Papillary dermal elastosis: a unique elastic tissue disorder or an unusual manifestation of pseudoxanthoma elasticum-like papillary dermal elastolysis?

There are numerous acquired elastic tissue disorders, several of which present cutaneously with small yellow-to-white papules resembling plucked chicken skin. Differential diagnoses depend on the abnormalities within the network of elastic tissues. We report a case with distinct histologic features, which may represent a unique elastic tissue disorder or a variant of pseudoxanthoma elasticum-like papillary dermal elastolysis. Our patient's clinical presentation includes scattered 1-2 mm white-to-yellow papules without surface change on the upper back and neck region. Histology is characterized by foci of clumped, granular elastic tissue, which have replaced the oxytalan and elaunin fibers, alternating with foci of decreased concentrations of normal-appearing elastic fibers within the papillary dermis. Given its characteristics, we have termed this novel entity 'papillary dermal elastosis'.

Trends in pediatric melanoma mortality in the United States, 1968 through 2004.

BACKGROUND AND OBJECTIVE: Mortality from melanoma in children is a poorly understood and controversial problem in dermatology. There is paucity of research into this important public health dilemma. The purpose of this study was to characterize pediatric melanoma mortality in the United States and to evaluate trends over time. METHODS AND MATERIALS: Deaths were derived from a database of more than 75 million records of the U.S. Center for National Health Statistics based on routine death certification. Information on age, race, gender, and geographic location was available for years 1968 through 2004. RESULTS: During the 37-year period, there were 643 deaths attributed to melanoma in children under 20 years of age in the United States, an average of 18 per year. The overall age-adjusted mortality rate for melanoma in children was 2.25 deaths per year (per 10 million at-risk individuals). Mortality rates were strongly associated with age. In the oldest age group (age 15-19 years) the mortality rate was approximately an order of magnitude 8-18 times higher compared to younger age groups. Mortality among males was 25% higher than females. Mortality rates for white children were more than twice as high as black children. Overall mortality from melanoma in children declined steadily from 1968 to 2004. The highest mortality rates were observed in Idaho, Nevada, Arizona, and New Mexico. CONCLUSIONS: Although mortality from melanoma among children in the United State is low, the magnitude of the public health burden from this preventable cause of death is substantial. In contrast to results of studies suggesting that the incidence of melanoma may be rising in children and adolescents, the data suggest that mortality in these groups may be falling. Additional study is warranted to further characterize and ultimately reduce mortality from childhood melanoma.

A meta-analysis of complications attributed to anticoagulation among patients following cutaneous surgery.

BACKGROUND: The frequency of postoperative bleeding and other complications in anticoagulated patients undergoing cutaneous surgery has not been firmly established and consensus on perioperative continuation of treatment is lacking. OBJECTIVE: The objective was to ascertain the risk of postoperative complications through meta-analysis of data pooled from previously published studies. METHODS: A PubMed search (1966-2005) was performed to identify controlled studies reporting bleeding and other complications among patients undergoing cutaneous surgery who were taking anticoagulant medications. Emphasis was placed on prescription anticoagulant medications (aspirin, NSAIDs, warfarin, clopidogrel) and over-the-counter herbal agents with anticoagulant properties. RESULTS: A total of six studies representing 1,373 patients met criteria for inclusion. Among patients taking aspirin or warfarin, 1.3 and 5.7% experienced a severe postoperative complication, respectively. Patients taking warfarin were nearly seven times as likely to have a moderate-to-severe complication compared to controls (OR, 6.69; 95% CI, 3.03-14.7), a statistically significant difference (p<.001). Patients taking aspirin or NSAIDs were more than twice as likely to have a moderate-to-severe complication compared to controls (OR, 2.0; 95% CI, 0.97-4.13), a strong trend toward statistical significance (p=.06). There were no studies in the literature that examined the effects of combination anticoagulant therapy or the effect of herbal agents on postoperative risk of bleeding. CONCLUSION: The results of this meta-analysis suggest that while low, the risk of bleeding among anticoagulated patients may be higher than baseline. Adequately powered prospective studies are required to more carefully delineate the risk of postoperative bleeding and other complications attributable to anticoagulation therapy. Particular emphasis should be placed on examining the effect of combination anticoagulant therapy as well as herbal agents with anticoagulant properties on risk of bleeding after cutaneous surgery.

