Extracorporeal photopheresis: a useful therapy for patients with steroid-refractory acute graft-versus-host disease but not for the prevention of the chronic form.

BACKGROUND: Extracorporeal photopheresis (ECP) has been used successfully to treat severe steroid-refractory acute and chronic graft-versus-host disease (aGVHD, cGVHD) since the late 1990s. OBJECTIVES: To evaluate retrospectively the efficacy and safety of ECP in patients with aGVHD. We also assessed whether ECP may play a role in the prevention of cGVHD. PATIENTS AND METHODS: Nine consecutive patients with allografts with aGVHD grade II-III, as defined by consensus criteria, and refractory to steroids, were treated with ECP. ECP was started at a median interval of 46·3 days (range 10-70) from aGVHD onset. Patients were treated initially on two consecutive days (one cycle) at 1-week intervals until improvement and then every 2 weeks. Treatment was then tapered off individually. To evaluate statistical relationships with outcome after 30, 60 and 90 days of ECP, all clinical and historical variables of the patients before treatment were analysed. RESULTS: All patients survived and responded within 90 days. The average aGVHD score was 1·72 at aGVHD onset, 2·44 when ECP was started and then gradually declined to 0·44 on day 90. At the same time, the average dose of methylprednisolone declined from 2·22 mg kg(-1) to 0·27 mg kg(-1) (day 90), while the average dose of ciclosporin declined from 2·46 mg kg(-1) to 0·77 mg kg(-1) (day 90). Six of nine patients showed a complete skin response after 90 days of treatment. All patients with liver and gastrointestinal tract involvement had complete responses after 90 days, apart from one patient. All our patients developed cGVHD, seven of nine while still on maintenance regimen (6-13 months after haematopoietic stem cell transplantation, HSCT) and the other two patients after suspension of ECP (6 and 9 months after HSCT). CONCLUSIONS: ECP is effective in patients with mild to moderate steroid-refractory aGVHD (grade II-III). On the other hand, ECP did not prevent the development of cGVHD in our patients.

Skin diseases in geriatric patients: our experience from a public skin outpatient clinic in Siena.

AIM: With the progressive aging of the Italian population, geriatric health care has become a major issue for health authorities. However, little data is available regarding geriatric skin diseases. In order to provide rapid access to specialist help, in 2003 we created a dermatology clinic dedicated only to geriatric patients age 65 and older. METHODS: To determine the characteristic pattern and the prevalence of various skin disorders among the geriatric patients seen at the clinic, we performed a retrospective and descriptive study of all skin diseases in patients seen in our office from January 2003 to December 2009. We evaluated: age, proportion and gender for all skin disease categories. RESULTS: A total of 2100 geriatric patients were examined. The male to female ratio was 1.4 to 1. The most common disorder was pruritus "sine materia" (18.9%) followed by benign tumors (13.5%); 9.1% of our patients presented with actinic keratoses and 13.2% with malignant tumors. As reported by others, the quality of life in patients with skin cancer was better than patients with rashes as skin cancer patients tended to wait longer before seeking specialist care. To improve the assessment of skin diseases, we often worked closely with CONCLUSION: The prevalence of skin diseases in our patients emphasized the importance of educating the elderly about sun protection, the early detection of skin cancer, the use of emollients and proper skin care in general.

Papular-purpuric "gloves and socks" syndrome.

Papular-purpuric "gloves and socks" syndrome (PPGSS), is an acute dermatosis characterized by a papular-purpuric edematous rash in a distinct ''gloves and socks'' distribution often accompanied by fever, asthenia and lymphadenophaties. It is mainly caused by parvovirus B19 (B19V) but other viruses and drugs such as trimethoprim/sulfametaxol or chemotherapics may be involved. We describe a case of PPGSS with a serologically proven B19V infection in a 42-year-old Italian kindergarten teacher suffering from acute bacterial pharyngitis Immunoglobulin M by enzyme-linked immunosorbent assay (ELISA) to parvovirus B19 were positive. Histological examination showed dermal-ipodermal inflammation with evidence of leukocytoclastic vasculitis principally interesting the small venules The cutaneous rash resolved after 2 weeks. We reported our case to support that PPGSS is an immunomediated disease and that B19V morbidity varies with the immunologic and hematologic status of the host. In addition, a patient with PPGSS might be infectious at the moment of diagnosis, with significant implication for susceptible contacts.

Geriatric teledermatology: store-and-forward vs. face-to-face examination.

BACKGROUND: Telemedicine could be useful in countries like Italy to meet the needs of elderly patients and in particular in those in precarious general conditions, for whom travelling even short distances can pose considerable practical and economical difficulties. OBJECTIVE: The aim of this study was to determine the efficacy of store-and-forward teledermatology vs face-to-face consultations in elderly patients. METHODS: A total of 130 geriatric patients with skin diseases requiring dermatological examination were enrolled. The patients examined, consisting of 60 men (46.15%) and 70 women (53.85%), were aged between 66 and 97 years (mean age 80.58 years). Three dermatologists of the department, with equal experience took turns in face-to-face examination and teledermatology (store-and-forward). To compare face-to-face dermatological examinations with the asynchronous store-and-forward approach of teledermatology, we considered diagnostic agreement (ICD-9 code), therapeutic agreement and concordance of diagnostic confidence. RESULTS: One hundred and fourteen of 130 patients were diagnosed with the same ICD-9 code, making a total observed agreement of 87.7% with a Cohen's κ estimated of 0.863. Agreement between therapies was 69.6% (Cohen's κ = 0.640). As it concerns diagnostic confidence, dermatologists appeared generally slightly less certain of their diagnosis by telemedicine. CONCLUSIONS: Store-and-forward teledermatology can improve diagnostic and therapeutic care for skin disease in elderly who lack easy and/or direct access to dermatologists.

