Livedoid vasculopathy and skin ulcers in patients with inherited thrombophilia.
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Adult↗
Biomedical subjects
Giampiero Girolomoni
Publications and source records attributed to Giampiero Girolomoni.
Disseminated lupus vulgaris.
Lupus vulgaris is the most common form of cutaneous tuberculosis, and usually presents as a solitary lesion on the face. We report two patients with multiple lesions on different skin areas. The first patient presented a diffuse involvement of the right foot, and reddish-brown plaques on the right leg, the back and the face. Spreading of the lesions followed a prolonged application of topical corticosteroids. The second patient showed a large plaque on the nape and occipital area resulting in scarring alopecia, and plaques on the right inguinal and thigh regions. Ziehl-Neelsen staining was negative in both cases, but diagnosis was supported by histology and polymerase chain reaction analysis. No visceral involvement was present. Antituberculosis polychemotherapy was rapidly effective.
Adrenal Cortex Hormones↗
Chemokine networks in inflammatory skin diseases.
The superfamily of chemokines comprises numerous small, cytokine-like chemotactic proteins, which have a fundamental role in the regulation of leukocyte trafficking. The chemokine-chemokine receptor system is highly redundant and promiscuous, and forms a complex network relevantly involved in the expression of chronic inflammatory skin diseases, including allergic contact dermatitis, atopic dermatitis and psoriasis. The pattern of chemokine expression shows overlapping features but also important differences in these diseases due to distinct sources and types of pro-inflammatory signals involved in chemokine induction, and the inherent capacity of resident skin cells to produce chemokines. Chemokine receptors (G-protein coupled receptors) rather than chemokines appear the appropriate therapeutic targets as they are more chemically tractable and play less redundant functions.
Dermatitis, Allergic Contact↗
Identification of the keratin K9 R162W mutation in patients of Italian origin with epidermolytic palmoplantar keratoderma.
Epidermolytic palmoplantar keratoderma (EPPK) is an autosomal dominant skin disorder characterized by hyperkeratosis of the palms and soles associated with histologic findings of hyperkeratosis and epidermolysis. Ultrastructurally, there is vacuolization of the cytoplasm and abnormal keratin filament network with tonofilament clumping. EPPK is caused by mutations in the keratin 9 gene (KRT9), which is expressed exclusively in suprabasal keratinocytes of palmoplantar epidermis. The mutation R162W is the most frequent keratin 9 alteration reported in patients from different geographical areas. We present three unrelated Italian families affected by EPPK in which we confirmed the presence of the R162W mutation, by RT-PCR analysis followed by sequencing of the KRT9 gene, in all affected members. The finding of the same mutation in all patients, together with the previous reports of the disease, strongly suggest that position 162 of the KRT9 gene represents a mutation "hot-spot", probably due to the peculiarity of the sequence.
Adult↗
Prevalence of psoriatic arthritis and joint complaints in a large population of Italian patients hospitalised for psoriasis.
We investigated the prevalence of psoriatic arthritis and joint complaints in a large population of Italian patients hospitalised for psoriasis. A total of 936 patients were evaluated for psoriatic arthritis according to the European Spondyloarthropathy Study Group (ESSG) criteria. They were visited by a dermatologist who evaluated specific joint signs and symptoms (i.e. arthralgia, stiffness, swelling, ankylosis, paresthesia) and assessed skin disease severity. Information on socio-demographic variables and other factors of clinical interest was also collected. Seventy-one patients (7.7% of the sample) had psoriatic arthritis (PsA). Overall, 90% of patients with PsA referred arthralgia, 70.4% stiffness, 67.7% swelling, 25.4% paresthesia, and 23.9% had ankylosis. However, among patients with plaque psoriasis who did not fulfil the criteria for PsA, 12.3% referred paresthesias, 7% arthralgia, 4.2% stiffness, 3.7% swelling, and 1.2% had ankylosis. Among 114 patients referring arthralgia, 42 (39.6%) did not meet the ESSG criteria for PsA. Therefore, patients with plaque psoriasis refer joint symptoms quite frequently making it difficult to distinguish patients with psoriatic arthritis from patients with joint complaints.
Adolescent↗
The contribution of keratinocytes to the pathogenesis of atopic dermatitis.
Atopic dermatitis (AD) develops from a complex interplay between environmental, genetic, immunologic and biochemical factors. Relevant to the amplification and persistence of inflammatory and immune responses in AD skin are keratinocytes, which can be induced to secrete proinflammatory mediators in response to a variety of stimuli, including epidermal barrier perturbation. Moreover, keratinocytes from AD patients synthesize exaggerated amounts of mediators (e.g., GM-CSF and RANTES/CCL5) important for enhanced recruitment as well as sustained survival and activation of T cells and dendritic cells. AD keratinocytes have a constitutive dysregulated activity of transcription factors that modulate the expression of inflammatory genes, suggesting the existence of predetermined mechanisms targeting atopic inflammation to the skin. Among these, the existence of a defective epidermal barrier, which appears related to decreased ceramide generation and abnormal degradation of corneodesmosomes, certainly plays a central role in the predisposition to AD.
Cytokines↗
Incidence and clinical predictors of Kaposi's sarcoma among 1721 Italian solid organ transplant recipients: a multicenter study.
Kaposi's sarcoma (KS) can be a complication of solid organ transplantation, but with an important incidence rate variability in different geographical areas. Here we analyzed the incidence rate, timing and clinical correlates of KS, in a cohort of Italian solid organ transplant recipients from four distinct transplantation centers. A total of 1721 renal, heart and liver transplant recipients were recruited between 1997 and 2004. KS was diagnosed in 40 patients, after a median follow up of 1 year (range 0.8-5.1). Visceral involvement was detected in 7/40 patients. Incidence rate of KS in the whole population was 2.3 cases per 1000 individuals per year. The standardized incidence rate (SIR) for KS in renal transplant recipients was 149.9 (95% CI 103.0-212.0), with the excess risk greater among women (SIR 316.0) than among men (SIR 133.6). In a Cox proportional hazard regression model, age at transplantation equal or older than 30 years and only combined immunosuppressive therapy with mycophenolate mofetil + cyclosporine + prednisolone were independently associated with KS. Italian organ transplant recipients have an increased risk (about 100 times greater) for KS compared to the general population, especially during the first two years after transplantation. Age older than 30 years at transplantation and a more aggressive immunosuppressive regimen were both independent risk factors for the disease.
Adult↗