Scanning Electron Microscopic Hair Shaft Analysis in Ectodermal Dysplasia Syndromes.

OBJECTIVES: The objective of the current study was to catalog hair shaft abnormalities in individuals with ectodermal dysplasia (ED) syndromes using scanning electron microscopy (SEM) and to compare the findings with those in unaffected controls. This is the second of a two-part study, the first of which used light microscopy as the modality and was previously published. METHODS: Scanning electron microscopy was performed in a blinded manner on hair shafts from 65 subjects with seven types of ED syndromes and 41 unaffected control subjects. Assessment was performed along the length of the shaft and in cross section. SETTING: Hair donations were collected at the 28th Annual National Family Conference held by the National Foundation for Ectodermal Dysplasia. Control subjects were recruited from a private dermatology practice and an academic children's hospital outpatient dermatology clinic. RESULTS: SEM identified various pathologic hair shaft abnormalities in each type of ED and in control patients. When hairs with all types of ED were grouped together and compared with those of control patients, the difference in the presence of small diameter and shallow and deep grooves was statistically significant (p < 0.05). When the EDs were separated according to subtype, statistically significant findings were also seen. CONCLUSION: SEM is a possible adjuvant tool in the diagnosis of ED syndromes. There are significant differences, with high specificity, between the hairs of individuals with ED and those of control subjects and between subtypes.

Persistent edematous-plaque photosensitivity observed with sitagliptin phosphate (Januvia®).

Photosensitivity to sitagliptin, a dipeptidyl peptidase-4 (DPP-4) inhibitor, is reported. No previous reports of photosensitivity to any DPP-4 inhibitors are known. Physical examination of the patient revealed edematous plaques confined to sun-exposed areas of the skin. An unusual finding in this case was the spongy sensation upon palpation of the plaques. Histopathology revealed parakeratosis and abundant eosinophils, supporting the clinical impression of cutaneous drug sensitivity. The eruption finally cleared, approximately two years after onset.

Comparative diagnostic accuracy in virtual dermatopathology.

BACKGROUND: Virtual microscopy can be used to teach histology and pathology and for in-training and certification examinations. A few online consultation websites already utilize virtual microscopy, thereby expanding the role of telemedicine in dermatopathology. There are, however, relatively few studies comparing the diagnostic accuracy and acceptability of virtual slides compared to traditional glass slides. METHODS: Ten Nordic dermatopathologists and pathologists were given a randomized combination of 20 virtual and glass slides and asked to identify the diagnoses. They were then asked to give their impressions about the virtual images. Descriptive data analysis and comparison of groups using Fisher's exact test were performed. OBJECTIVE: To compare the diagnostic ability of dermatopathologists and pathologists in two image formats: the traditional (glass) microscopic slides, and whole mount digitized images, and to elucidate their assessment of virtual microscopy in dermatopathology. RESULTS: Dermatopathologists and pathologists performed similarly in diagnosing dermatopathological disorders using virtual slides vs. glass slides, virtual 0.85 and glass 0.81, P=0.286. The order of administration of virtual or glass slides did not affect the percentage of questions answered correctly. Seven of nine participants completing the questionnaire, felt virtual microscopy is useful for both learning and testing. CONCLUSION: There was no significant difference in the participants' diagnostic ability using virtual slides compared with glass slides. Most participants agreed that virtual microscopy is a useful tool for learning and testing.

Sweet syndrome in infancy.

Sweet syndrome, or acute febrile neutrophilic dermatosis, is characterized by the presence of fever, peripheral leukocytosis, painful erythematous plaques and nodules, and a predominately neutrophilic dermal infiltrate. We report a case occurring in a 10-week-old male child, with preceding upper respiratory tract, and gastrointestinal infection symptoms. Sweet syndrome occurring in an infant should prompt a work-up for immunodeficiency, as well as a review of the peripheral blood smear to rule out the rare case of malignancy.

Patient safety: Part I. Patient safety and the dermatologist.

UNLABELLED: Congress is grappling with ways to fund health care in the future. Much of the focus rests on paying physicians for their patients' outcomes, rather than the current system of payment for services provided during each visit. The years ahead will be years of change for American health care, with an increasing emphasis on the comparison of patient outcomes and measures of quality. Patient safety initiatives will be an integral part of the overall strategy to improve American health care. Part one of this two-part series on patient safety examines what we know about patient safety in dermatology, including data from medicolegal claims and published data on patient safety in the setting of office-based surgery. The article also focuses on how medical societies, payers, the US government, and the Board of Medical Specialties are responding to calls for accountability and improvements in patient safety. LEARNING OBJECTIVES: After completing this learning activity, participants should be able to identify risks to patient safety based on an understanding of the major causes of legal claims against dermatologists, use published patient safety data to improve the practice of office surgery, and be able to improve patient safety through an understanding of requirements for maintenance of certification.

A fatal case of dermatomyositis with underlying metastatic esophageal adenocarcinoma.

