Congenital herpes simplex virus infections. Report of three cases and review of the literature.

Three infants with congenital herpes simplex virus infection were examined and treated. One demonstrated findings of individual vesicles on an erythematous base; another had pustules. The children were treated with vidarabine intravenously. Two of the three survived. The literature suggests a potential increase in the occurrence of this infection in neonates. Since the dermatologist is often asked to aid in the diagnosis of this entity and early recognition is vital, it is important to be familiar with the possible causes of vesicopustular lesions, as well as the epidemiology, clinical manifestations, pathogenesis, and current forms of treatment of congenital herpes simplex virus infections.

Tuberous sclerosis: clinicopathologic features and review of the literature.

INTRODUCTION: Tuberous sclerosis is a hamartoneoplastic syndrome, which may involve multiple organ systems. Oral hard tissue manifestations of the syndrome have been described in the literature only as recently as 1955. Patients who presented with clinical manifestations of tuberous sclerosis did not routinely undergo oral surveys to rule out 'lesions', and consequently data on 'lesions' in the maxillofacial complex is scant. Ten cases have been found in the English language literature, which describe maxillofacial 'lesions', which may be tumours, new growths, neoplasms or overgrowths occurring in patients diagnosed with tuberous sclerosis. PURPOSE: To review the literature for all maxillofacial lesions associated with tuberous sclerosis and to present an eleventh case of a patient with a maxillofacial lesion diagnosed as having tuberous sclerosis. RESULTS: Eleven cases were found with maxillofacial fibroblastic lesions associated with tuberous sclerosis. These lesions were all fibrous benign neoplasms found in the maxillofacial bony complex. CONCLUSIONS: Maxillofacial fibroblastic lesions in tuberous sclerosis have various histopathological presentations, some of which may be difficult to differentiate. Consequently, close microscopic examination of these lesions is necessary so that adequate surgical treatment is provided.

Congenital plate-like osteoma cutis of the forehead: an atypical presentation form.

Cutaneous ossifications or osteoma cutis can be found in many syndromes. Primary osteoma cutis, present since birth or the first months of life, in the absence of metabolic disorders or trauma, is found in congenital plate-like osteoma cutis and progressive osseous heteroplasia, coexisting in the latter with deep connective tissue ossifications. This report documents the case of a 7-year-old female with a plate-like cutaneous ossification of the forehead causing aesthetic deformities. Other dermal ossifications in the inguinal and preauricular region, as well as the left hand and a small focus in the extraocular muscles of the left orbit, were also present. These lesions did not show progression, and most of them were present since birth. The lesion of forehead was treated surgically. The lack of progression and the fact that the orbital lesion was solitary still fits the criteria for the diagnosis of plate-like osteoma cutis. Plate-like osteoma cutis and progressive osseous heteroplasia may represent varieties of the same disorder.

Use of distortions in the treatment of skin lesions of the face: the distorting excision.

Reconstruction after excision of a cutaneous lesion of the face is done by various complex old and new flaps to avoid any distortion. The distortions following primary closure of defects in the face are given undue importance. We have observed and used the capacity of self-correction of these distortions, which is partially due to the muscular actions. We show our experience in various distorting reconstructions of the face in 36 patients. Because of our partial presence in a children's hospital, most of our patients were children.

Olmsted syndrome: report of two new cases and literature review.

Olmsted syndrome is a rare keratinization disorder; 18 cases have been published so far. It associates a mutilating cogenital palmoplantar keratoderma with periorificial erythematokeratotic lesions. We report herein two new unrelated male children with Olmsted syndrome (OS), one of whom was studied by light and electron microscopy. Our histological, immunohistochemical, and ultrastructural findings suggest that this disease is related to epidermal hyperproliferation. We present herein a review of the twenty cases published so far and discuss the major clinicopathological and genetic features of this disease.

Olmsted syndrome--a rare syndrome with oral manifestations.

Olmsted syndrome is a rare, congenital condition characterized by severe palmo-planter keratosis, periorificial keratosis, and hypotrichosis. Though orofacial keratosis is one of the consistent findings of Olmsted syndrome, it has never been reported in the dental literature. We report a case of Olmsted syndrome in an eight-year-old boy who presented with massive and crippling palmoplanter keratosis and bilateral oral lesions in the form of keratotic plaques at the corners of the mouth, as well as on the dorsum of the tongue.