Adjuvant local irradiation for Merkel cell carcinoma.

OBJECTIVES: To determine the effect of adjuvant local irradiation on (1) disease recurrence and (2) survival rates in Merkel cell carcinoma (MCC). DATA SOURCES: An Ovid MEDLINE search (January 1966-May 26, 2004) was performed using the following criteria: group 1, "Merkel cell OR trabecular OR neuroendocrine skin OR APUDoma skin OR primary small cell skin OR primary undifferentiated skin OR endocrine skin OR neuroepithelial" AND group 2, "carcinoma OR tumor OR cancer" with mapping modifiers "-title, -abstract, -keyword, -subject heading." The search yielded 843 citations. STUDY SELECTION: The Ovid set was then searched using the following criteria: "surgery OR radiation OR radiotherapy," which yielded 242 discrete citations. Reports from all 242 citations were reviewed. For the remaining 601 citations, abstracts (when available) were reviewed to assess the level of relevance for potential inclusion; reports from 63 of these citations were reviewed. An additional 28 secondary references were reviewed, for a total of 333 reports. DATA EXTRACTION: The following criteria for inclusion were applied to each potential patient: (1) a histopathologic diagnosis of MCC; (2) a single, primary tumor arising on the skin, for which (3) the primary treatment was surgical excision (local excision, wide excision, or Mohs surgery) with or without the use of adjuvant irradiation (to the tumor bed); (4) following surgery, negative (clear) surgical margins were obtained; (5) during the postoperative follow-up period, disease recurrence, progression, and survival and/or duration of event-free interval was documented with (6) a minimum follow-up of 1 month. A total of 1254 patients were included in the analysis. RESULTS: Statistically significant reductions in local (hazard ratio [HR], 0.27; P < .001) and regional (HR, 0.34; P < .001) recurrence were observed among patients treated with combination therapy compared with surgery alone. Similar rates of distant metastasis were observed between treatment groups (HR, 0.79; P = .31). Overall survival rates were 87% (1 year) and 49% (5 years). Cause-specific survival rates were 90% (1 year) and 62% (5 year). In general, differences in overall (HR, 0.78; P = .16) and cause-specific (due to MCC: HR, 0.72; P = .14) survival rates between treatment groups did not reach statistical significance. A subgroup analysis that excluded single-patient case reports and studies of only 1 treatment group revealed a significant overall (HR, 0.63; P = .02) and cause-specific (HR, 0.62; P = .04) survival advantage after treatment with combination therapy. CONCLUSIONS: Surgery plus local adjuvant irradiation was associated with significantly lower rates of local and regional recurrence of MCC than surgery alone. Prospective investigation is needed to clarify the presence of a survival benefit from combination therapy.

Skin cancer after transplantation: a guide for the general surgeon.

The success of organ transplantation has been accompanied by serious concerns regarding the incidence and management of potentially catastrophic cutaneous carcinogenesis in transplant recipients. Delivery of the highest quality of care requires a concerted effort toward collaboration between multiple surgical and medical specialties. The purpose of this review is to provide the general surgeon with a practical, user-friendly guide to the important components of comprehensive dermatologic care for organ transplant recipients (OTRs) with references to more detailed sources of information.

Ocular Involvement Following Postnatally Acquired Toxoplasma gondii Infection in Southern Brazil: A 28-Year Experience.

