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Trichoscopy
Published in Aimilios Lallas, Zoe Apalla, Elizabeth Lazaridou, Dimitrios Ioannides, Theodosia Gkentsidi, Christina Fotiadou, Theocharis-Nektarios Kirtsios, Eirini Kyrmanidou, Konstantinos Lallas, Chryssoula Papageorgiou, Dermatoscopy A–Z, 2019
Aimilios Lallas, Zoe Apalla, Elizabeth Lazaridou, Dimitrios Ioannides, Theodosia Gkentsidi, Christina Fotiadou, Theocharis-Nektarios Kirtsios, Eirini Kyrmanidou, Konstantinos Lallas, Chryssoula Papageorgiou
Aplasia cutis congenita (ACC) is the result of congenital focal aplasia of the epidermis and dermis. It clinically presents as a smooth, yellowish, solitary area of focal permanent alopecia, and it is usually accompanied by other defects, enabling early diagnosis. The differential diagnosis includes alopecia areata, nevus sebaceous, and cicatricial alopecia secondary to mechanical trauma.
Conditions
Published in Sarah Bekaert, Women's Health, 2018
Aplasia cutis – a rare disorder with a complicated pattern of inheritance, in which babies are born with the absence of certain layer(s) of skin, most often on the scalp, but also on the trunk and/or arms and legs. The affected area is typically covered with a thin transparent membrane. The skull and/or underlying areas may be visible and abnormally developed. Aplasia cutis congenita may be the primary disorder, or it may occur in association with other underlying disorders.
Genodermatoses affecting the nail
Published in Eckart Haneke, Histopathology of the NailOnychopathology, 2017
The histopathology of aplasia cutis congenita depends on the time of biopsy. Fresh lesions exhibit a complete lack of the dermis and epidermis or just contain a flat epidermis with hyperkeratosis. With time there is scar tissue that develops into fibrosis and later sclerosis. The nail dystrophy itself has not yet been examined histopathologically. Immunohistochemical examination shows that collagen IV is localized at the roof of blistered skin.192 Electron microscopy demonstrates that the anchoring fibrils are poorly developed.
Aplasia cutis congenita: a case report
Published in Journal of Obstetrics and Gynaecology, 2019
Seyma Banu Arslanca, Tufan Arslanca, Acar Koç
Aplasia cutis congenita (ACC) is a rare congenital disease characterised by varying degrees of absence of skin and subcutaneous tissues (Bajpai and Pal 2003). This is most commonly observed on the scalp; however, it can be observed on the forearms, the knees, on either side of the body, or on the neck (Koshy et al. 2001). ACC can be accompanied by hypoplasia or missing muscles or bones (Verhelle et al. 2004). At birth, the appearance of lesions may vary from superficial erosion to deep ulcers, with the affected areas covered with a thin, transparent membrane; lesions may or may not be isolated. Diseases that may be comorbid with might ACC include skin, eye, ear, nose, and extremity abnormalities, and cardiovascular, gastrointestinal, genitourinary, and central nervous system defects (Magliah and Alghamdi 2018). Lesions appear early in pregnancy and may heal before birth, leaving a congenital atrophic alopecic scar. This report will present a case of ACC accompanied by epidermolysis bullosa, in which antenatal ultrasound (USG) revealed structural deformities.
Aplasia Cutis Congenita with Ischemic Cortical Change and Normal Array Cytogenetic Analysis with a Fetus Papyraceus Twin
Published in Fetal and Pediatric Pathology, 2018
Laura A. Skillen, Damien Gates, Julie-Ann Collins, Nivedita Saxena, Daniel Hurrell, Kevin McKenna, Patrick J. Morrison
Aplasia cutis congenita often presents with a range of types and causes. The two main classifications include the Frieden classification of nine groups according to defect location and associated features [1], and the Sybert dermatogenetic classification [2] with five main dermatological subtypes (Table 1). Causes include specific chromosomal syndromes and dermatogenetic disorders. Frieden group 5 and Sybert type III both refer to the association of ACC with fetus papyraceus (FP; the loss of one fetus during a twin pregnancy in the late first or early second trimester). Around 50 reports of ACC with FP (ACC-FP) have been documented but no definite cause has been identified and cases are generally isolated case reports or occasional descriptive case series without genetic testing concentrating on the live twin [3–10]. Several etiological theories have been suggested and debate is uncertain as to the exact etiology and recurrence risks [11–15]. We describe twins with a surviving twin having ischemic change on brain magnetic resonance imaging (MR) with aplasia cutis congenita. A focal ischemic event due to in-utero hypovolemia resulting in the second twin forming into a fetus papyraceus is a possible cause.
A neonate with a facial congenital pressure injury: a case report
Published in Case Reports in Plastic Surgery and Hand Surgery, 2022
Nanouk van der Sluis, Britt Theodora Wilhelmina ten Hoope, Tess Bosch, Marie Elise Wiesman, Ute Schmidbauer, Hinne Albert Rakhorst
In the differential diagnosis for non-iatrogenic pressure injuries one must rule out aplasia cutis congenita (ACC). ACC is a rare congenital skin defect with an incidence of approximately 1–3 out of 10,000 births. It involves solitary or multiple lesions with focal or extensive absence of epidermis, dermis, sometimes subcutaneous tissue and 80 percent of the lesions are localized to the vertex of the scalp [12]. During a good clinical exam, when attention is paid to both location and type of skin defect, ACC can be discriminated from congenital pressure injury.