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

2021 ◽  
Vol Volume 14 ◽  
pp. 1413-1418
Author(s):  
Raden Mohamad Rendy Ariezal Effendi ◽  
Lita Nuraeni ◽  
Inne Arline Diana ◽  
Srie Prihianti Gondokaryono ◽  
Hendra Gunawan ◽  
...  
2021 ◽  
Vol 15 (1) ◽  
Author(s):  
V. Thadchanamoorthy ◽  
Kavinda Dayasiri ◽  
M. Thirukumar ◽  
N. Thamilvannan ◽  
S. H. Chandraratne

Abstract Background Aplasia cutis congenita is regarded as congenital focal absence of skin in the newborn, and occurrence of more than three similar skin defects is rare. The etiology is thought to be multifactorial, and precise etiopathogenesis is unknown. Case presentation A 13-day-old newborn Sri Lankan Tamil girl was referred to the dermatologic clinic with multiple skin defects at birth. There were six lesions on the body, and two of them had healed during intrauterine period, leaving scars. This was a second twin of her pregnancy. Her first twin fetus had demised before 19 weeks of pregnancy and was confirmed to be fetus papyraceous based on ultrasound-guided fetal assessment. The said child was thoroughly investigated and found to have no other congenital abnormalities. Chromosomal studies yielded normal findings. She was treated with tropical antibacterial ointment, and all lesions resolved spontaneously within 4 weeks, leaving scars. Physiotherapy was commenced to prevent contracture formation, and follow-up was arranged in collaboration with the plastic surgical team. Conclusions Aplasia cutis congenita is a rare condition of uncertain etiology, but consanguinity may play a role. This report described a newborn with type V cutis aplasia congenita in whom the diagnosis was confirmed based on clinical features and revision of antenatal history. The management depends on the pattern, extent, location, severity, underlying causes, and associated anomalies.


Author(s):  
Vivekananda Ittigi ◽  
Aparna Anto

<p class="abstract">Aplasia cutis congenita is a condition characterized by congenital absence of all skin layers in a localized or widespread area. Frieden's classification recognized a rare subtype, type V, that is associated with multiple gestations in which there is the in-utero demise of a twin with resultant fetus papyraceus or mummification. A twin pregnancy was complicated by in-utero death of one twin at 5 months. On full term birth of the other twin with fetus papyraceus large defects in the skin of knees were noted bilaterally. Conservative management with topical antibiotics and emollients helped in complete re-epithelialization in few months. As the use of reproductive technologies increase the incidence of multiple gestation and associated conditions are expected to increase. This type is clinically unique in that it is characterized by stellate lesions in a symmetrical distribution over the trunk and extremities, differing from other subtypes, which are typically localized to scalp (70%-85% of cases) or extremities. Management ranges from conservative to surgical grafts.</p>


2010 ◽  
Vol 30 (2) ◽  
pp. 171-172 ◽  
Author(s):  
Umar A. Qureshi ◽  
Nisar Ahmed

2012 ◽  
Vol 30 (6) ◽  
pp. e208-e213 ◽  
Author(s):  
Daniel Morrow ◽  
Robert Schelonka ◽  
Alfons Krol ◽  
Michael Davies ◽  
Anna Kuang

2021 ◽  
Vol 8 (3) ◽  
pp. 997
Author(s):  
Naim Sulaiman Abuzarifa ◽  
Wan Azman Wan Sulaiman

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. 


2021 ◽  
Vol 9 (12) ◽  
pp. 251-254
Author(s):  
Ahmed Mead ◽  
◽  
Yordanos Alem ◽  
Omar Adam Sheikh ◽  
Layla Ibrahim Hussein ◽  
...  

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.


2017 ◽  
Vol 56 (6) ◽  
pp. e118-e121 ◽  
Author(s):  
Benjamin M. Perry ◽  
Cory B. Maughan ◽  
Matthew S. Crosby ◽  
Scott D. Hadenfeld

2020 ◽  
Vol 3 (3) ◽  
pp. 66-68
Author(s):  
Bittmann Stefan ◽  
◽  
Luchter Elisabeth ◽  
Villalon Gloria ◽  
Moschüring-Alieva Elena ◽  
...  

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.


2008 ◽  
Vol 39 (6) ◽  
pp. 435-437 ◽  
Author(s):  
Po-Chuang Wu ◽  
Jian-Ping Jiang ◽  
Chih-Chien Wang ◽  
Shyi-Jou Chen

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