Cases reported "Hypertrichosis"

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1/13. Congenital generalized terminal hypertrichosis with gingival hyperplasia.

    Congenital generalized terminal hypertrichosis is a rare disease, especially when associated with gingival hyperplasia. Congenital hypertrichosis can be a clinical feature of several syndromes, so these patients must be studied by a multidisciplinary team that should include a dermatologist, geneticist, psychologist, odontologist, and an endocrinologist. We report a 7-year-old girl with congenital generalized hypertrichosis and gingival hyperplasia, and analyze the clinical approach, differential diagnosis, and treatment.
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2/13. hypertrichosis terminalis, gingival hyperplasia, and a characteristic face: a new distinct entity.

    Congenital generalized hypertrichosis terminalis has been described in association with other features as gingival hyperplasia, osteochondrodysplasia, and a dysmorphic face. Bondeson and Miles [1993: Am J Med Genet 47:198-212] described a woman with universal congenital hypertrichosis terminalis associated with gingival hyperplasia; the face of this patient was coarse and different from other forms of hypertrichosis described before. We present an 11-year, 6-month-old girl with universal congenital hypertrichosis terminalis, gingival hyperplasia, and a characteristic coarse face resembling the patient described by Bondeson and Miles [1993: Am J Med Genet 47:198-212]. We propose that this type of congenital generalized hypertrichosis terminalis, associated with gingival hyperplasia and a coarse face, is a distinctive new entity.
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keywords = gingival
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3/13. Zimmermann-Laband syndrome: further clinical delineation.

    Zimmermann-Laband syndrome (ZLS) is an autosomal dominant disorder characterized by gingival fibromatosis, absent or dysplastic distal phalanges, vertebral defects, hepatosplenomegaly, hypertrichosis and sometimes mental retardation. We describe two unrelated patients, a girl aged 9 years and a boy 11 months whose clinical and radiological findings permit us to diagnose the ZLS. Body overgrowth, present in both patients, was identified as a main clinical feature not previously reported as well as the presence in neuroimaging studies of a cavernous hemangioma on the frontal and the left cerebellar regions in the boy. The girl also presented important radiological characteristics such as broad medulary canals and metaphyses of long bones, thin cortices, broad ribs, accelerated skeletal maturation as well as high intelligence level. A wide clinical spectrum in ZLS is also considered.
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keywords = gingival
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4/13. Idiopathic gingival fibromatosis associated with mild hypertrichosis.

    A case of gingival fibromatosis with hypertrichosis is reported in a 10 year old girl. The condition is a syndrome and presents as a triad of gingival fibromatosis, hypertrichosis and mental retardation. Following surgical management, no recurrence was seen in the immediate post-surgical period. Even if recurrence occurs excision is recommended as the psychological benefits resulting from cosmetic improvement outweigh the danger of recurrence.
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5/13. Idiopathic familial gingival fibromatosis associated with mental retardation, epilepsy and hypertrichosis.

    Gingival fibromatosis, a rare but often familial condition, is described in two siblings, associated with mental retardation, epilepsy and hypertrichosis. In one child a maxillary giant-cell tumour was found and excised. It is important to distinguish idiopathic gingival fibromatosis from phenytoin-induced gingival hypertrophy.
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6/13. Congenital hypertrichosis lanuginosa: neonatal shaving.

    A newborn baby presented with overgrowth of lanugo hair and a precocious tooth. She was shaved infrequently until aged 9 months, when spontaneous loss of body hair began. pyloric stenosis at 6 weeks was surgically corrected. There was a family history of hypertrichosis and dental anomalies.
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keywords = dental
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7/13. Gingival fibromatosis with hypertrichosis. A case report.

    A case of gingival fibromatosis with hypertrichosis is reported in a 3-year-old girl. Partial recurrence was observed 6 months after surgical treatment. Psychological benefits resulting from the cosmetic improvement may outweigh the probability of recurrences in this rare condition.
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8/13. Congenital marked hypertrichosis and Laband syndrome in a child: overlap between the gingival fibromatosis-hypertrichosis and Laband syndromes.

    Gingival fibromatosis may be reported as an isolated finding or associated with a number of distinct and frequently inherited group of disorders. The characteristics of the Laband syndrome include gingival hyperplasia, dysplasia of the terminal phalanges and nails of extremities, hepatosplenomegaly and facial dysmorphism. Another well-known syndrome with gingival fibromatosis associates generalized hypertrichosis and inconstant mental retardation and epilepsy. We report a case with features of Laband syndrome and congenital marked hypertrichosis, suggesting overlap between these two genetic disorders.
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keywords = gingival
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9/13. hypertrichosis universalis congenita: a separate entity, or the same disease as gingival fibromatosis?

    hypertrichosis universalis congenita is an extremely rare disorder characterized by generalized hypertrichosis. It is generally accepted as being inherited as an autosomal dominant trait with varying expression. Many aspects of this disease are still unknown. Several reports associating hypertrichosis and gingival fibromatosis raise the question of whether they are separate entities or the same disease with different expressions of the underlying process. hypertrichosis universalis congenita occurred in a 6-year-old girl without known family history. Her facial features were simian-like and her gingiva was moderately hyperplastic. We pose the question of whether or not these phenomena are related.
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keywords = gingival
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10/13. hypertrichosis, coarse face, brachydactyly, obesity and mental retardation.

    A 17-year-old boy with a complex array of dysmorphic features including generalized hypertrichosis of the terminal hair, coarse face, short stature, obesity, brachydactyly with broad proximal phalanges, small and dyschromic nails, and mental retardation is reported. Virilizing endocrinopathies, obesity/mental retardation syndromes, and conditions with hirsutism associated with small or absent nails are discussed and particular emphasis is given to the syndrome of "generalized hypertrichosis terminalis and gingival hyperplasia" which shares some features with the patient.
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