreta |
| title | Extensive Type V Aplasia Cutis Congenita Without Fetus Papyraceus or Placental Infarction: A Rare Case |
| title_full | Extensive Type V Aplasia Cutis Congenita Without Fetus Papyraceus or Placental Infarction: A Rare Case |
| title_fullStr | Extensive Type V Aplasia Cutis Congenita Without Fetus Papyraceus or Placental Infarction: A Rare Case |
| title_full_unstemmed | Extensive Type V Aplasia Cutis Congenita Without Fetus Papyraceus or Placental Infarction: A Rare Case |
| title_short | Extensive Type V Aplasia Cutis Congenita Without Fetus Papyraceus or Placental Infarction: A Rare Case |
| title_sort | extensive type v aplasia cutis congenita without fetus papyraceus or placental infarction a rare case |
| topic | aplasia cutis congenita fetus papyraceus placenta accreta |
| url | https://www.dovepress.com/extensive-type-v-aplasia-cutis-congenita-without-fetus-papyraceus-or-p-peer-reviewed-fulltext-article-CCID |
| work_keys_str_mv | AT effendirmra extensivetypevaplasiacutiscongenitawithoutfetuspapyraceusorplacentalinfarctionararecase AT nuraenil extensivetypevaplasiacutiscongenitawithoutfetuspapyraceusorplacentalinfarctionararecase AT dianaia extensivetypevaplasiacutiscongenitawithoutfetuspapyraceusorplacentalinfarctionararecase AT gondokaryonosp extensivetypevaplasiacutiscongenitawithoutfetuspapyraceusorplacentalinfarctionararecase AT gunawanh extensivetypevaplasiacutiscongenitawithoutfetuspapyraceusorplacentalinfarctionararecase AT dwiyanarf extensivetypevaplasiacutiscongenitawithoutfetuspapyraceusorplacentalinfarctionararecase |