PURPOSE: To determine the incidence of, and risk factors for, ocular involvement among people known to have postnatally acquired Toxoplasma gondii infection in a region of southern Brazil where there is a high prevalence of endemic disease. DESIGN: Retrospective longitudinal cohort study. METHODS: Records of 302 patients with serologic evidence of recent T gondii infection (a positive anti-T gondii IgM antibody test) from Erechim, Rio Grande do Sul state, Brazil (1974-2002) were analyzed. The incidence of ocular involvement was calculated in terms of person-years (PY) of follow-up. Risk factors for ocular involvement were analyzed using log-rank and Fisher exact tests. RESULTS: At initial ocular examination (baseline), 30 patients (9.9%) had intraocular inflammation only (anterior chamber cells and flare, vitreous inflammatory reactions, retinal whitening), without clinically apparent necrotizing retinochoroiditis. At baseline, men were more likely to have ocular involvement (P = .043) and antiparasitic treatment was associated with less ocular involvement (P = .015). Follow-up examinations were performed on 255 patients (median follow-up, 13.7 months [range 0.4-261.9 months]). Among those without ocular involvement at baseline, the incidence of necrotizing retinochoroiditis was 6.4/100 PY. Patients >40 years of age at first IgM test had a greater risk of incident necrotizing retinochoroiditis (hazard ratio = 4.47, 95% CI = 1.67-11.93, P = .003) than younger patients. The incidence of recurrent necrotizing retinochoroiditis was 10.5/100 PY. CONCLUSION: Isolated intraocular inflammatory reactions can be an initial manifestation of T gondii infection, with necrotizing retinochoroiditis occurring months or years later. Male sex and older age are risk factors for toxoplasmic retinochoroiditis. Antitoxoplasmic treatment may protect against early ocular involvement.

Ocular toxoplasmosis: a 50th anniversary tribute to the contributions of Helenor Campbell Wilder Foerster.

In 1952, Helenor Campbell Wilder (later Helenor Campbell Wilder Foerster) confirmed the growing suspicion that Toxoplasma gondii was a cause of uveitis in otherwise healthy adults by identifying the presence of parasites in eyes enucleated because of severe intraocular inflammation. Ocular toxoplasmosis was previously known to occur only in newborns with congenital T gondii infection. Her report ushered in a new era in the field of uveitis in which toxoplasmosis, rather than tuberculosis, was confirmed to be the most common cause of retinochoroiditis. Fifty years later, issues raised in her landmark publication are still being investigated.

Nonmelanoma skin cancer mortality (1988-2000): the Rhode Island follow-back study.

OBJECTIVES: To estimate (1) the magnitude of and the components and factors associated with nonmelanoma skin cancer (NMSC) mortality and (2) the proportion of deaths misclassified as NMSC. DESIGN: Population-based follow-back study. SETTING AND PATIENTS: All Rhode Island residents whose deaths between 1988 and 2000 were attributed to NMSC. MAIN OUTCOME MEASURES: Distribution of diagnoses, verification of the causes of death, and characterization of associated factors. RESULTS: The proportion of misclassified deaths was significantly higher for nongenital NMSC (57%) than for genital NMSC (18%; P<.001). Most of the deaths misclassified as nongenital NMSC were caused by squamous cell carcinoma of mucosal surfaces. The age-adjusted NMSC mortality rate was 0.91 (per 100 000 persons per year), of which almost half (0.45) were due to genital carcinoma. Nonmelanoma skin cancer mortality increased sharply with age. The mortality rate from nongenital NMSC in men was more than twice that in women, but for genital NMSC this ratio was reversed. Skin cancers originating on the ear were responsible for more than a quarter of all deaths caused by nongenital NMSC. No cases of NMSC mortality occurred in organ transplant recipients. Many individuals had comorbid psychiatric conditions or evidence of unreasonable delay in seeking medical care for their lesions. CONCLUSIONS: Misclassifying the cause of death as nongenital NMSC accounts for a large source of error on death certificates in Rhode Island. Overall, nongenital squamous cell carcinoma and basal cell carcinoma death rates have declined, and mortality due to genital carcinoma was about half of total NMSC deaths. The dermatology community should emphasize prevention of mortality from genital skin cancer, while continuing to stress the importance of reducing excessive exposure to UV light and prompt treatment of NMSC.

