Cases reported "Scalp Dermatoses"

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1/58. The wide spectrum of clinical expression in Adams-Oliver syndrome: a report of two cases.

    Two children are described with the combination of aplasia cutis congenita (ACC) and transverse limb defects known as Adams-Oliver syndrome. Whereas in the first child the typical features of ACC, syndactyly and transverse nail dystrophy were only mildly expressed and associated defects of the central nervous system and cardiac malformations were absent, the second child suffered from a very severe expression of the syndrome, with a combination of ACC, syndactyly, cutis marmorata telangiectatica congenita and multiple cardiac and central nervous system malformations which resulted in fatal central respiratory insufficiency.
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ranking = 1
keywords = aplasia cutis congenita, aplasia cutis, cutis congenita, cutis, congenita, aplasia, defect
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2/58. Cutis verticis gyrata (case reports of rare causes).

    Two cases of cutis verticis gyrata (CVG) one in association with a saccular aneurysm of the internal carotid artery and another with intraventricular ependymoma are reported. Not only is CVG rare, its association with intracranial aneurysms has not been reported in the literature. This paper documents those rare associations and gives a short review of CVG.
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ranking = 0.0041025261655006
keywords = cutis
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3/58. Primary pachydermoperiostosis: a case report.

    Pachydermoperiostosis (PDP), a rare genodermatosis, occurred in a 38-year-old Indian male. He presented with progressive thickening of the skin on the face and scalp of 15 years duration. Widening of his wrists and ankles and broadening of the fingers and toes had also developed since then. He was born of a consanguineous marriage and had no family history of a similar disorder. He had the typical findings of complete form of PDP including cutis verticis gyrata, coarse facial features, clubbing of the digits in the skin, and periostosis and cortical thickening at the distal ends of long bones of the extremities and small bones of the hands and feet. PDP has two different forms--primary and secondary. These two entities are differentiated by family history and presence or absence of a primary lesion, usually in the lungs. Clinically, in secondary PDP, the cutaneous findings (pachydermia, seborrhoea, oiliness) are less severe than primary PDP; osteoarthropathy is more severe and painful in secondary PDP, especially with congenital heart disease. The present case was suffering from primary PDP that had expressed itself in its complete form.
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ranking = 0.0062552553226952
keywords = cutis, congenita
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4/58. Aplasia cutis congenita with precancerous transformation - the first case. Why do these scars never develop invasive tumors?

    The term aplasia cutis congenita characterizes a heterogeneous group of diseases which have in common a focal absence of the skin. The defect may be limited to the epidermis but often involves the full thickness of the skin including the underlying bone. At birth the lesions present as erosive patches and they heal rather rapidly with a residual scar. Although more than 200 publications on aplasia cutis congenita have appeared in the medical literature between 1966 and 1999, surprisingly no case of malignant degeneration has been described. We observed a 58-year-old male patient with aplasia cutis congenita who developed crusted changes within the scar over the past 10 years. Repeated biopsies over the years have always documented a precancerous lesion without solar elastosis. Invasion has never been observed in this patient. We hypothesize that for invasive malignancies dermal-epidermal interactions are necessary. Such a cell to cell communication seems to be impossible in patients with aplasia cutis congenita, as the dermal-epidermal unit is not developed. Aplasia cutis congenita might serve as an interesting model for further investigations on the importance of epidermal-dermal interactions.
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ranking = 5.106767213371
keywords = aplasia cutis congenita, aplasia cutis, cutis congenita, cutis, congenita, aplasia, defect
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5/58. psoriasis in a patient with neurofibromatosis.

    A seven-year-old boy with neurofibromatosis who developed scalp psoriasis is presented. The clinical evaluation of the patient revealed multiple cafe au lait spots, axillary freckling, bilateral Lisch nodules and a psoriatic plaque on his scalp. Though there is no known direct relationship between neurofibromatosis and psoriasis, one is suggested in this patient, possibly related to a common genetic defect.
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ranking = 1.4215435833481E-5
keywords = defect
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6/58. Stellate purse-string closure.

    BACKGROUND: There are fundamental concepts we use in managing surgical defects. Whether planning a primary closure or a local flap, we frequently modify the basic design to maximize aesthetic outcomes, taking into consideration a number of factors including the location of the defect and tissue availability. OBJECTIVE: We describe the stellate modified purse-string closure, a novel flap modification. METHOD: Report of an illustrated case. RESULT: A patient with vertex scalp defect was reconstructed using the stellate purse-string flap. CONCLUSION: Certain modifications of commonly used reconstructive techniques can be utilized in specific situations to enhance cosmesis. Advantages of this modification are discussed.
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ranking = 4.2646307500443E-5
keywords = defect
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7/58. Closure of a scalp defect.

    In repairing cutaneous defects, the facial plastic and reconstructive surgeon is faced with many specialized areas of tissue. Reconstruction of thin eyelid skin and thick, sebaceous nasal skin requires different methods. The unique characteristics of the scalp make it one of the greatest repair challenges in the head and neck region, sometimes requiring multiple different reconstructive techniques for the same defect.
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ranking = 8.5292615000887E-5
keywords = defect
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8/58. microcephaly, cutis verticis gyrata of the scalp, retinitis pigmentosa, cataracts, sensorineural deafness, and mental retardation in two brothers.

    We describe the cases of two brothers with microcephaly, primary cutis verticis gyrata of the scalp, prominent supraorbital ridges, large nose, hypertelorism, exotropia, progressive retinitis pigmentosa, cataracts, sensorineural hearing loss, kyphoscoliosis, and mental retardation. A review of the literature focusing on the major clinical findings suggests that our cases may represent a hitherto unreported new syndrome.
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ranking = 0.020512630827503
keywords = cutis
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9/58. Necrotizing fasciitis of the scalp in a neonate.

    We report an 11-day-old baby who presented with necrotizing fasciitis of the scalp from which escherichia coli was cultured. Treatment consisted of administration of parenteral broad-spectrum antibiotics and debridement. skin grafting of the resulting scalp defect was not permitted by the parents. The wound healed with scar tissue over 3 months.
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ranking = 1.4215435833481E-5
keywords = defect
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10/58. Surgical treatment of aplasia cutis in the Adams-Oliver syndrome.

    Aplasia cutis is one of the features of the Adams-Oliver syndrome, beside limb anomalies. Aplasia cutis, congenital absence of skin, is a lesion that usually presents over the vertex of the skull. Management of aplasia cutis depends on the size of the skin defect and the child's physical condition. scalp defects larger than approximately 1 cm should be treated surgically. In patients with aplasia cutis, surgery should preferably be performed using rotation scalp flaps, and additional split skin grafts. The history of two babies with the Adams-Oliver syndrome is presented. In the Adams-Oliver syndrome, large rotation scalp flaps are not reliable due to the abnormal vascularity of the skin. skin grafting is the safest way, preventing hemorrhage and infection, in the operative treatment of aplasia cutis in these babies.
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ranking = 1.7877514666136
keywords = aplasia cutis, cutis, congenita, aplasia, defect
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