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Aplasia cutis congenital (ACC) is a rare, heterogeneous congenital disorder.
Abdominal aplasia cutis congenital: case reprt and review of literature.
Aplasia cutis congenita of the trunk in a Saudi newborn.
In addition to the classical combination of transverse limb anomalies and ACC, other malformations such as cardio-vascular, pulmonary and orofacial defects have also been reported with AOS.2,3 Furthermore, there is a phenotypic overlap with other well-characterized malformations like Poland syndrome (OMIM 173800), cutis marmorata telangiectatica congenita (OMIM 219250), and aplasia cutis congenita (OMIM 107600), which make its diagnosis challenging and may compromise accurate genetic counseling and risk estimation.1 Hence, a thorough assessment of clinical presentation is warranted.
Some cases of aplasia cutis congenita will have a so-called hair collar sign, which is a ring of dark hair that often surrounds a scalp lesion.
Adams and CP Oliver in 1945.1-3 It is a clinical syndrome associated with aplasia cutis congenita (ACC), which is classified into nine groups according to location, hereditary mode and associated anomalies.
Aplasia cutis congenita (ACC) is a term used to describe congenital absence or defects of the skin.
Finally we arrived at the diagnosis of Non-syndromic Aplasia Cutis Congenita.
Amongst the less frequent causes, systemic lupus erythematosus (SLE) was seen in 5% cases followed by scleroderma, dermatomyositis, keratosis follicularis spinulosa decalvans (Figure 3), aplasia cutis, kerion, follicular mucinosis, pemphigus, dissecting cellulitis of scalp/ pyogenic folliculitis (Figure 4) and acne keloidalis nuchae in 2.5% cases each.
"Extensive aplasia cutis is usually fatal in the first weeks of life.
Abstract: Aplasia cutis is a disorder characterized by developmental absence of portion of skin.