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A Anstey

Publications and source records attributed to A Anstey.

At least 19 recordsLinked to original sources
PubMed2001

Photomedicine: lessons from the Smith-Lemli-Opitz syndrome.

The Smith-Lemli-Opitz syndrome (SLOS) is an autosomal recessive disorder caused by a discrete block in the cholesterol biosynthetic pathway. Recognition of the metabolic basis of this condition has bought into focus knowledge from fields as diverse as clinical genetics, sterol biochemistry, foetal nutrition, and experimental teratology, resulting in a rush of new insights in these fields. Unfortunately, late recognition of photosensitivity as part of this syndrome has largely excluded photodermatologists and photobiologists from the recent frenzy of research in this disorder. However, clinical features of this new inherited photosensitivity syndrome have now been established, and monochromator light testing has confirmed it to be a UVA-mediated condition. Current knowledge concerning photosensitivity in SLOS is reviewed and future research into the pathogenesis of this disorder is discussed.

Animals↗
PubMed2000

The dermatosis of chronic granulomatous disease.

A family with X-linked cytochrome-negative chronic granulomatous disease (CGD) involving three generations is reported. The diagnosis of CGD in both the latest male patient and the index male was confirmed by marked impairment in polymorphonuclear leucocyte oxidative burst activity in association with absence of both subunits of cytochrome b. The two female carriers have suffered from chronic inflammatory skin disorders characterized by slowly fluctuating erythematous plaques. The reported cases are discussed in the context of a literature review of the dermatosis of CGD.

Child↗
PubMed1999

Home phototherapy: report on a workshop of the British Photodermatology Group, December 1996.

Phototherapy is a popular and effective treatment for many patients with skin diseases. However, repeated journeys to hospital for phototherapy can be inconvenient and expensive. If it were available, many patients might prefer home-based phototherapy as long as it was safe and effective. Indeed, many psoriasis patients already self-treat with ultraviolet A sunbeds at home. This report represents a consensus view from a British Photodermatology Group workshop held in December 1996, the purpose of which was to examine the potential role of home-based phototherapy in dermatological practice. We conclude that home-based therapy represents a suboptimal treatment with greater attendant risks than phototherapy in a hospital environment. The level of medical supervision of the home treatment is crucial to its safety and effectiveness. Until further studies are forthcoming, home phototherapy should be largely restricted to those with overwhelming difficulties in attending hospital.

Home Care Services↗
PubMed1999

Ultraviolet radiation-blocking characteristics of contact lenses: relevance to eye protection for psoralen-sensitised patients.

Nine brands of contact lens marketed as "UV protective" were tested for ultraviolet (UV) transmission in order to assess potential suitability for psoralen-sensitised patients. UV-transmission characteristics of hydrated lenses was tested with a Bentham monochromator spectro-radiometer system. All lenses showed minimal transmission loss in the visible band. The performance of the nine lenses was uniform for ultraviolet B radiation with negligible transmission, but showed variation in transmission for ultraviolet A radiation. None of the lenses complied with UV-transmission criteria used previously to assess UV-blocking spectacles. Only two lenses had UV-blocking characteristics which came close to the arbitrary criteria used. The performance of ordinary soft and hard lenses was very similar, with negligible blocking of UV radiation. None of the nine contact lenses marketed as "UV protective" excluded sufficient UVA to comply with criteria in current use to assess UV protection in spectacles for psoralen-sensitised patients. However, the improved UV-blocking characteristics of contact lenses identified in this paper compared to previous studies suggests that such a contact lens will soon become available. Meanwhile, contact lens-wearing systemically sensitised PUVA patients should continue to wear approved spectacles for eye protection whilst photosensitised with psoralen.

Contact Lenses↗
PubMed1998

Photosensitivity associated with the Smith-Lemli-Opitz syndrome.

A case of severe photosensitivity in a girl with the Smith-Lemli-Opitz syndrome is reported. Children with this recessively inherited metabolic disorder of cholesterol metabolism present with a variety of congenital abnormalities of the nervous system and internal organs in association with varying degrees of mental retardation. Photosensitivity is a feature which has previously only briefly been mentioned in the literature in association with this syndrome. However, more recently, it has become apparent that photosensitivity is not uncommon among children with the Smith-Lemli-Opitz syndrome, although the nature of the photosensitivity in these patients has remained undefined. Our patient has suffered from sunlight intolerance since early infancy, with redness and pruritus of sun-exposed skin developing within minutes of sun exposure. Monochromator ultraviolet (UV) radiation and visible light testing revealed an immediate and persistent reaction to low-dose UVA at 350 nm, and an abnormal erythemal response to visible light at 400 nm.

Child, Preschool↗
PubMed1996

In vivo photoinduction of metallothionein in human skin by ultraviolet irradiation.