Dermatomyositis (DM) is known to be associated with underlying malignancy, though the strength of this relationship and its predisposing factors are not clearly defined. We present a case of a patient who was first diagnosed with DM and, subsequently, metastatic esophageal adenocarcinoma. Despite aggressive immunosuppressive therapy, the patient's cutaneous eruption failed to resolve and his muscle weakness progressed. He had respiratory failure and died less than 2 months after his initial presentation. To our knowledge, this is only the second case of metastatic esophageal adenocarcinoma associated with DM reported in the English language literature.

Acquired acrodermatitis enteropathica secondary to alcoholism.

Acrodermatitis enteropathica is a zinc deficiency disorder characterized by well-demarcated, erythematous, eczematous plaques in a periorificial and acral distribution. Hereditary and acquired forms have been described. We report a case of acquired acrodermatitis enteropathica secondary to alcoholism. Treatment of the underlying disorder and zinc replacement therapy resulted in rapid resolution of the condition.

Laptop computer-induced erythema ab igne: a case report.

Erythema ab igne is a condition characterized by reticulated telangiectasia and hyperpigmentation caused by repeated long-term exposure to infrared radiation insufficient to produce a burn. We report a case of laptop computer-induced erythema ab igne.

Complement Activation and STAT4 Expression Are Associated with Early Inflammation in Diabetic Wounds.

Diabetic non-healing wounds are a major clinical problem. The mechanisms leading to poor wound healing in diabetes are multifactorial but unresolved inflammation may be a major contributing factor. The complement system (CS) is the most potent inflammatory cascade in humans and contributes to poor wound healing in animal models. Signal transducer and activator of transcription 4 (STAT4) is a transcription factor expressed in immune and adipose cells and contributes to upregulation of some inflammatory chemokines and cytokines. Persistent CS and STAT4 expression in diabetic wounds may thus contribute to chronic inflammation and delayed healing. The purpose of this study was to characterize CS and STAT4 in early diabetic wounds using db/db mice as a diabetic skin wound model. The CS was found to be activated early in the diabetic wounds as demonstrated by increased anaphylatoxin C5a in wound fluid and C3-fragment deposition by immunostaining. These changes were associated with a 76% increase in nucleated cells in the wounds of db/db mice vs. CONTROLS: The novel classical CS inhibitor, Peptide Inhibitor of Complement C1 (PIC1) reduced inflammation when added directly or saturated in an acellular skin scaffold, as reflected by reduced CS components and leukocyte infiltration. A significant increase in expression of STAT4 and the downstream macrophage chemokine CCL2 and its receptor CCR2 were also found in the early wounds of db/db mice compared to non-diabetic controls. These studies provide evidence for two new promising targets to reduce unresolved inflammation and to improve healing of diabetic skin wounds.

Enhanced cutaneous inflammatory reactions to Aspergillus fumigatus in a murine model of chronic granulomatous disease.

Chronic granulomatous disease is the manifestation of genetic defects of the leukocyte NADPH oxidase resulting in the absence of a respiratory burst. Patients with chronic granulomatous disease can develop chronic granulomas in many locations of the body, including the skin. Using an established murine model of X-linked chronic granulomatous disease (X-CGD) created by homologous recombinant disruption of the gene encoding the gp91phox component of the NADPH oxidase, in this study we examined cutaneous reactivity to sterile Aspergillus fumigatus hyphae. Injection of Aspergillus fumigatus into the dorsal ears of X-CGD mice resulted in an enhanced inflammatory response by 24 h, consisting of neutrophils, which developed into suppurative granulomas by 10 d. Intradermal injection of Aspergillus fumigatus into wild-type mice only resulted in a transient inflammatory response that resolved by 10 d. Injection of Aspergillus fumigatus into female carrier mice resulted in an acute inflammatory response that was similar to that of wild-type mice, but, at higher doses of Aspergillus fumigatus, many carriers subsequently developed granulomatous lesions that were qualitatively similar but smaller than those seen in X-CGD mice by 30 d. Consistent with the ability of X-CGD mice to mount an enhanced neutrophil-rich inflammatory response to Aspergillus fumigatus, significant levels of the potent neutrophil activator/chemoattractant leukotriene B4 were measured by mass spectrometry in skin biopsies at 24 and 72 h. In contrast to the exaggerated inflammatory response to intradermal Aspergillus fumigatus in X-CGD mice compared to their wild-type counterparts, similar levels of inflammation were seen in a model of delayed-type hypersensitivity using 2,4-dinitrofluorobenzene. This study represents the first report of a cutaneous granuloma model in mice with X-CGD, which may also prove useful as a functional test to evaluate the efficacy of gene therapy protocols being developed for chronic granulomatous disease.

Awareness of the risks of tanning lamps does not influence behavior among college students.

