Mycosis fungoides with a CD56+ immunophenotype.

We report 3 cases of mycosis fungoides (MF) with a CD56+ cytotoxic immunophenotype. Each patient presented with a different clinical phenotype: one exhibited limited poikilodermatous patches (skin stage T1); one, widespread hypopigmented lesions (skin stage T2); and one, poikiloderma with a single cutaneous tumor (skin stage T3). MF was confirmed both histologically and by the presence of a T-cell receptor clone in lesional skin in all cases.

Is disseminated juvenile xanthogranulomatosis benign cephalic histiocytosis?

We describe an infant with a congenital form of non-Langerhans cell histiocytosis with clinical and pathologic features of both disseminated juvenile xanthogranulomatosis and benign cephalic histiocytosis. The findings in this case support the concept of these non-Langerhans cell histiocytoses forming part of a spectrum of disease rather than being separate pathologic entities.

Juvenile sarcoidosis after BCG vaccination.

We report the case of a 2-year-old boy with juvenile sarcoidosis, in whom the cutaneous lesions first arose at the site of and soon after a BCG vaccination. Juvenile sarcoidosis is rare, and the pattern of clinical features is distinct from the adult form of sarcoidosis, possibly related to immunologic development. The cause of sarcoidosis is unknown, although there is much interest in the possibility of mycobacterial species operating as antigenic stimuli to initiate the disease.

Psoriasis, psoriatic arthropathy and relapsing orbital myositis.

Orbital myositis is an inflammatory disorder of the orbital muscles causing orbital pain and restriction of eye movements. Although rare in children, it is most frequently seen after orbital trauma or as a post-infectious process. We describe a child with chronic relapsing psoriasis, juvenile psoriatic arthritis and relapsing bilateral orbital myositis.

Hemostatic abnormalities in Sneddon's syndrome.

Sneddon's syndrome is a rare condition comprising widespread livedo retucularis and multiple episodes of transient cerebral ischemia. Treatment to date has been empirical. The hemostatic/thrombotic status of 4 patients with Sneddon's syndrome was studied by a unique technique, hemostatometry, which measures primary hemostasis (shear-induced platelet plug formation), the overall coagulation, and thrombolysis (dislodgment of the hemostatic plugs) from nonanticoagulated blood.

Relapsing polychondritis: autoimmunity to type II collagen and treatment with cyclosporin A.

A case of relapsing polychondritis (RPC) with strongly positive antibodies to both native and denatured type II collagen is reported. This is only the second report of the use of cyclosporin A in the treatment of RPC.

A cutaneous B-cell lymphoma of novel immunophenotype.

A case of low-grade B-cell malignant lymphoma with a prolonged restriction to the skin is described. A rearrangement of the immunoglobulin heavy-chain gene was detected using molecular techniques. The histology showed many similarities to primary mucosal lymphomas and this may represent a skin counterpart of these tumours.

Langerhans-cell histiocytosis--excellent response to etoposide.

We describe an adult with progressive LCH who received oral etoposide as primary treatment. The response is documented and the strategical implications of this drug in Langerhans-cell histiocytosis discussed. Langerhans-cell histiocytosis (LCH) is the term recognized since 1987 for the group of diseases previously designated histiocytosis X.

Infusion of a stable prostacyclin analogue, iloprost, to patients with peripheral vascular disease: lack of antiplatelet effect but risk of thromboembolism.

Purpose: Prostacyclin, a potent inhibitor of platelet function and vasodilator, has been used to treat peripheral vascular disease. The aim of this study was to monitor the thrombotic status of patients treated by infusion of a stable prostacyclin analogue, iloprost.

Patients And Methods: Thirteen patients with peripheral vascular disease underwent iloprost infusion for 3 days (8 hours each day) in a dose ranging from 0.

Paraneoplastic rhinophyma and the Leser-Trélat sign.

A case of the Leser-Trélat sign associated with an underlying malignant haemangiopericytoma is described. In addition, the patient had profound hypoglycaemia and a rhinophyma-like nasal swelling which rapidly resolved post-operatively. The tumour appeared to be secreting a factor(s) which cross-reacted with both insulin receptors to induce hypoglycaemia and epidermal growth factor receptors inducing a profusion of eruptive seborrhoeic warts.

The spectrum of livedo reticularis and anticardiolipin antibodies.

We document a study of 65 patients presenting to our clinics, over a 2-year period, with livedo reticularis. All patients were screened for the presence of anti-cardiolipin antibodies and assessed for the presence of central nervous system disease and features of the 'antiphospholipid' syndrome, including venous and arterial thromboses and foetal loss. Patients were also assessed for other clinical features such as Raynaud's phenomenon and valvular lesions.

Cholinergic dermographism.

We report a patient with cholinergic urticaria in whom stroking the skin produced a band of erythema studded with the small weals characteristics of cholinergic urticaria. This response was suppressed by pre-treatment with topical scopolamine. Light and electron microscopy of the weal showed mast cell degranulation and a moderate mononuclear cell infiltrate.