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Biomedical subjects

R Russell Jones

Publications and source records attributed to R Russell Jones.

At least 19 recordsLinked to original sources

Systemic involvement in scleredema of Buschke associated with IgG-kappa paraproteinaemia.

Scleredema is a rare primary cutaneous mucinosis. Systemic involvement is uncommon and histological confirmation is often lacking. We report a case of a 60-year-old man with scleredema and evidence of mucin deposition on biopsies from multiple extracutaneous sites. The bone marrow, nerve, hepatic and salivary gland involvement seen on histology in our patient has not, to our knowledge, been previously reported in this condition.

Bone Marrow↗

Clinical and pathological heterogeneity in cutaneous gamma-delta T-cell lymphoma: a report of three cases and a review of the literature.

Cutaneous gamma-delta (gamma delta) T-cell lymphoma is rare. Eleven cases have been reported to date including four cases of mycosis fungoides (MF), two of pagetoid reticulosis and five of pleomorphic cutaneous T-cell lymphoma (CTCL). We report three further cases of cutaneous gamma delta T-cell lymphoma; one of MF, one of a pleomorphic CTCL and one of a subcutaneous T-cell lymphoma. Combined data suggest that although cutaneous gamma delta T-cell lymphomas do not appear to comprise a single clinicopathological entity, they may be associated with aggressive clinical behaviour and a poor prognosis.

Adult↗

HIV-associated eosinophilic folliculitis: case report and review of the literature.

We report human immunodeficiency virus (HIV)-associated eosinophilic folliculitis in a previously well 40-year-old Portuguese lady. This condition, although similar to Ofuji's disease, has distinguishing features which allow the two to be differentiated. To the best of our knowledge, this is the first reported case of HIV-associated eosinophilic folliculitis in a female and in a patient in whom the HIV was heterosexually transmitted. Our patient is also unusual in being, we believe, only the second reported in whom this eruption was the presenting feature of HIV infection.

AIDS-Related Opportunistic Infections↗

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.

Adult↗

Lupus vulgaris--a case report.

Although lupus vulgaris is the most common form of cutaneous tuberculosis seen in Europe, the incidence has steadily declined and it is now rarely seen. We report a case in a 69-year-old lady who presented with a 10-year history of a slowly growing plaque on the left cheek. Skin biopsy confirmed caseating granulomata, and acid-fast bacilli were seen. She was commenced on triple antituberculous therapy with good effect.

Aged↗

Bullosis diabeticorum. A case report and literature review.

We report a case of bullosis diabeticorum with blisters confined to the lower legs and feet. Histology of a lesion demonstrated a bulla at the dermo-epidermal junction, and ultrastructural studies confirmed the split to be at the level of the lamina lucida which we propose is the site of the pathology in this condition. Immunofluorescence studies were negative, excluding an immunobullous disease. We postulate that in our patient a combination of increased venous pressure and poor vascular supply in diabetic skin, led to the blister formation. The literature on this condition is reviewed.

Aged↗

Immunostaining for CD31 and CD34 in Kaposi sarcoma.

AIMS: To evaluate antibodies directed against CD31 (JC70/A) and CD34 (QBEND/10 and anti-HPCA-1) more extensively in Kaposi sarcoma; to assess their value in routine diagnosis; and to compare them with the traditional endothelial cell markers Ulex europaeus agglutinin 1 (UEA-1) and factor VIII related antigen. METHODS: Twenty four cases of Kaposi sarcoma were studied retrospectively. All specimens had been fixed in formalin and embedded in paraffin wax. The antibodies were applied using the Streptavidin biotin technique in all cases except for UEA-1, for which an indirect two stage method was used involving peroxidase conjugated anti-ulex as the secondary antibody. RESULTS: Tumours were classified into those showing angiomatoid or lymphangiomatoid elements and spindle cell lesions. Universal labelling of all lesions and virtually all elements within lesions was seen with the anti-CD34 antibodies QBEND/10 and HPCA-1. Labelling of spindle cells was less consistent with JC70/A but both markers were superior to the traditional endothelial cell markers UEA-1 and factor VIII related antigen. CONCLUSIONS: These data confirm that Kaposi sarcoma is a tumour of endothelial cell origin. They shed further light on the histogenesis of this complex tumour and demonstrate that immunostaining for CD34 and CD31 can be used as an aid to diagnosis in routinely processed tissue.

