A purple plaque in a patient with systemic sclerosis.

We present the case of a 43-year old woman with anti-U3 ribonucleoprotein antibody-positive systemic sclerosis presenting with an enlarging purple plaque on the left upper arm. The skin was not sclerotic; however, there had been a cluster of long-standing telangiectases preceding the plaque. Histology and immunohistochemistry confirmed an angiosarcoma. There are five reported cases in the literature about angiosarcoma arising in the skin of patients with systemic sclerosis; however, to our knowledge, this is the first to have arisen from non-sclerotic skin. We would urge clinicians to adopt a high index of suspicion for atypical vascular tumours presenting in patients with systemic sclerosis.

Development and Validation of a Clinical Grading Scale to Assess the Vulvar Region: The Vulvar Architecture Severity Scale.

BACKGROUND: The vulva is composed of aesthetic units that can be affected differently by vulvar conditions. A reliable, comprehensive, and quick-to-use clinical scoring system is required to assess the disease extent in the vulvar area. OBJECTIVES: The aim of this study was to develop and validate a grading scale based on the aesthetic unit principle to evaluate the extent of vulvar lichen sclerosus (VLS). METHODS: After reviewing photographs of 100 patients affected by VLS, the authors targeted the aesthetic units most frequently affected. The disease signs were recorded and graded in 4 levels of severity (none, mild, moderate, severe) taking into account the vulvar architecture and skin involvement. To validate the scale, 14 observers were asked to apply it to photographs of 25 VLS patients on 2 different occasions. Intra- and inter-observer reliabilities were determined employing Pearson's and intraclass correlation coefficients. RESULTS: A 6-region, 4-point grading system was designed and identified as the Vulvar Architecture Severity Scale (VASS). In all 6 areas, the Pearson's r was greater than 0.9 (mean, 0.994; 95% confidence interval [CI] = 0.992), indicating that the intra-observer reliability of the VASS was consistent over time (P < 0.001). Intraclass correlation at time 1 was 0.928 (95% CI = 0.910, 0.943) and at time 2 was 0.944 (95% CI = 0.931, 0.996), indicating a high reliability level among different observers. CONCLUSIONS: The VASS is a reliable scale to assess the severity of VLS, and it might be considered as an outcome measure in future VLS trials.

Fat Grafting Improves Fibrosis and Scarring in Vulvar Lichen Sclerosus: Results From a Prospective Cohort Study.

OBJECTIVE: The aim of the study was to evaluate the effect of lipotransfer in women presenting with fibrosis and scarring due to lichen sclerosus. MATERIALS AND METHODS: This prospective cohort study included 33 women attending the vulvar clinic of a public hospital. Patients received one lipotransfer treatment. Validated measures were used prospectively to assess the sexual function (Female Sexual Function Index, Female Sexual Distress Scale); symptoms (visual analog scale for itching, burning, soreness), pain (Pain Anxiety Symptoms Scale 20); psychological status and quality of life (Hospital Anxiety and Depression Scale, Relationship Assessment Scale, Wound Management Questionnaire Revised); physician-based disease signs (Vulvar Architecture Severity Scale). Data were analyzed using paired t test with nonparametric Wilcoxon matched-pairs signed rank test and unpaired t test with nonparametric Mann-Whitney test (Prism6 Software). RESULTS: The mean (SD) follow-up was 12.9 (3.5) months. Sexual function improved after treatment (p < .001), as well as the distress associated with sexuality (p < .0001). A significant improvement was reported in itching (p < .001), burning (p < .05), soreness (p < .001), and pain (p < .0001). Patients reported a significant improvement in romantic relationship (p < .05), anxiety (p < .0001), and depression (p < .0001). Improvement was not significant in the self-care associated with self-disgust assessment (p = .42). The clinical physician-based score showed an overall improvement in all the treated areas to lesser or greater extent. CONCLUSIONS: The use of fat grafting in lichen sclerosus is promising. Further studies are required to rule out a potential placebo effect and to better understand the underlying molecular mechanism of action.

Primary and secondary intralymphatic histiocytosis.

BACKGROUND: Intralymphatic histiocytosis (IH) is a rare condition often associated with systemic disease. A benign condition, clinical presentations can vary greatly and its cause is largely unknown. Histologically, there are macrophages within distended lymphatic vessels, although this can be an incidental finding or the primary abnormality. OBJECTIVE: We present a series of 7 cases of IH with and without disease associations, and a review of the literature. We propose IH as either primary (without associated conditions) or secondary (associated with systemic disease). METHODS: This was a retrospective collection of patients whose skin biopsy specimens revealed intralymphatic collections of histiocytes. We reviewed their clinical presentation, disease associations, and staining of slides with CD68 in all cases, D2-40 in 5 cases, and HLA-DR in 4 cases. RESULTS: Clinical features were highly variable, and not all cases were associated with systemic disease. One case had admixed reactive angioendotheliomatosis. All 4 cases stained for HLA-DR showed strong expression by the intralymphatic macrophages. LIMITATIONS: Retrospective analysis and limited numbers are limitations. CONCLUSION: IH is not always associated with systemic disease although macrophage activation nevertheless implies immune activation.

High frequency and diversity of cutaneous appendageal tumors in organ transplant recipients.

BACKGROUND: Recipients of organ transplant who are immunosuppressed are at greatly increased risk of nonmelanoma skin cancers compared with the general population, but their risk of appendageal tumors is unknown. OBJECTIVE: Our aim was to conduct a systematic examination of cutaneous appendageal tumors arising in recipients of organ transplants compared with individuals who were immunocompetent (ICP). METHODS: We conducted a retrospective, clinicopathologic analysis of consecutive appendageal tumors arising in 650 recipients of organ transplants and in the general population of approximately 605,000 people served by our institution. RESULTS: Between 1993 and 1998, 231 appendageal tumors were identified in 211 individuals; 23 tumors were found in 21 of 650 patients undergoing transplant (3%), 10 in individuals with other immunosuppressive conditions, 3 in 2 patients with Muir-Torre syndrome, and 195 in 178 apparently ICP. In addition to the increased frequency of appendageal tumors among recipients of transplants, malignant tumors were overrepresented (43% of transplant tumors vs 4% in ICP; P <.0001) as were tumors of sebaceous origin (30% vs 6%; P <.0001). CONCLUSIONS: Recipients of organ transplant who are immunosuppressed have a greatly increased risk of cutaneous appendageal tumors compared with apparently ICP. In addition, their tumors are more likely to be malignant and of sebaceous origin.