Inflammatory myopathy in a patient with cutaneous findings of pityriasis rubra pilaris: a case of Wong's dermatomyositis.

We report the case of a 46-year-old woman presenting cutaneous lesions similar to those of pityriasis rubra pilaris, characterized by diffuse scaling erythroderma and palmoplantar keratoderma; skin biopsy showed follicular hyperkeratosis with a perivascular lymphocytic infiltrate. One year later she developed an inflammatory myopathy; on the basis of clinical features, a diagnosis of dermatomyositis was made. Treatment with prednisone and hydroxychloroquine led to complete control of the cutaneous and muscular involvement, which was maintained during prednisone tapering.

Primary cutaneous large B-cell lymphoma of the leg: histogenetic analysis of a controversial clinicopathologic entity.

This study analyzes the pathologic and molecular features of 5 cases of primary cutaneous large B-cell lymphoma of the leg (PCLBCL-leg), recently included in the European Organization for Research and Treatment of Cancer (EORTC) classification of primary cutaneous lymphoma. PCLBCL-leg accounts for 5% to 10% of all primary cutaneous B-cell lymphoma (PCBCL), usually affects elderly patients and carries a worse prognosis than other forms of PCBCL. It has been proposed that the malignant cells of PCLBCL-leg originate from germinal center (GC)-related cells, but their effective normal counterpart is unclear, and the rationale behind the inclusion of this lymphoma as a separate entity is based on its prognosis rather than on its proved histogenesis.

Primary cutaneous cryptococcosis in an HIV-negative patient.

We report a case of primary cutaneous cryptococcosis in an HIV-negative patient, who presented with painless, ulcerated lesions involving the right forearm (fingers and elbow), which developed over 45 days. On the basis of the clinical appearance, serological and cultural examinations were performed to confirm the diagnosis; the histological evaluation of a skin biopsy showed an acute inflammatory infiltrate containing several PAS + Cryptococci. Subsequently, the patient was treated with fluconazole (400 mg/day for 10 days, then a maintenance therapy of 200 mg/day); after one month, the cutaneous lesions were remarkably improved, but, although a series of further laboratory and clinical examinations was scheduled, the patient repeatedly refused any other re-evaluation, and he was lost from follow-up.

Feasibility and safety of a new technique of extracorporeal photochemotherapy: experience of 240 procedures.

Background And Objective: Extracorporeal photochemotherapy (ECP) is a therapeutic approach based on the biological effects of ultraviolet light (UV) - A and psoralens on mononuclear cells collected by apheresis. Recently, ECP has been under investigation as an alternative treatment for various immune and autoimmune diseases. The aim of this study was to evaluate the safety and feasibility of a new three-step ECP technique, in terms of reproducibility, acceptance, tolerability, and short and long term side effects.

Blood transfusions in the therapy of a case of prolidase deficiency.

A case is reported of a 15-year-old boy with prolidase deficiency and marked urinary excretion of the iminodipeptide gly-pro. Prolidase activity of erythrocytes against substrate glycyl-proline was deficient, but after blood transfusions this was increased to 15.7% of donor activity and declined to 12% and 3.