An update on current practices in the management of ocular toxoplasmosis.

PURPOSE: To update information that was published by the AMERICAN JOURNAL OF OPHTHALMOLOGY in 1991 about treatment practices for ocular toxoplasmosis by uveitis specialists. DESIGN: Physician survey. METHODS: A written questionnaire was distributed to all physician-members (n = 147) of the American Uveitis Society. The questionnaire was modeled after a similar device used to survey uveitis specialists in 1991. Information contained on 96 returned questionnaires was tabulated. RESULTS: Among 79 respondents who evaluate and manage patients with ocular toxoplasmosis, 15% treat all cases regardless of clinical findings (in contrast to 6% in 1991). The major indications for treatment among other respondents were severe inflammatory responses and proximity of retinal lesions to the fovea and optic disk. The majority of clinical factors considered in five categories (vision, lesion location, lesion size, lesion characteristics, and vitreous inflammatory reaction) were identified to be relative or absolute indications for treatment by a greater proportion of respondents in the current survey than in the 1991 survey. A total of nine drugs (or commercially available combinations) were used in 24 different regimens as treatments of choice for typical cases of recurrent toxoplasmic retinochoroiditis, with the combination of pyrimethamine, sulfadiazine, and prednisone being the most commonly used regimen (29% of respondents). CONCLUSIONS: Uveitis specialists appear to be more likely to treat patients with ocular toxoplasmosis in 2001 than in 1991. Although the majority of survey respondents adhere to a traditional approach to the management of toxoplasmic retinochoroiditis (a discrete course of systemic drug treatment during active disease using multiple antiparasitic drugs with or without corticosteroids), there is still no consensus regarding the choice of antiparasitic agents for treatment regimens. Survey results provide useful information for treating physicians and for clinical investigators interested in therapy.

Acquired disorders of elastic tissue: Part II. decreased elastic tissue.

Elastic fibers in the extracellular matrix are integral components of dermal connective tissue. The resilience and elasticity required for normal structure and function of the skin are attributable to the network of elastic tissue. Advances in our understanding of elastic tissue physiology provide a foundation for studying the pathogenesis of elastic tissue disorders. Many acquired disorders are nevertheless poorly understood owing to the paucity of reported cases. Several acquired disorders in which loss of dermal elastic tissue produces prominent clinical and histopathologic features have recently been described, including middermal elastolysis, papular elastorrhexis, and pseudoxanthoma-like papillary dermal elastolysis, which must be differentiated from more well-known disorders such as anetoderma, acquired cutis laxa, and acrokeratoelastoidosis. Learning objective At the conclusion of this learning activity, participants should have an understanding of the similarities and differences between acquired disorders of elastic tissue that are characterized by a loss of elastic tissue.

Acquired disorders of elastic tissue: part I. Increased elastic tissue and solar elastotic syndromes.

Elastic fibers in the extracellular matrix are an integral component of dermal connective tissue. The resilience and elasticity required for normal structure and function of the skin may be attributed to the network of elastic tissue. Advances in our understanding of elastic tissue physiology provide a foundation for studying the pathogenesis of elastic tissue disorders. Many acquired disorders are nevertheless poorly understood due to the paucity of reported cases. Several acquired disorders in which accumulation or elastotic degeneration of dermal elastic fibers produces prominent clinical and histopathologic features have recently been described. They include elastoderma, linear focal elastosis, and late-onset focal dermal elastosis and must be differentiated from better-known disorders, among them acquired pseudoxanthoma elasticum, elastosis perforans serpiginosa, and Favré-Racouchot syndrome. Learning objective At the conclusion of this learning activity, participants should understand the similarities and differences between acquired disorders of elastic tissue that are characterized by an increase in elastic tissue, as well as the spectrum of solar elastotic dermatoses.

