Inherited epidermolysis bullosa.

Inherited epidermolysis bullosa (EB) refers to a clinically and genetically heterogeneous group of rare disorders characterized by fragility of the skin and mucous membranes. Despite the preclinical development of different molecular and cell-based treatment strategies, no cure is still available for EB. Therefore it is important to establish the management of EB patient since neonatal age in order to ensure an early diagnosis and contribute to prevent complications and to improve quality of life. A review of literature was performed underlining the specific attention to coordinated multidisciplinary approach. The major topics treated comprise the multidisciplinary approach to EB patients, global skin care including wound care, management of itching and pain, and early diagnosis of squamous cell carcinoma. The rarity of EB and the variable involvement of several organs and systems challenge the appropriate treatment of these patients. The care of EB requires a coordinated multidisciplinary approach. The team should be specifically trained and a regular follow-up is required to prevent cutaneous and systemic complications. A well-organized and structured continuity of care is important in EB like in all chronic and rare diseases. The management of EB patients is very important because a specific attention to the fragile skin is required in order to reduce pain, risk of trauma, ulceration and infection. The center of expertise stays as the main structure offering specialized care to EB patients and also helps patients and their families to understand and deal with the disease and to ensure an adequate liaison with the community healthcare system.

Safety and efficacy of Adalimumab in the treatment of moderate to severe palmo-plantar psoriasis: an open label study.

INTRODUCTION: Psoriasis of the hands and feet is highly debilitating and difficult to treat. Lesions are very painfull, disabilitating and impair quality of life of patients. Most treatment options have limited efficacy, short duration of response and several adverse events. OBJECTIVE: To investigate the safety and efficacy of Adalimumab in the management of palmo-plantar psoriasis. PATIENTS AND METHODS: Adults patients with moderate to severe palmoplantar psoriasis were enrollend in this trial. They received a 6 courses of Adalimumab 40 mg 1 vial every 2 weeks. The study consisted of treatment period of 12 weeks (Weeks 1-12). Safety and efficacy were assessed at weeks 0.6 and 12. PGA (Physician's Global Assesment) and DLQI were used to measure the efficacy. Primary end point of the study was to evaluate patients who achieved a reduction in PGA at week 12. The secondary end point was to evaluate patients who achieved a 50% reduction in PGA at week 12. The tertiary end point evaluated patients who achieved a PGA rating of clear or almost clear. RESULTS: Of 11 patients enrolled 6 showed overall improvement of at least one point of PGA at week 12; 4 of them obtained a PGA of 0 while 5 patient of 11 a ≥ 50% improvement from the beginning of the study. 8 patients showed an increase in quality of life score while receiving the drug at week 12. No serious adverse events were reported during the study. CONCLUSION: Continuous treatment with Adalimumab for 12 weeks was safe and efficacious in this open-label clinical trial of patients with palmoplantar psoriasis.

Efficacy of electrochemotherapy in ulcerated basal cell carcinoma.

Basal cell carcinoma is the most common cutaneous malignant tumor, accounting for up to 80% of non melanoma skin cancers. Surgery, radiotherapy and chemotherapy have been for long time the main options for its treatment. Electrochemotherapy (ECT) is a novel local treatment successfully used in primary skin tumors. We report a case of a man affected by ulcerated basal cell carcinoma treated with ECT. In our case ECT was successful in the management of extensive basal cell carcinoma in clinical conditions whereas other approaches, would have been dangerous and inappropriate. To our knowledge, ECT must be considered as an alternative of traditional techniques when they are contraindicated in relation to the appearance of the lesions or the patient medical history.

Fusariosis and skin T cell lymphoma: concomitant more than a differential diagnosis.

Cytotoxic T cell lymphomas of the skin include a spectrum of a peripheral T cell and natural killer (NK) cell lymphomas with primary and secondary skin manifestation and bad prognosis. Fusarium species have recently emerged as the second most common pathogenic fungi in immunocompromised patients, and they are moderately resistant to most antifungal agents. We report a woman with concomitant cytotoxin T cell lymphomas of the skin and Fusarium spp infection. Patient was treated at the same time with antiblastic and antifungal therapy. First line antifungal therapy was amphotericin B-lipid complex (3 mg/Kg iv/die) and then for clinical failure voriconazole (6 mg/Kg bid, loading dose and 4 mg /Kg bid). Lymphoma was treated with a CHOEP 21 regiment without remission and after with gemcitabine and vinerolbine. Patient presented a partial remission of cutaneous and pulmonary lesions. Our case is intrinsically interesting because Fusarium infection was concomitant to cutaneous lymphoma and did non occur during neutropenic phases of chemotherapy. In a case with multiple ulcerated nodules of the skin is very important to discriminate from disseminated cutaneous Fusarium infection and neoplastic conditions such as cutaneous lymphoma. Early treatment of Fusarium infection in a patient with neoplastic disease could avoid a dissemination during immunosuppressive condition caused by antiblastic therapy.

Folgoration as an Example of Pathergy in a Patient Affected by Pyoderma Gangrenosum and Takayasu's Arteritis.

Pyoderma gangrenosum (PG) is a neutrophilic dermatosis of unknown aetiology. Clinical manifestations of PG are characterized by destructive, necrotizing, and noninfective ulceration of the skin. 20-30% of cases are initiated and aggravated by minor trauma or surgery, a phenomenon named pathergy. PG is related to several autoimmune diseases including ulcerative colitis, Crohn's disease, rheumatoid arthritis, and monoclonal gammopathy. The association with Takayasu's arteritis (TA), a chronic inflammatory and stenotic disease of large and medium-sized arteries, is instead less common. We report a case of PG associated with TA that was induced by an accident with folgoration of the skin; in this case the folgoration can be considered as an exemple of Pathergy, that is, a characteristic feature of PG.

A case of infliximab-induced psoriasis.

Anti-tumor necrosis factor (anti-TNF-alpha) are a group of new drugs able to inhibit the action of this cytokine. Although systemic side effects have been well described, cutaneous adverse reactions have not yet been clearly elucidated. The authors report a case of a 29-year-old man affected by Crohn disease and ankylosing spondylitis who developed psoriatic lesions after IV infusion of infliximab 5 mg/Kg. The patient underwent cyclosporine treatment after interruption of biological therapy, and had complete resolution of cutaneous lesions. The reason for this phenomenon is not clear, Obviously more studies are necessary to define more clearly this paradoxical reaction. In addition, dermatologists must be informed about this potential cutaneous adverse event.

Efficacy of Bosentan in treatment of refractory sclerodermic bone prominences skin ulcers.

Scleroderma is an autoimmune disease characterized by skin and internal organs involvement. Cutaneous ulcerations is one of the most important complication. It may cause pain, disability and may lead to infections, scarring and amputation. Sclerodermic skin ulcers management is quite complex and involves non-pharmacologic and pharmacologic modalities both for the treatment and the prevention. In this report, authors describe a case of refractory skin ulcerations in a sclerodermic patient treated with endothelin receptor antagonist Bosentan. Bosentan changed the course of cutaneous lesions leading to their complete healing. This treatment represents an alternative therapeutic approach for sclerodermic skin ulcers and it may be taken into consideration for the ongoing development of a new management of cutaneous wounds.