The aims of this study were to confirm and substantiate the in vivo cutaneous induction of metallothionein (MT) in human skin by UVR, which we have reported in brief previously, and to make a preliminary attempt to characterize the time course of this phenomenon. Buttock skin in 32 volunteers was irradiated with 2 MED of UVB and biopsies were taken at 24 h from matched non-irradiated and irradiated sites. In the kinetic study, skin biopsies from six volunteers were taken at 0, 2, 8, 24, and 48 h after 2 MED UVB irradiation. MT was immunolocalized in formalin-fixed, paraffin-embedded tissue with the monoclonal antibody E9 by an indirect immunoperoxidase method. Statistically significant differences between immunocytochemical scores were identified between non-irradiated (NI) and irradiated (I) skin within suprabasal keratinocytes (mean: NI = 1.2, I = 5.1; P = 0.01), superficial dermal fibroblasts (mean: NI = 2, I = 43; P < 0.001), mid-dermal fibroblasts (mean: NI = 0, I = 27; P < 0.001), and deep dermal fibroblasts (mean: NI = 0, I = 11; P < 0.001). In the kinetic study, no consistent rise in MT score with time was observed for the epidermal component. In dermal fibroblasts, however, the first statistically significant rise in immunocytochemically detectable MT was detected at 2 h and this was found to plateau beyond 8 h. These results confirm that ambient levels of UV irradiation are capable of inducing MT in human skin in vivo. Taken together with the relative rapidity of the response, this suggests a physiological photoprotective role for MT in human skin cells. The lack of a kinetic increase in epidermal MT may be due to high basal levels. Induction of MT in dermal fibroblasts may reflect the effects of a diffusible factor released from keratinocytes after UVR.

Buttocks↗
PubMed1994

Desmoplastic malignant melanoma. An immunocytochemical study of 25 cases.

Using a panel of seven cell markers, we studied the value of immunocytochemical labelling in the histological diagnosis of desmoplastic malignant melanoma. Sections from routine formalin-fixed tissue of 45 surgical specimens were obtained from 25 cases of malignant melanoma that showed well-marked desmoplastic or neurotropic features. Routinely stained sections (Haematoxylin- and -eosin and melanin stains) were compared with the following panel of seven antibodies: S-100, neuron-specific enolase (NSE), vimentin, factor XIIIa (FXIIIa), desmin and the newer, supposedly more specific antimelanoma antibodies HMB45 and NKIC3. S-100 and NSE were the most sensitive antibodies for desmoplastic malignant melanoma with strong labelling of spindle cells in most cases. In contrast, results for NKIC3 were more variable; results were negative in nearly half the tumours, but strong labelling was seen in six cases (27%). Positive labelling for HMB45 was noted in five tumours (22%); it was mostly confined to small groups of cells in the superficial part of the lesions. Tumour spindle cells were negative for FXIIIa in all cases; there was no increase in the number of positive dermal dendritic cells compared to conventional and spindle cell melanoma. All tumours were desmin-negative, but most were vimentin-positive. Our findings indicate that immunocytochemistry is of less value in the diagnosis of desmoplastic malignant melanoma that it is with other types of malignant melanoma. However, positive or negative labelling for S-100 protein and NSE is useful for suggesting or excluding a diagnosis of desmoplastic malignant melanoma; neither marker is specific and, in particular, positive labelling is also found in most neurofibromas and benign cellular naevi.(ABSTRACT TRUNCATED AT 250 WORDS)

Adult↗
PubMed1993

Desmoplastic malignant melanoma: a clinicopathological study of 25 cases.

Sixteen cases of malignant melanoma which showed prominent desmoplastic and/or neurotropic features occurring throughout the tumour were compiled from the St John's Dermatology Centre histopathological archives. A further nine melanomas in which both conventional and desmoplastic melanoma were present concomitantly were also studied (three tumours with 66% desmoplastic change, two with 50%, and four with less than 50%). There were 14 males and 11 females, with a mean age of 64 years (range 39-86). The mean interval between presentation and diagnosis was 8 months. Eighteen of the 25 tumours were located on the head and neck, three were on the trunk, one was on the upper limb and three were on the lower limb. Histological review revealed 21 of 25 tumours with overlying atypical lentiginous hyperplasia, lentigo maligna melanoma, or superficial spreading malignant melanoma. Neurotropism was present in nine tumours, with the changes confined to local recurrences in two instances; neuroid differentiation was present in four tumours, and neural and perineural tumour spread was present in four tumours. The depth of invasion exceeded 6 mm in seven tumours, and was 2-6 mm in 16, and less than 2 mm in two. Eighteen of the 25 tumours were incompletely excised at the time of the first excision. Lymphoid aggregates were present in 16 tumours, but in most cases were limited to a few lymphoid foci. Melanin was identified in the dermal component of only five tumours, but not in areas showing typical histological features of desmoplastic malignant melanoma. Treatment was by surgical excision in all cases, and was preceded by radiotherapy in one case. Details of follow-up were obtained in all cases, and the duration ranged from 9 months to 10 years (mean, 3 years 11 months). Eleven patients had died; nine from melanoma and two from other causes. One patient was alive, with deep, inoperable local recurrence. Thirteen patients were alive and clinically free from tumour, including two patients in whom there had been local recurrence. A lower rate of neurotropism was present in the nine patients with partial desmoplastic change compared with those with desmoplasic change throughout the tumour, and represented the only significant difference between the two groups of patients.

Adult↗
PubMed1993

Isotretinoin-associated pseudoporphyria.

We present two patients who developed pseudoporphyria whilst taking isotretinoin (Roaccutane). At that time, both patients were receiving other agents which could be incriminated as causing their pseudoporphyria. However, it is significant that these other agents had been longstanding and that the porphyria-like cutaneous changes did not develop until after the introduction of the isotretinoin (Roaccutane). In both cases, withdrawal of the isotretinoin resulted in clinical improvement of their cutaneous signs. The interaction of isotretinoin and another agent, rather than isotretinoin independently, may be involved in the development of pseudoporphyria.

Adult↗