Mortality of bullous skin disorders from 1979 through 2002 in the United States.

OBJECTIVES: To identify and analyze trends in bullous disease mortality from 1979 through 2002 in the United States. DESIGN: Retrospective population-based analysis. SETTING: Mortality records from the Centers for Disease Control and Prevention mortality database. PARTICIPANTS: Mortality records from 1979 through 2002 for persons who died of bullous disease. MAIN OUTCOME MEASURES: Age-adjusted mortality rates and trends for 4 bullous disease subgroups: toxic epidermal necrolysis, pemphigoid, pemphigus, and epidermolysis bullosa. RESULTS: The overall age-adjusted (to the 2000 US standard population) annual mortality rate from bullous diseases of the skin was 0.103 death per 100 000. The average mortality from bullous disorders was 0.098 per 100 000 in 1979 through 1982 and remained stable at 0.099 per 100 000 during the final 4 years of the study, 1999 through 2002. Pemphigoid had a significant increase in mortality from 1979 through 2002, while pemphigus demonstrated a significant decrease in mortality. The mortality rate for toxic epidermal necrolysis was much higher among blacks (0.192 death per 100 000) than whites (0.025 per 100 000) (P < .001), with a mortality rate ratio of 7.57 (95% confidence interval, 6.97-8.21). CONCLUSIONS: Overall mortality from bullous diseases remained stable from 1979 through 2002, although an increasing mortality from pemphigoid and a decreasing mortality from pemphigus occurred during this period. A very large racial disparity in mortality from toxic epidermal necrolysis was observed.

Trends in nonmelanoma skin cancer mortality rates in the United States, 1969 through 2000.

The purpose of this population-based study was to assess trends in mortality rates for nonmelanoma skin cancer (NMSC) in the United States. Particular emphasis was placed on the subgroup of malignancies arising on genital skin. Nearly 75,000 deaths in the United States were attributed to NMSC from 1969 to 2000. The age-adjusted US mortality rate for NMSC arising on nongenital skin from 1969 to 2000 was 0.69/10(5)/year; the rate among men was twice that among women. Mortality rates among white men exceeded that of black men by a factor of two; the same was observed among women, but by a smaller multiple. Corresponding mortality rates for malignancies arising from genital skin (penis, scrotum, vulva) were higher in women (0.54) than in men (0.30). In contrast to nongenital NMSC, mortality rates among black men were twice that of white men; however, rates for white and black women were similar. These results suggest that greater emphasis could be placed on reducing mortality from genital NMSC while continuing to stress reduction of excess sun exposure.

Nephrogenic fibrosing dermopathy and calciphylaxis with pseudoxanthoma elasticum-like changes.

Nephrogenic fibrosing dermopathy (NFD) and calciphylaxis are rare conditions that are associated with chronic kidney disease. Histopathologic changes, including dystrophic dermal calcification, often in association with elastic fibers have been observed in NFD and calciphylaxis. A pattern of dermal elastic fiber calcification that mimics pseudoxanthoma elasticum (PXE) has been previously reported as an incidental finding in the setting of calciphylaxis. Despite a shared association with renal disease and abnormal calcium deposits, however, NFD and calciphylaxis are discrete pathologic processes with distinct clinical and histopathologic features. Criteria for each are reviewed through case presentation of a patient meeting the clinical and histopathologic criteria for both NFD and calciphylaxis with histologic features mimicking PXE.

Lack of difference in the rates of hypopigmentation with 90-microsecond pulsed and longer dwell time carbon-dioxide laser resurfacing.