A case of sebaceous nevus sindrome (Schimmelpen-ning-Feuerstein-Mims syndrome).

The sebaceous nevus syndrome (SNS) is characterized by the presence of a sebaceous nevus and extracutaneous abnormalities, usually involving organs derived from the neuroectoderm. The authors report the case of a 22 year-old patient with a systematized sebaceous nevus on the forehead and scalp and a history of developmental delay and severe seizure disorders. The father of the patient also indicated prior surgery to correct an ocular coloboma and prior removal of a squamous cell epithelioma of the tongue. Firstly described by Gustav Schimmelpenning in 1957, SNS, also known as 'Schimmelpenning-Feuerstein-Mims syndrome', is one of the six different types of epidermal nevus syndromes. In patients with SNS the risk of developing neoplasms is higher than that of the general population. The particularity of our case lies in the late diagnosis. The authors believe that early diagnosis and awareness of the potential presence of neoplasms are important for those involved in the care of patients with SNS.

Ocular manifestations of chronic graft-versus-host disease in patients treated with extracorporeal photochemotherapy.

PURPOSE: Eye involvement has long been appreciated in patients with chronic graft versus host disease (cGVHD). In particular, ocular complications are frequent and can be potentially severe in patients with steroid-refractory cGVHD, and therefore necessitate close monitoring. This prospective study was designed to describe eye manifestations of cGVHD in a large series of patients monitoring them before and after 1 year of extracorporeal photochemotherapy (ECP). ECP is a relatively new therapeutic approach based on the biological effects of psoralen 8-methoxypsoralen (8-MOP) and ultraviolet A light (UVA) on mononuclear cells collected by apheresis, and reinfused into the patient. METHODS: Only patients with steroid-refractory cGVHD under treatment with ECP, who developed cGVHD-related eye symptoms, were selected for the study. Ophthalmologic examination was repeated every 3 months. Only patients with complete recovery of the ocular manifestations and symptoms were considered responsive. RESULTS: In our study we observed eye alterations in 24 out of 140 patients (17%) with cGVHD. After 12 months of ECP, 10 out of 21 patients (48%) completely responded to the therapy. In all these cases the contribution of ECP was also essential in all the other organs subject to cGVHD. CONCLUSIONS: Further studies are necessary to clarify the role of ECP in patients with cGVHD, especially in associated eye manifestations. Although our experience is limited, it suggests that ECP could be a safe and effective therapy for steroid-refractory eye manifestations of cGVHD.

Basal cell carcinoma of the vulva.

Basal cell carcinoma (BCC), the most common human malignancy, accounting for 75% of all non-melanoma skin cancer, is uncommon on unexposed skin such as the perianal and genital regions. We describe a woman with BCC of the vulva treated with local resection. All margins of excision were free of disease. The patient recovered without complication and there have been no recurrences after 2 years of follow-up. Approximately 200 cases of BCC on perianal and genital skin have been reported in the literature. Although the aetiology of vulvar BCC is not known, early diagnosis is important. Because BCC in these sites sometimes seems innocuous, biopsy of all suspect lesions is advisable.

A case of Darier's disease successfully treated with topical tacrolimus.

Tacrolimus is a macrolide that inhibits T-cell activation. The most extensive experience with topical tacrolimus has been in treating atopic dermatitis but it has been used in various skin diseases, including Hailey-Hailey disease, with encouraging results. We report a case of extensive Darier's disease successfully treated with topical tacrolimus, after suspension of oral isotretrinoin due to major depression.

Malignant hypercalcemia in vulvar cancer.

Humoral hypercalcemia of malignancy (HHM) is a common paraneoplastic syndrome, most often associated with squamous cell carcinoma of the lung, esophagus, kidneys and breast, but rarely with vulval cancer. In most patients, HHM is associated with over-production of a peptide analogue of parathormone (parathyroid hormone-related protein, PTHrP). The case of a 70-year-old patient with massive squamous cell epithelioma of the vulva complicated by HHM is reported. To our knowledge, this is the first documented case with high serum concentrations of PTHrP and immunohistochemical confirmation of PTHrP production by neoplastic cells.

Discoid lupus erythematosus of the vulva.

Genital involvement is a rare manifestation in discoid lupus erythematosus (DLE) and systemic lupus erythematosus (SLE), and there are few reports on vulvar clinical features. We describe a 41-year old Caucasian woman with bilateral vulvar DLE. Although vulvar lesions in lupus are probably more common than is realized, the present report is the first case of genital DLE documented in a female.