Keratolytic winter erythema with facial involvement: a novel presentation.

We present a 23-year-old woman with a diagnosis of keratolytic winter erythema (erythrokeratolysis hiemalis), who developed facial lesions following a traumatic experience. This rare genodermatosis usually affects the palms and soles, and appears as mild erythema and annular scaling. The limbs and trunk can rarely be affected. To our knowledge, this is the first reported case of facial involvement.

Co-existence of lichen planus and sarcoidosis.

We present a 53-year-old patient with respiratory sarcoidosis who subsequently developed cutaneous lichen planus (LP). Biopsy of the skin lesions showed typical LP with no evidence of sarcoid granulomas. To the best of our knowledge the two conditions have not been reported in association.

Contrasting effects of an ultraviolet B and an ultraviolet A tanning lamp on interleukin-6, tumour necrosis factor-alpha and intercellular adhesion molecule-1 expression.

BACKGROUND: Recent studies have demonstrated that a tanning lamp emitting predominantly ultraviolet (UV) A induces significant yields of the type of potentially mutagenic DNA damage that are associated with the onset of skin cancer (i.e. cyclobutane pyrimidine dimers). UV-induced immunosuppression is also an important event leading to skin cancer. OBJECTIVES: To the modulation of key immunological molecules following exposure to a broad-spectrum UVB lamp and a predominantly UVA-emitting tanning lamp using model in vitro systems. METHODS: We compared secretion and mRNA expression of interleukin (IL)-6 and tumour necrosis factor (TNF)-alpha in normal human epidermal keratinocytes, and interferon (IFN)-gamma-induced intracellular adhesion molecule (ICAM)-1 in normal human fibroblasts irradiated in vitro with a broad-spectrum UVB lamp or with a Philips 'Performance' tanning lamp. RESULTS: With broad-spectrum UVB irradiation, upregulation of IL-6 and TNF-alpha mRNA was detected 6 h after irradiation, and a dose-dependent increase of cytokines in the supernatants of irradiated cells was found 24 h after irradiation. In contrast, there was no cytokine secretion and little evidence for mRNA upregulation following exposure to a tanning lamp. When cells were exposed first to broad-spectrum UVB, then the tanning lamp, UVB-induced cytokine secretion was inhibited, although mRNA levels were upregulated to a level close to that observed with UVB alone. By using a Schott WG 320 nm filter to attenuate the level of UVB relative to UVA emitted by the tanning lamp, the inhibition of cytokine secretion was shown to be associated with UVA exposure. Both UV sources inhibited IFN-gamma-induced ICAM-1 mRNA expression in a dose-dependent fashion. By using a Schott WG 335 nm filter, inhibition of ICAM-1 mRNA expression by the tanning lamp was shown to be associated with UVB exposure. CONCLUSIONS: These results suggest that UV sources emitting different levels of UVA and UVB have differential effects on the modulation of different immunoregulatory molecules, and indicate that there are potential interactions between these wavelengths.

Nodular localized primary cutaneous amyloidosis: a long-term follow-up study.

We present long-term follow-up data on patients with nodular localized primary cutaneous amyloidosis (NLPCA) seen at the St John's Institute of Dermatology between 1968 and 1999. This is the largest clinical follow-up study of this type of amyloid to date. Based on these cases we estimate the rate of progression of NLPCA to systemic amyloidosis to be only 7%, much lower than the 50% rate currently quoted in the literature.

Multiple cutaneous immunocytoma with secondary anetoderma: a report of two cases.

We describe two men with multiple erythematous dermal nodules which were clinically and histologically consistent with a diagnosis of primary cutaneous immunocytoma. Both patients exhibited the very unusual feature of secondary anetoderma occurring in spontaneously resolving lesions. There is one previous report of anetoderma in association with a plasmacytoma. The pathogenesis remains unknown but release of cytokines such as interleukin-6 may be implicated.

Complications of trans-sphenoidal surgery: the Wellington experience.

BACKGROUND: All patients who underwent trans-sphenoidal surgery between January 1984 and December 1998 were reviewed to assess morbidity resulting from this operation. METHODS: There were 185 operations on 165 patients. The operative approach was sublabial in 80 cases and transnasal in 105. One surgeon (VB) performed the vast majority of operations. RESULTS: Complications included nasal perforation (7.6%), transient diabetes insipidus (4.9%), permanent diabetes insipidus (3.8%), cerebrospinal fluid fistula (4.3%), donor site haematoma (2.2%) and residual tumour haemorrhage (1.6%) causing ophthalmoplegia (1.1%) and loss of vision (1.1%). Other complications included epistaxis (1.1%), meningitis (0.5%) and sinusitis (0.5%). Injury to the anterior superior alveolar nerve also occurred in the sublabial approach in 6.3% of patients. There were no perioperative deaths. CONCLUSIONS: There is a small but significant risk of a number of complications that should be considered for informed consent of this procedure.

Cutis verticis gyrata of the scalp in a patient with autosomal dominant insulin resistance syndrome.

Cutis verticis gyrata (CVG) is a rare disorder; it is characterized by thickening of the scalp which becomes raised to form ridges and furrows resembling the cerebral gyri. We report a case of CVG associated with the autosomal dominant insulin resistance syndrome. This syndrome is characterized by obesity, mild mental retardation, delayed puberty, acanthosis nigricans and hyperinsulinaemia. The association of CVG and autosomal dominant insulin resistance has not been previously described.

Non-functioning pituitary adenomas: indications for postoperative radiotherapy.