BACKGROUND: Hypopigmentation is an adverse outcome associated with carbon-dioxide (CO(2)) laser resurfacing. A 90-microsecond pulse produces a more favorable postoperative course of healing, erythema, and pain compared with a 900-microsecond dwell time. The rate of hypopigmentation after 90-microsecond pulsed CO(2) resurfacing may also be reduced. To date, there have been no comprehensive reports on the effect of varying pulse duration on the occurrence of hypopigmentation. OBJECTIVE: We sought to investigate the relationship between pulse duration and the occurrence of hypopigmentation after CO(2) laser resurfacing. METHODS: We conducted a retrospective review of 447 consecutive patients who were treated with a 90-microsecond pulsed CO(2) laser (n = 229) or a continuous wave CO(2) laser with a modifiable dwell time (100-950 microseconds, n = 218). Follow-up ranged from 8 to 61 months (median: 27). RESULTS: A series of 4 threshold dwell times (range: 90-950 microseconds) were used to divide patients into 2 treatment groups (above and below) at each threshold. The rates of hypopigmentation between groups were similar (range: 6.37%-9.09%) and serial chi-square testing revealed no statistical differences between groups for each dwell time tested (P < 1.0). CONCLUSION: No significant relationship between pulse duration and the occurrence of hypopigmentation was observed.

Mid-dermal elastolysis preceded by acute neutrophilic dermatosis.

BACKGROUND: Mid-dermal elastolysis is a rare idiopathic elastic tissue disorder that is characterized by localized patches of finely wrinkled skin and a "band-like" loss of elastic tissue in the mid-reticular dermis. Lesions may be preceded by erythema and/or urticaria, and histological examination of inflamed lesional skin may demonstrate lymphohistiocytic dermal infiltration. CASE REPORT: We report a case of mid-dermal elastolysis in a 31-year-old woman who developed multiple erythematous and urticarial plaques on the arms and trunk. Histologic examination of a representative lesion revealed a neutrophilic infiltrate and a normal pattern of elastic tissue. Several months later, the erythema and urticaria was noted to have resolved, leaving soft, pendulous plaques with overlying finely wrinkled skin. A follow-up biopsy at this time showed minimal lymphocytic inflammation but almost complete absence of elastic tissue in the mid-reticular dermis. CONCLUSIONS: To our knowledge, acute neutrophilic dermatosis resulting in mid-dermal elastolysis has not been previously described. This observation lends support to an emerging theory that the pathogenesis of mid-dermal elastolysis may be inflammatory.

Acquired disorders of elastic tissue: Part I. Increased elastic tissue and solar elastotic syndromes

Elastic fibers in the extracellular matrix are an integral component of dermal connective tissue. The resilience and elasticity required for normal structure and function of the skin may be attributed to the network of elastic tissue. Advances in our understanding of elestic tissue physiology provide a foundation for studyng the pathogenesis of elastic tissue disorders. Many acquired disorders are nevertheless poorly understood due to the paucity of reported cases. Several acquired disorders in which accumulation or elastotic degeneration of dermal elastic fibers produces prominent clinical and histopathologic features have recenthy been described. They include elasdoterma, linear focal elastosis, and late-onset focal dermal elastosis and must be differentiated from better-known disorders, among them acquired pseudoxanthoma elasticum, elastosis perforans serpiginosa, and Favré-Racouchot syndrome.

Acquired disorders of elastic tissue: Part II. Decreased elastic tissue

Elastic fibers in the extracellular matrix are integral components of dermal connective tissue. The resilience and elasticity required for normal structure and function of the skin are attributable to the network of elastic tissue. Advances in our understanding of elastic tissue physiology provide a foundation for studyng the pathogenesis of elastic tissue disorders. Many acquired disorders are in which loss of dermal elastic tissue produces prominent clinical and histopathologic features have recently been described, including middermal elastolysis, papular elastorrhexis, and pseudoxanthoma-like papillary dermal elastolysis, which must be differentiated from more well-known disorders such as anetoderma, acquired cutis laxa, and acrokeratoelastoidosis.