1/8. Multiple congenital malformations including generalized hypertrichosis with gum hypertrophy in a child exposed to valproic acid in utero.Fetal valproate syndrome results from in utero exposure to valproic acid. It is characterized by a distinctive facial appearence, a cluster of minor and major anomalies, and central nervous system dysfunction. We report on a child exposed prenatally to valproic acid with unusual anomalies. This patient was the first child of young parents. Mother had several generalized seizures one year before this pregnancy, and since than she took valproic acid. pregnancy was otherwise uneventful. At birth physical examination showed generalized hypertrichosis sparing palms and soles, coarse face, gum hypertrophy, hypotonia, club feet and club hands, two annular constrictions of the right lower leg, and abnormal dermatoglyphics. Skeletal x-rays were normal. Gum hypertrophy and hypertrichosis may be part of a broader pattern of altered morphogenesis in fetus exposed to valproic acid or this patient had two conditions, fetal valproate syndrome and hypertrichosis with gum fibromatosis.- - - - - - - - - - ranking = 1keywords = fibromatosis (Clic here for more details about this article) |
2/8. 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.- - - - - - - - - - ranking = 1keywords = fibromatosis (Clic here for more details about this article) |
3/8. 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.- - - - - - - - - - ranking = 6keywords = fibromatosis (Clic here for more details about this article) |
4/8. 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.- - - - - - - - - - ranking = 6keywords = fibromatosis (Clic here for more details about this article) |
5/8. 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.- - - - - - - - - - ranking = 5keywords = fibromatosis (Clic here for more details about this article) |
6/8. 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.- - - - - - - - - - ranking = 6keywords = fibromatosis (Clic here for more details about this article) |
7/8. 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.- - - - - - - - - - ranking = 5keywords = fibromatosis (Clic here for more details about this article) |
8/8. Localized hypertrichosis associated with periorbital neurofibroma: clinical findings and differential diagnosis.Background: Congenital localized hypertrichosis in the periorbital region is an uncommon finding. The authors report two patients with hypertrichosis and cutaneous hyperpigmentation overlying a periorbital neurofibroma. methods: In addition to a complete ophthalmic and systemic examination, the patients underwent computed tomography of the head and biopsy of the tumor. Results: Case 1 previously had received a diagnosis of neurofibromatosis type I. On examination, hyperpigmentation, hypertrichosis, and swelling in the right supraorbital region were noted. A computed tomographic scan showed a tumor in the same region. The tumor was removed, and a plexiform neurofibroma was diagnosed. Case 2 was admitted with hyperpigmentation, hypertrichosis, and swelling of the left half of her face. Other signs of neurofibromatosis were absent. A computed tomographic scan showed a tumor, which was underlying the skin changes. Results of histologic examination of the biopsy specimen showed a plexiform neurofibroma. Conclusion: neurofibroma-associated hypertrichosis should be considered in the differential diagnosis of congenital localized hypertrichosis.- - - - - - - - - - ranking = 2keywords = fibromatosis (Clic here for more details about this article) |