Antigens, CD34↗

Verruciform xanthoma: an immunocytochemical study.

Verruciform xanthoma is a rare lesion that occurs predominantly on the oral mucosae, but also on other mucosal sites and on the skin. We report an immunocytochemical analysis of the xanthoma cells in three cases of verruciform xanthoma (two vulval and one scrotal), and also attempt to identify human papilloma virus (HPV) as a possible trigger for the production of these lesions. We employed a panel of seven histiocytic markers (CD68 [KP1], KiM1P, HAM 56, lysozyme, vimentin, peanut agglutinin and factor X111a) and two others to identify HPV involvement (CAMVIR-1 and bovine papilloma virus-1 [BPV-1]). Results showed the xanthoma cells to be positive for CD68, KiM1P, HAM 56 and vimentin, with less consistent labelling for peanut agglutinin and lysozyme. CAMVIR-1 and BPV-1 were negative in all three cases. These findings support the view that the xanthoma cells are derived from a monocyte macrophage lineage and fail to demonstrate HPV as the cause.

Adolescent↗

Bullous eruption of systemic lupus erythematosus: a clinicopathological study of four cases.

We describe the clinical, histological and immunopathological features of four female patients with the bullous eruption of systemic lupus erythematosus (bullous SLE). Three patients had circulating anti-basement membrane zone (BMZ) antibodies, and Western blot analysis in two cases revealed binding to type VII collagen. Immunoelectron microscopy in one of these patients demonstrated deposition of antibody in the lamina densa and sublamina densa regions, thus sharing immunopathological features with epidermolysis bullosa acquisita (EBA). The vesiculobullous lesions developed 8 months to 6 years after the initial symptoms of SLE, and cleared promptly with dapsone treatment.

Adult↗

Clinicopathological significance of cutaneous epidermal nuclear staining by direct immunofluorescence.

Epidermal antinuclear antibody (ANA) staining was noted during routine direct immunofluorescence (DIF) of skin biopsies from 22 cases at St John's Dermatology Centre over a 2-year period. We have reviewed the clinical, serological and immunopathological features of these patients. They comprised 13 cases of lupus erythematosus (LE), 3 dermatomyositis, 1 morphoea, 1 systemic sclerosis, 1 CREST syndrome, 1 mixed connective tissue disorder and 1 probable cutaneous sarcoidosis. Five (38.4%) patients with LE had moderate to severe oral mucosal involvement. Epidermal nuclear staining (ENS) was seen following IgG deposition in 21 cases and IgA in only 1 case. Complement C3 staining was an additional feature in 1 patient. Circulating ANA was absent in 7 cases at the time of biopsy, confirming that this pattern of staining does not occur as a result of tissue contamination during processing. The presence of ENS by DIF corroborates a diagnosis of a connective tissue disorder, and our results suggest that it may also be associated with oral involvement in L.E.

Adolescent↗

Epidermolysis bullosa simplex Dowling-Meara: troublesome blistering and pruritus in an adult patient.

A 46-year-old woman with the Dowling-Meara variant of epidermolysis bullosa simplex (DM-EBS) presented with worsening recurrent, pruritic, circinate crops of clear and haemorrhagic herpetiform blisters affecting her trunk and limbs. Electron microscopy showed tonofilament aggregation and an intra-epidermal level of blister formation confirming a diagnosis of DM-EBS rather than an acquired immunobullous disorder. Antihistamines failed to control the intense pruritus, but dapsone (up to 150 mg daily) was beneficial. Mutations of basal cell keratin genes (K14 and K5) are thought to be of aetiopathological significance in this form of epidermolysis bullosa, but the underlying cellular mechanisms responsible for the clinical deterioration and severe itching in this adult patient are not yet clear.

Blister↗