Search PubMed⌕ Search

Biomedical subjects

R Russell-Jones

Publications and source records attributed to R Russell-Jones.

102 records · Page 6Linked to original sources

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. This emphasises the variable disease expression and the importance of recognising this condition so that patients can be followed up with regard to complications, and genetic counselling can be offered. The issue of prenatal diagnosis is discussed.

Family↗

HHV8 and female Kaposi's sarcoma.

Kaposi's sarcoma (KS) is an enigmatic tumour of uncertain histogenesis. Epidemiological data have long suggested that KS may be caused by an infectious agent, possibly sexually transmitted. Following the documentation of human herpesvirus 8 (HHV8) and its strong association with all forms of KS, it now appears that the putative agent has at last been identified. As KS is rare in females, a unique group was screened for the presence of HHV8 using both conventional solution-phase polymerase chain reaction (PCR) and the newly described technique of TaqMan PCR. The presence of HHV8 was demonstrated in 10/12 of these female patients. This further supports the direct role of HHV8, in conjunction with cytokines and other factors, in the pathogenesis of KS.

Adolescent↗

Thalidomide-induced toxic pustuloderma.

We report a case of toxic pustuloderma secondary to thalidomide in a patient with severe nodular prurigo. To our knowledge this is the first reported case. With the increasing use of thalidomide for a variety of dermatological conditions it is clearly important that this rare side-effect now be recognised.

Adult↗

Management of cutaneous lymphoma.

The foregoing underlines the advances which have been made in our understanding of cutaneous lymphoma and the areas where further research is needed. With a few noteable exceptions the aim of therapy in CTCL is palliative rather than curative and treatment success is measured in terms of disease-free interval. There is still no evidence that any chemotherapeutic regimen prolongs survival. A possible exception is the effect of photopheresis in Sézary syndrome but our own experience differs from that in the USA and underlines the need to identify patients with clonal disease when defining subjects for study. The combination of genotypic analysis and new treatment methods offers exciting new prospects in the management of patients with cutaneous lymphoma.

Humans↗

Neutrophil zinc levels in psoriasis and seborrhoeic dermatitis.

The median zinc content of neutrophils was significantly reduced in 16 patients with psoriasis in comparison to both normal controls and six patients with seborrhoeic dermatitis (P less than 0.05). This reduction was unrelated to the extent of skin involvement. Plasma and erythrocyte zinc levels were unchanged.

Adult↗

Naevoid psoriasis.

A six-year-old boy presented with an eruption comprising multiple psoriasiform plaques, arranged in linear bands distributed along the lines of Blaschko and confined to the left side of the body. We believe that the eruption is true psoriasis, occurring in an unusual naevoid distribution. The existence of a genuinely naevoid form of psoriasis has frequently been debated, and has tended to be discounted. We believe that we have encountered a child who has this condition.

Child↗

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. These findings, in combination with the clinical picture, favor a diagnosis of Herlitz JEB. Immunohistochemistry findings greatly facilitated an accurate diagnosis, which is essential in view of the poor prognosis for patients with this form of junctional epidermolysis bullosa.

Epidermolysis Bullosa, Junctional↗