HYPOTHESIS: Awareness of the risks of artificial tanning influences tanning behavior among college students. OBJECTIVE: To correlate the prevalence of tanning lamp use, the perceived benefits and risks associated with UV exposure, and knowledge about skin cancer among university students. DESIGN: A survey was designed and administered to college students seeking "walk-in" care at a university student health center from September 7, 1999, through September 30, 1999. SETTING: A large midwestern public university student health center. PARTICIPANTS: Undergraduate and graduate students attending the student health center for any medical condition. INTERVENTION: None. MAIN OUTCOME MEASURE: Completion of the survey. RESULTS: Of the surveyed students, 47% had used a tanning lamp during the preceding 12 months. Female students were more common users than male students. Of the students surveyed, 39% reported never having used tanning lamps. More than 90% of users of tanning lamps were aware that premature aging and skin cancer were possible complications of tanning lamp use. CONCLUSIONS: Despite adequate knowledge of the adverse effects of UV exposure, university students freely and frequently use tanning lamps, primarily for desired cosmetic appearance. To alter this risky behavior will require a fundamental change in the societal belief that tans are attractive and healthy.

Vascular inflammation (vasculitis) in sweet syndrome: a clinicopathologic study of 28 biopsy specimens from 21 patients.

BACKGROUND: Sweet syndrome is characterized by painful, erythematous plaques of rapid onset accompanied by fever. Absence of vasculitis is a histologic criterion for diagnosis. However, recent reports suggest that vasculitis should not exclude the diagnosis. We hypothesized that vasculitis can occur in Sweet syndrome and that it represents an epiphenomenon rather than a primary immune-mediated process. DESIGN: Skin biopsy specimens from patients with Sweet syndrome were reviewed to determine the prevalence of vasculitis. The clinicopathologic features of cases with vasculitis were evaluated for statistically significant associations. Specimens with vasculitis underwent immunofluorescence staining. SETTING: University department of dermatology, university hospital, and private practice. PATIENTS: Medical records and biopsy specimens of 21 patients meeting diagnostic criteria for Sweet syndrome were reviewed. INTERVENTIONS: None. RESULTS: The prevalence of vasculitis was 29% (6 of 21 patients). There was a significant association of vasculitis with lesions of longer duration (P =.02). Vascular immunoglobulin and complement could not be demonstrated in cases of Sweet syndrome with vasculitis. CONCLUSIONS: Vasculitis is not a primary, immune-mediated process in Sweet syndrome but occurs secondary to noxious products released from neutrophils. Blood vessels in lesions of longer duration are more likely to develop vasculitis than those of shorter duration because of prolonged exposure to noxious metabolites. Vasculitis does not exclude a diagnosis of Sweet syndrome.

Histopathologic recognition of involved margins of lentigo maligna excised by staged excision: an interobserver comparison study.

OBJECTIVES: To assess interobserver and intraobserver concordance for identifying positive and negative margins in staged excisions of lentigo maligna and lentigo maligna melanoma and to determine if control biopsy specimens are useful to improve concordance. DESIGN: Retrospective, randomized interobserver and intraobserver comparison study of archived pathologic specimens. The study was conducted in 3 phases, and slides were evaluated blindly and independently by 5 pathologists: in phase 1, all slides were randomized and diagnosed as positive or negative. In phase 2, every third slide was evaluated again and diagnosed as positive or negative. In phase 3, slides were organized into cases, allowing evaluation of each margin in the context of the positive control (tumor from the center of the lesion) and negative control (control biopsy specimen), if available. SETTING: University referral center. STUDY MATERIAL: A total of 301 glass microscopic slides from 27 patients who underwent staged excision for lentigo maligna or lentigo maligna melanoma from March 1997 to April 2001. MAIN OUTCOME MEASURES: Interobserver and intraobserver concordance between original diagnoses and study diagnoses rendered on all slides by 5 pathologists. RESULTS: Phase 1 and 3 agreement was moderate (kappa range, 0.4-0.5). Phase 2 (intraobserver) agreement was moderate to good for all pathologists (kappa range, 0.6-0.9). Subset analysis revealed a statistically significant increase in agreement with the use of a control strip biopsy specimen for difficult slides. CONCLUSIONS: Interobserver concordance for margin analysis in lentigo maligna and lentigo maligna melanoma is moderate, and intraobserver concordance is moderate to good. A control strip biopsy specimen may improve concordance in some cases.

Nevi of special sites.

Melanocytic nevi can have a wide range of histologic appearances. Within the spectrum of nevi, there exists a group that presents in certain anatomic locations with histologically worrisome features but nonetheless benign behavior. This group of nevi has been broadly categorized as nevi of special sites. The anatomic locations affected by this group include the embryonic milkline (breast, axillae, umbilicus, genitalia), flexural areas, acral surfaces, ear, and scalp. Nevi in these locations may be mistaken for melanomas because of their histologic appearance, resulting in inappropriate overtreatment of patients. In this article, the authors review the histologic features of these special site nevi and discuss the criteria that help distinguish them from melanoma.