ANCA associated with Behçet's disease.

Behçet's disease is a multisystem disorder affecting primarily mucocutaneous and ocular sites although the gastrointestinal, cardiovascular, central nervous and respiratory systems may also be involved. Hulusi Behçet, a Turkish dermatologist, first described Behçet's disease in 1937 and suggested a possible infectious aetiology. The pathogenesis of this condition still remains unclear although it is likely that infection acts as a trigger in genetically susceptible individuals.

Treatment of extramammary Paget's disease by radiotherapy.

Extramammary Paget's disease (EMPD) is a rare cutaneous malignancy, which usually occurs in the elderly. Wide local excision is the recommended treatment, although this may not always be feasible. We report our experience of EMPD treated by radiotherapy in five patients.

Antineutrophil cytoplasmic antibodies and their relevance to the dermatologist.

The term vasculitis embraces a heterogeneous group of conditions which may occur as primary phenomena or secondary to disorders such as rheumatoid arthritis or systemic lupus erythematosus. Classification of vasculitis is often difficult in the absence of identifiable aetiological factors or specific serological markers. However, the primary systemic vasculitides can be segregated morphologically, according to the size of blood vessels involved and the presence of granulomata (Table 1).

Erythrodermic mycosis fungoides treated with total body irradiation and autologous bone marrow transplantation.

We describe a middle-aged man with aggressive erythrodermic mycosis fungoides whose responses to several well-established therapies for the disease were either poor or short-lived. Infusion of antibodies engineered against cells expressing the CD4 molecule produced little response. Total body irradiation coupled with autologous bone marrow transplantation led to an encouraging but unfortunately unsustained improvement.

Measurement of mRNA for E-selectin, VCAM-1 and ICAM-1 by reverse transcription and the polymerase chain reaction.

Stimulation of cultured human umbilical vein endothelial cells by cytokines such as interleukin-1 and tumour necrosis factor induces de novo synthesis and expression of the adhesion molecules E-selectin, vascular cell adhesion molecule-1 (VCAM-1) and intercellular adhesion molecule-1 (ICAM-1). In general, alterations in cell surface expression of these molecules are known to be related to increased gene transcription and altered levels of mRNA. The extension of these observations to the study of inflammatory processes in different human organs necessitates the development of techniques for the quantification of mRNA in small tissue samples.

Comparison of cell adhesion molecule expression in cutaneous leucocytoclastic and lymphocytic vasculitis.

Aims: To compare the expression of the cell adhesion molecules intercellular adhesion molecule-1 (ICAM-1), ELAM-1 (E-selectin), and vascular cell adhesion molecule-1 (VCAM-1) in cutaneous leucocytoclastic and lymphocytic vasculitis.

Methods: Immunohistochemical analysis was performed on early lesional skin biopsy specimens of leucocytoclastic vasculitis (n = 14), lymphocytic vasculitis (n = 10), non-lesional skin (n = 12), and normal skin (n = 5). A standard immunoperoxidase technique was used to detect expression of ICAM-1, E-selectin, VCAM-1, and the cell markers CD11a, CD11b, CD11c, von Willebrand factor, CD3, CD68, and neutrophil elastase (NP57).

Multiple lentigines confined to psoriatic plaques.

The development of naevus spilus-like hyperpigmentation at sites of resolving plaques of psoriasis has been reported previously. Although the mechanism is not understood, PUVA therapy has been implicated in most cases. We report an additional case in which lentigines, confined to sites of resolving psoriasis, occurred following oral PUVA.

A trial of oral zinc supplementation in psoriasis.

A controlled double-blind study of oral zinc supplementation was performed in twenty-five patients with chronic plaque psoriasis over twelve weeks to assess changes in both psoriasis (using the psoriasis area and severity index) and neutrophil zinc content. There were no statistically significant differences in the psoriasis area and severity index during the trial between the placebo- and zinc-treated group, nor in the zinc levels. There was therefore no evidence of a benefit from zinc supplementation in patients with this disease.

Venous ulcer presenting as a pathological fracture.

The case of a 71-year-old woman is reported, who neglected a chronic venous ulcer, and presented to the orthopaedic surgeons with a pathological fracture of her tibia due to an invasive squamous cell carcinoma arising from the ulcer. Malignant degeneration of venous ulcers is fortunately rare. However, this case emphasizes the need for regular assessment of long-standing venous ulcers, and illustrates that they may present as an acute emergency to other specialities.

Scleroderma and the carcinoid syndrome.

The follow up of a case of the carcinoid syndrome complicated by scleroderma is reported, in which progress of the disease may have been halted by treatment with a combination of cyproheptadine, parachlorophenylalanine and prednisolone. Impairment of tryptophan and 5-hydroxytryptamine (serotonin) metabolism appears central to the development of skin fibrosis in the carcinoid syndrome and may be indicative of important mechanisms in the pathogenesis of idiopathic scleroderma.

Auricular embryonal rhabdomyosarcoma.

Rhabdomyosarcoma is an uncommon tumour that may present at a wide variety of different sites. We report a 4-year-old girl who developed an embryonal rhabdomyosarcoma arising in the left pinna which was clinically indistinguishable from a lymphangioma. The case illustrates that this neoplasm can be easily misdiagnosed because of its variable morphology.

Cutaneous vasculitis presenting on the penis.

Cutaneous vasculitis is frequently located on the lower limbs. We describe a patient who developed palpable purpura affecting the penis as the presenting sign of more widespread lesions of Henoch-Schönlein purpura.