To determine the indications for postoperative radiotherapy after surgical resection of a nonfunctioning pituitary macroadenoma. A retrospective chart review of 72 patients with histologically proven chromophobe adenoma who presented for pituitary surgery between January 1985 and June 1998, with a minimum follow-up period of 12 months. The study endpoint was tumour recurrence or progression detected either by routine follow-up imaging or by clinical progression with subsequent confirmation by imaging. A proportional hazards model was used to determine independent prognostic factors. Mean follow-up was 64 months. In the radiotherapy group 13 of 50 recurred (or progressed) (26%), while in the nonradiotherapy group 10 of 22 recurred (46%), logrank test, P = 0.025. In patients assessed as having complete excision of tumour (n = 20) only two recurred (10%), both in patients without radiotherapy. No further treatment has been required in either case to date. In patients with residual tumour (n = 52), 41 had radiotherapy with 13 recurrences (32%), while 11 patients had no radiotherapy with eight subsequent recurrences (73%); logrank test, P = 0.007. Further treatment has been required in the majority of these cases. Cox's proportional hazards model analysis showed that only complete tumour removal and postoperative radiotherapy were independent favourable prognostic factors. The goal of surgery should be complete surgical excision where possible. The risk of recurrence in patients with no residual tumour on postoperative imaging is low enough to justify withholding routine postoperative radiotherapy in this group. In patients with residual tumour, conventional external beam radiotherapy administered within 12 months of surgery is effective at reducing recurrence or progression.

Michel's medium: a potential alternative to cryoprotection for tissue transport in the investigation of genetic skin disease.

Michel's medium is now well established as a transport medium to maintain tissue-fixed immunoreactants prior to direct immunofluorescence and immunoelectron microscopy. This is a practical alternative to cryofixation prior to transportation when sending skin biopsies for the investigation of cutaneous immunobullous disease. In this study we have demonstrated preservation of the cutaneous basement membrane zone proteins in skin biopsies stored in Michel's medium for periods of up to 28 days - proving that Michel's medium can be used as a transport medium when sending skin biopsies for immunohistochemical studies to determine the structural and molecular deficiencies in genodermatoses such as inherited forms of epidermolysis bullosa.

Necrobiotic cutaneous T-cell lymphoma.

We report 3 patients with granulomatous cutaneous T-cell lymphoma (CTCL) who showed necrobiosis histologically with palisading granulomas. Although granulomatous change may be present in up to 4% of cases of CTCL, necrobiosis is rare. Misdiagnosis may occur if epidermotropism is minimal or if atypical cells are masked by the granulomatous infiltrate. T-cell receptor gene analysis confirmed the presence of clonal T-cell populations in lesional skin from all 3 cases.

Childhood dermatitis herpetiformis: an unusual presentation.

Childhood dermatitis herpetiformis (DH) is rare. The true prevalence and incidence of this condition are unknown. We report a 7-year-old boy presenting with nonpruritic inflammatory papules on the buttocks and extensor surfaces, clinico-pathologically consistent with Sweet's neutrophilic dermatosis. Immunofluorescence studies showed IgA deposits in the dermal papillae consistent with DH. Remission was achieved with a gluten-free diet and dapsone. Childhood DH may present different clinical signs to the adult form and misdiagnosis can occur if immunofluorescence is not requested on skin biopsy.

The 0.8% ultraviolet B content of an ultraviolet A sunlamp induces 75% of cyclobutane pyrimidine dimers in human keratinocytes in vitro.

Tanning lamps, emitting predominantly ultraviolet (UV) A, are used widely throughout the U.K. and other countries, but little is known about the long-term risks associated with their use, especially with respect to skin cancer. We have exposed normal human epidermal keratinocytes to a commercial tanning lamp and used the comet assay in association with DNA repair enzymes T4 endonuclease V and endonuclease III to investigate the relative yields of directly formed cyclobutane pyrimidine dimers (CPDs) and indirectly formed types of oxidative DNA damage. To put the risk of using tanning lamps into perspective, the sunbed used in this study (five Philips Performance 80W-R UVA tubes at a distance of 35 cm) was found to be approximately 0.7 times as potent at inducing CPDs as U.K. natural sunlight around noon on a fine summer day. This compares with a relative risk for CPD induction and erythema of 0.8 and 0.7 times, respectively, calculated from the relevant action spectra of tanning lamps and British noontime sunlight. To determine the relative contribution of UVB and UVA to the induction of CPDs and oxidative DNA damage, we modified the spectral output of the tanning lamps with a series of Schott WG UVB filters. The induction of CPDs was more dependent on the UVB component of the sunbed than oxidative types of damage. Schott WG UVB filters with 50% transmission at 305 nm reduced the yield of T4 endonuclease V sites by 42% while there was only a 17% decrease in the yield of endonuclease III sites. CPD induction was not completely abolished after irradiation through WG335 and WG345 nm filters despite there being no detectable UVB. From these data, it was estimated that, although the tanning lamps emitted only 0.8% of their total output in the UVB range, these wavelengths were responsible for the induction of over 75% of CPDs and 50% of the oxidative damage to DNA.

Xanthoma disseminatum: a case with hepatic involvement, diabetes insipidus and type IIb hyperlipidaemia.

Xanthoma disseminatum (XD) is a rare benign non-X-histiocytic disorder of unknown aetiology. We report a 37-year-old man who presented with XD preceded by a decade of cranial diabetes insipidus, with associated type IIb hyperlipidaemia and computed tomographic evidence of hepatic involvement. A review of the literature is also included.

Chronic endemic hydroarsenicism.

Chronic endemic hydroarsenicism in a 48-year-old man from Antofagasta, Chile, is reported. The literature on the global health problems of hydroarsenicism is reviewed, especially with regard to the carcinogenic action of arsenic.