Incontinentia pigmenti: variable disease expression within an affected family.

We report a florid case of incontinentia pigmenti in a neonate in which linear vesiculobullous, verrucous and pigmented lesions were present simultaneously at birth. Histology of a vesiculobullous lesion showed vesiculation with numerous eosinophils in the epidermis, and a sparse infiltrate in the dermis with pigmentary incontinence. The mother of our patient described a streaky linear rash on her legs during her own childhood which resolved spontaneously, in addition to partial anodontia, suggesting that she too has the disease, although previously undiagnosed.

Physiological secretion of chemokines in human breast milk.

Human breast milk has been observed to contain high concentrations of the chemotactic cytokines (chemokines) interleukin-8 (IL-8) and RANTES. Concentrations are greatest in colostrum, but are measurable in milk after several months of lactation. These chemokines are also found in the secretions of patients with galactorrhoea and in the "witch's milk" of the newborn.

The clinical significance of T-cell superantigens.

Superantigens are a diverse collection of molecules that share the ability to activate specific lymphocyte subsets. In animal models, many of these molecules delete T-cell populations or render them anergic. The clinical impact of superantigens is significant, but much of their pathophysiology remains unclear.

The quality of operation notes: can simple word processors help?

In a retrospective study the quality of operation notes on specifically designed proformas was compared to those produced by word processors and predesigned templates and notes in patients undergoing operations for colorectal cancer. The operations in the two groups were similar. Computer notes scored higher on all criteria, were all legible and took the same time to generate.

Mortality and morbidity from diabetes in South Asians and Europeans: 11-year follow-up of the Southall Diabetes Survey, London, UK.

Over 20% of middle aged and elderly South Asian people throughout the world have diabetes. The associated mortality and morbidity risks are unclear. We compared mortality and morbidity in a cohort of South Asian and European people with diabetes in London, UK, in an 11-year follow-up of a population-based sample of 730 South Asians (mean age 55 in 1984) and 304 Europeans (mean age 67 in 1984) with diabetes aged 30 years and above in 1984.

Herlitz junctional epidermolysis bullosa: a case report and review of current diagnostic methods.

We report an infant with Herlitz junctional epidermolysis bullosa (JEB) presenting at birth with erosions on the scalp, thigh and periumbilical area in addition to nail abnormalities. Ultrastructural studies demonstrated a split through the lamina lucida with poorly formed hemidesmosomes and no clearly defined subbasal dense plates. Indirect immunofluorescence staining with antibodies GB3 (antilaminin 5) and 19-DEJ-1 (antiuncein) was totally absent.

Helicobacter pylori infection and upper gastrointestinal patholgy in a British immigrant Indian community.

Objectives: To assess the pattern of upper gastrointestinal pathology and the prevalence of Helicobacter pylori infection in the Southall Indian community.

Design: A prospective study of endoscopic findings in 124 Indian and 107 whites from the Southall area. In a separate study blood samples were taken from 100 Indian subjects presenting to a single general practitioner in Southall.

A comparison between epidural anaesthesia using alkalinized solution and spinal (combined spinal/epidural) anaesthesia for elective caesarean section.

In a double-blind investigation, 40 women undergoing elective lower segment caesarean section were randomly divided into two groups. Group I (n = 20) received spinal anaesthesia with 2.0 ml hyperbaric 0.

Mycosis fungoides with spontaneously regressing CD30-positive tumorous lesions.

Mycosis fungoides (MF) is the most common cutaneous T-cell lymphoma. We report a case in which identical T-cell clones were identified in both patch and tumour stage lesions and in which the tumorous deposits, containing CD30-positive cells, regressed spontaneously. We discuss the differential diagnosis of the tumorous lesions and the spectrum of CD30-positive proliferative T-cell disorders.

Sweet's syndrome in association with non-Hodgkin's lymphoma.

Sweet's syndrome is an acute neutrophilic dermatosis frequently found in association with other conditions, particularly inflammatory and neoplastic disease. We report here a patient who developed the condition 2 years after the diagnosis of non-Hodgkin's lymphoma (NHL), in this case affecting a tonsil, the thirteenth report of such an association. We discuss the diagnosis, investigation and management of Sweet's syndrome in the context of the current case.

Diagnostic accuracy and appropriateness of general practitioner referrals to a dermatology out-patient clinic.

A study was undertaken of new referrals by GPs to a dermatology clinic in a district general hospital over a 6-month period. Six hundred and eighty-six consecutive referrals to one consultant were analysed for diagnostic accuracy and requirement for referral. Only 47% of referral letters contained the correct diagnosis.

An acquired-pseudo Bernard Soulier syndrome occurring with autoimmune chronic active hepatitis and anti-cardiolipin antibody.

A case of autoimmune chronic active hepatitis with unusual extrahepatic manifestations is described. The patient exhibited marked thrombocytopenia and platelet aggregation dysfunction and morphological changes suggesting an acquired Bernard Soulier-type syndrome. This has not previously been described in association with chronic active hepatitis.

Successful use of a transjugular intrahepatic portosystemic stent shunt to control severe refractory oesophageal variceal haemorrhage in a poor risk patient.

This report describes a 44 year old man with a severe gastrointestinal haemorrhage from oesophageal varices. Bleeding could not be controlled with conservative therapy and sclerotherapy. He was successfully treated with a radiologically guided transjugular intrahepatic stent shunt at a time when his condition was too poor to attempt an open surgical procedure.

Pustular drug eruptions: a histopathological spectrum.

We have reviewed the histopathological features of 11 patients with pustular drug eruptions. Two main histological patterns were seen. Eight cases revealed features of toxic pustuloderma with the presence of spongiform intraepidermal pustules in association with papillary oedema and a mixed inflammatory cell infiltrate around upper dermal blood vessels.