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A Case of Coexistence of aplasia cutis congenita and Giant congenital Melanocytic Nevus:Coexistence of Two Rare Skin Diseases

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Chinese Journal of Plastic and Reconstructive Surgery 2020年第2期2卷 107-108,119页

作者： Ping CHEN Liansheng ZHONG Department of Dermatology XuZhou Children’s HospitalXuZhouChina. Department of Dermatology Children’s hospital of Fudan University Xiamen BranchXiamenChina.

aplasia cutis congenita(ACC)is a rare disease that is characterized by complete or partial absence of skin at birth,either in a localized or widespread *** nevi refers to tumor-like malformations of the skin or mucous membrane caused by benign proliferation of *** is classified as a giant congenital melanocytic nevus(GCMN)when the diameter of the largest nevus exceeds 20 *** co-occurrence of ACC and GCMN is extremely rare,to the best of our *** report a case of coexistence of ACC and GCMN of infancy in a 2-month-old male *** lesions consisted of a large hyperpigmented plaque occupying most of the trunk and pelvic region,and smaller hyperpigmented plaques on the trunk,head,and ***,there were large,sharply marginated,triangular,depressed atrophic plaques covered by thin,translucent,glistening epithelial membranes in the center of the GCMN on the *** presumptive diagnosis was coexistence of GCMN and *** could be a manifestation of SCALP syndrome,a rare neuro-cutaneous condition characterized by the presence of Sebaceous nevus,Central nervous system(CNS)malformations,aplasia cutis congenita,Limbal dermoid and Pigmented(giant melanocytic)nevus.

关键词： aplasia cutis congenita giant congenital melanocytic nevus neurocutaneous melanosis melanoma

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Type VI aplasia cutis congenita: About a Case Report at University Teaching Hospital of Bouaké

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Open Journal of Pediatrics 2023年第1期13卷 146-152页

作者： Yao Kouassi Christian Yeboua Yao Kossonou Roland Yenan John Patrick Akanji Iburaima Alamun Adou Leioh Romeo Sahi Gnantin Josette Landryse Amani Ehi Alexise Eleonore Avi-Siallou Christelle Honorine Aka-Tanoh Koko Aude Hélène Asse Kouadio Vincent Department of Pediatrics Universiy Teaching Hospital of Bouaké Bouaké Ivory Coast

Introduction: aplasia cutis congenita is a rare congenital dermatosis of which type VI represents the Bart’s syndrome. The aim of this case is to describe the epidemiological, clinical, therapeutic and prognostic characteristics of this condition in a country with limited resources, for the improvement of prognosis and professional practice. Observation: This is a eutrophic newborn, born at term by vaginal delivery, who presented at birth with a unilateral absence of skin on the anteromedial aspect of the right leg starting from the knee and extending to the medial aspect of the right foot, with a dystrophy of the nail of the right big toe without any other visible physical malformation. The evolution was marked at D3 of life by the appearance of bullae on the right hand and elbow as well as on the posterior aspect of the neck, making epidermolysis bullosa suspect. The mother was 38 years old, 8th gesture, 7th pare with history of consanguinity and collodion baby. The association of a localized congenital absence of skin on the lower limbs, epidermolysis bullosa and a nail anomaly led to the diagnosis of congenital cutaneous aplasia of type VI of Frieden’s classification or Bart’s syndrome. The evolution was satisfactory on the 7th day of life with the beginning of scarring. The management was medical. The outcome was unfavorable with the appearance of sepsis and hemorrhage leading to death. Conclusion: Although rare, the clinical diagnosis of Bart’s syndrome is simple. However, the management is complex and the prognosis is reserved. To improve this prognosis, the treatment must guarantee excellent control of the infectious and hemorrhagic risks, an adhesion and good therapeutic compliance by the parents and a rigorous monitoring.

关键词： aplasia cutis congenita Bart’s Syndrome Newborn Prognosis Côte d’Ivoire

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Bone Defect of the Cranial Vault: Difficulty of the Diagnostic about a Case, and Revew of Literature

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Open Journal of Modern Neurosurgery 2023年第1期13卷 33-40页

作者： Broalet Maman You Espérance Konan Landry Moulot Martial Olivier Esso Didier Bankole Sanni Département de Neurochirurgie Saint Joseph Moscati Catholic Hospital Yamoussoukro C&#244 te d’Ivoire Département de Neurochirurgie Centre Hospitalier Universitaire Yopougon C&#244 te d’Ivoire Département de Chirurgie Pédiatrique Centre Hospitalier Universitaire Treichville C&#244 te d’Ivoire

The bone defects of the cranial vault encompassed rare malformations including acalvaria, hypocalvaria, acrania, hypocrania, anencephaly and exencephaly. They are also described in some pathological entities such as aplasia cutis congenita of the scalp. We report an unusual case of cephalic malformation which combine defects of the skin, the dura mater, and the bones of the vault, with a malformation of the central nervous system. This unique case emphasizes a problem of nosological definition between the terms mentioned above. acalvaria, the acrania, the hypocalvaria and the aplasia cutis congenita. Thus, herein, we proceed to a literature review of bone defects of the skull and their differential diagnosis.

关键词： Cranial Vault Defects Acalvaria Hypocalvaria aplasia cutis congenita congenital Malformation Diagnosis

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