Foam sclerotherapy for reticular veins of the chest: a retrospective review of efficacy and safety.

BACKGROUND: No study has evaluated the use of foam sclerotherapy in treating clinically conspicuous reticular chest veins. OBJECTIVE: This retrospective study evaluates patient-rated efficacy, safety, and satisfaction after foam sclerotherapy for reticular veins of the chest. MATERIALS AND METHODS: A telephone-based questionnaire was used for patient self-assessment of overall improvement, satisfaction, and adverse events. All patients had been treated with 0.25% to 0.50% sodium tetradecyl sulfate (STS) foam using room air (1:4 ratio). The mean length of follow-up was 3.7 years. RESULTS: Twelve of 23 patients were successfully contacted, with a total of 14 treatment sessions. Overall, patients reported scores of 2.4 ± 0.8 for overall improvement (0 = none, 1 = mild, 2 = moderate, and 3 = complete resolution) and 1.75 ± 0.6 for satisfaction with results (0 = not satisfied at all, 1 = mildly satisfied, and 2 = very satisfied), with minor treatment-related adverse events. CONCLUSION: Foam sclerotherapy with STS is effective for management of reticular veins of the chest with an excellent safety profile and high long-term patient satisfaction.

Pediatric erythromelalgia: a retrospective review of 32 cases evaluated at Mayo Clinic over a 37-year period.

BACKGROUND: Erythromelalgia has not been well characterized in the pediatric population. OBJECTIVE: We sought to review our experience of erythromelalgia in the pediatric age group. METHODS: We conducted a retrospective review of patients 18 years of age and younger with a diagnosis of erythromelalgia who were examined at Mayo Clinic in Rochester, MN, from 1970 to 2007. RESULTS: The records of 32 patients (girls, 22 [69%]) were evaluated. Mean age was 14.1 years (range, 5-18 years) and mean time to diagnosis was 5.2 years. Seven patients (22%) had a first-degree relative with erythromelalgia; 4 were from the same family. Physical activity was limited because of discomfort in 21 patients (66%) and school attendance was affected in 11 patients (34%). Noninvasive vascular studies, which compared temperature, laser Doppler flow, and transcutaneous oximetry in the toes, identified vascular abnormalities in 13 (93%) of 14 patients. Neurophysiologic studies with autonomic reflex screening (including quantitative sudomotor axon reflex test and thermoregulatory sweat testing) showed evidence of a small-fiber neuropathy involving the skin in 10 (59%) of 17 patients studied; there was no evidence of large-fiber neuropathy in 20 patients in whom electromyographic and nerve conduction studies were performed. Topical lidocaine was the most commonly prescribed treatment (44%). Fifteen patients were monitored for an average of 9.1 years (median, 5.0 years; range, 0.4-23.7 years). At last follow-up, 5 patients had stable disease, 4 showed improvement, two had resolution, one reported worsening of symptoms, and 3 had died (one suicide). LIMITATIONS: Conclusions are limited because this was a retrospective chart review. CONCLUSION: Erythromelalgia in pediatric patients is associated with substantial morbidity and even death. The majority of cases are not inherited. Most patients studied have associated small-fiber neuropathy. The disease course is variable. A reliable and safe treatment has not been determined.

Th17 cells and activated dendritic cells are increased in vitiligo lesions.

BACKGROUND: Vitiligo is a common skin disorder, characterized by progressive skin de-pigmentation due to the loss of cutaneous melanocytes. The exact cause of melanocyte loss remains unclear, but a large number of observations have pointed to the important role of cellular immunity in vitiligo pathogenesis. METHODOLOGY/PRINCIPAL FINDINGS: In this study, we characterized T cell and inflammation-related dermal dendritic cell (DC) subsets in pigmented non-lesional, leading edge and depigmented lesional vitiligo skin. By immunohistochemistry staining, we observed enhanced populations of CD11c+ myeloid dermal DCs and CD207+ Langerhans cells in leading edge vitiligo biopsies. DC-LAMP+ and CD1c+ sub-populations of dermal DCs expanded significantly in leading edge and lesional vitiligo skin. We also detected elevated tissue mRNA levels of IL-17A in leading edge skin biopsies of vitiligo patients, as well as IL-17A positive T cells by immunohistochemistry and immunofluorescence. Langerhans cells with activated inflammasomes were also noted in lesional vitiligo skin, along with increased IL-1ß mRNA, which suggest the potential of Langerhans cells to drive Th17 activation in vitiligo. CONCLUSIONS/SIGNIFICANCE: These studies provided direct tissue evidence that implicates active Th17 cells in vitiligo skin lesions. We characterized new cellular immune elements, in the active margins of vitiligo lesions (e.g. populations of epidermal and dermal dendritic cells subsets), which could potentially drive the inflammatory responses.

Albinism in Africa: stigma, slaughter and awareness campaigns.

Oculocutaneous albinism is an autosomal recessive disorder characterized by a lack of pigment in the hair, skin, and eyes. Albinism is caused by defective or absent tyrosinase, an enzyme necessary for melanogenesis. Although rare in the western world, albinism is quite common in sub-Saharan Africa, likely as a result of consanguinity. Albinism has long been associated with stigma and superstitions, such as the belief that a white man impregnated the mother or that the child is the ghost of a European colonist. Recently, a notion has emerged that albino body parts are good-luck charms or possess magical powers. These body parts may be sold for as much as $75,000 on the black market. As a result there have been over 100 albino murders in Tanzania, Burundi, and other parts of Africa in the past decade, which is now beginning to garner international attention and thus prompting novel legislation. To ameliorate the plight of individuals with albinism in Africa, a coordinated effort must be organized, involving medical professionals (dermatologists, ophthalmologists, oncologists), public health advocates and educators, social workers, human rights and antidiscrimination activists, law-enforcement agencies, and governmental support groups. The main issues that should be addressed include skin cancer prevention education, stigma and discrimination denouncement, and swift prosecution of albino hunters and their sponsors.

Drug-associated reversible granulomatous T cell dyscrasia: a distinct subset of the interstitial granulomatous drug reaction.

BACKGROUND: A cutaneous T-cell infiltrate exhibiting cytologic and architectural atypia, an aberrant phenotypic profile and clonal restriction would fall under the rubric of a T-cell dyscrasia. Although such an infiltrate could represent a lymphoma, this constellation of findings can also be seen in drug-associated pseudolymphoma. METHODS: In 2001, two of the authors (CMM and AEC) proposed the term reversible T-cell dyscrasia to describe atypical T-lymphocytic infiltrates that manifest a light microscopic, phenotypic and molecular profile that closely parallels cutaneous T-cell lymphoma but regress when the causal drug is withdrawn. RESULTS: Herein we report our 10 cases of drug-associated pseudolymphoma resembling granulomatous mycosis fungoides. CONCLUSIONS: We term this reaction pattern drug-associated reversible granulomatous T-cell dyscrasia and consider it a distinct subset of the interstitial granulomatous drug reaction.