[Nevus of Ota. Apropos of a bilateral case]. / Le naevus de Ota. A propos d'un cas bilatéral.

A case of bilateral congenital oculodermal melanocytosis is reported. Ota's naevus is characterized by benign melanosis of the skin of the face in the area supplied by the first (and second) branch of the trigeminal nerve. This is associated with ipsilateral pigmentation of the episclera++, uveal tract, conjonctiva and fundus. The condition is usually unilateral, occurring mainly in the dark races (black races and orientals). Eighty percent of patients are women. It is very rarely potentially malignant, this occurring most frequently in white races and when only ocular (and not oculodermal) melanocytosis is present.

[Pili multigemini: a pilar dysplasia with linear disposition]. / Pili multigemini: une dysplasie pilaire à disposition linéaire.

BACKGROUND: Pili multigemini is an uncommon pilar dysplasia with linear disposition which could be explained by the pattern of Blaschko's lines. CASE REPORT: A 37-year-old man with no medical history developed pili multigemini over a heavily bearded chin. DISCUSSION: Mili multigemini is an uncommon developmental defect of hair follicles resulting from hairs with multiple matrices and papillae emerging through a single pilosebaceous canal. This defect has a linear distribution on the chin we found to follow Blaschko's lines. Pili multigemini has been observed in association with a few rare malformations. Treatment is difficult.

Parental experiences with propranolol versus oral corticosteroids for complicated infantile hemangioma, a retrospective questionnaire study.

BACKGROUND: Infantile hemangiomas (IHs) are common and mostly emerge in the head-neck area. Recently, propranolol has been replacing oral corticosteroids (OCS) as the main treatment modality. OBJECTIVES: The aim of this study was to explore the impact of treatment, contentment with treatment outcome and quality of life for families and patients with cervicofacial IHs, treated with propranolol versus OCS. MATERIALS AND METHODS: This study was performed using questionnaires administered by a phone interview. Parents of 16 patients with a cervicofacial IH treated by OCS and 16 patients with an IH of similar localization and overall severity treated with propranolol were interviewed. The questions concerned the impact of treatment at different time periods and the contentment with treatment results. Parents were also asked to give a quality of life (QoL) score (1 to 10) for different time-points. RESULTS: Parents from the OCS group seemed to feel significantly more worried during treatment. Moreover, parents from the propranolol group perceived less negative impact on normal life issues, including work and vaccination of their child. During and after treatment, the parents of propranolol-treated IH patients gave significantly higher QoL scores. CONCLUSION: Propranolol seems to change the impact of IHs, their treatment and the quality of life. Propranolol treatment interferes less with normal issues in daily life, compared to OCS. These findings underline propranolol as the first choice treatment for life- or function-threatening IHs.

Congenital facial linear porokeratosis.

We report a unique case of congenital linear porokeratosis with exclusive facial involvement in a 27-year-old Chinese man. No other family member was affected. To our knowledge, this is the first report in the English literature of congenital linear porokeratosis confined to the face.

Congenital erosive and vesicular dermatosis.

An 8-year-old boy born prematurely at 26 weeks' gestation presented with a reticulate scar on the left cheek. This started as an eroded area on day 3 postpartum, followed by crusting and scarring. There were also linear scars on the left forearm, left lower back and abdomen. The clinical picture fits the entity of congenital erosive and vesicular dermatosis. This retrospective case report delineates the outcome with diagnosis only evident after an 8-year time period.

Neonatal lupus erythematosus.

We present a case of neonatal lupus erythematosus (NLE) in a black infant presenting with symmetrical depigmented macules on the face resembling vitiligo. NLE is a rare condition affecting newborn infants of mothers who have connective tissue disease, with or without autoantibodies to extractable nuclear antigens Ro (SS-A), La (SS-B) or ribonucleoproteins. Infants present with cutaneous lesions or congenital heart block or both. The skin lesions are usually annular and erythematous and transient and resemble those of subacute cutaneous lupus erythematosus. The presentation of this patient was therefore striking.