Epidermolysis Bullosa and Kindler Syndrome

Mellerio, Jemima E.; Martinéz, Anna E.; Has, Christina

doi:10.1002/9781119142812.ch76

Harper's Textbook of Pediatric Dermatology 2019

DOI: 10.1002/9781119142812.ch76

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Epidermolysis Bullosa and Kindler Syndrome

Jemima E. Mellerio

Anna E. Martinéz

Christina Has

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2021

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“…Bullous dermolysis of the newborn (BDN) or transient bullous dermolysis is an extremely rare clinical type of dystrophic EB, characterized by subepidermal blistering at birth or shortly thereafter. 9 Skin lesions in BDN are found in friction areas and the extremities at birth, 10,11 and new blisters can appear due to local trauma followed by rapid improvement with pigmentation or minimal scarring. 9,10 In this patient, no blisters were found and there were no new skin lesions following local trauma.…”

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Extensive Type V Aplasia Cutis Congenita Without Fetus Papyraceus or Placental Infarction: A Rare Case

Effendi

Nuraeni

Diana

et al. 2021

CCID

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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.

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confidence: 99%

Extensive Type V Aplasia Cutis Congenita Without Fetus Papyraceus or Placental Infarction: A Rare Case

Effendi

Nuraeni

Diana

et al. 2021

CCID

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Epidermolysis Bullosa and Kindler Syndrome

Cited by 1 publication

References 230 publications

Extensive Type V Aplasia Cutis Congenita Without Fetus Papyraceus or Placental Infarction: A Rare Case

Extensive Type V Aplasia Cutis Congenita Without Fetus Papyraceus or Placental Infarction: A Rare Case

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