Metastatic melanoma and melanogenuria.

We report a case of a 70-year-old Hawaiian man with an exophytic black nodule on the left suprascapular region of several years' duration. Histopathologic examination of the excised lesion showed a nodular melanoma with 17-mm Breslow thickness. The patient had firm fixed lymph nodes circumferentially around his neck. He underwent palliative cervical lymph node dissection to remove the compressive nodes but declined further therapy. One year later, the patient's skin was noted to have a generalized uniformly gray-brown color. Physical examination showed ulcerated masses on his trunk, right arm, and both axillae. A urine specimen initially was dark yellow but turned black after exposure to air at room temperature and ambient light for several minutes. Black urine, termed melanuria, is a rare finding in patients with disseminated melanoma. In melanogenuria, the urine is yellow and darkens as the colorless melanin precursors oxidize in the presence of air. Detection of these urinary melanin precursors may someday help determine the prognosis of melanoma and monitor response to treatment.

Lichen planus pigmentosus-inversus: case report and review of an unusual entity.

Lichen planus pigmentosus-inversus is a rare variant of lichen planus, with less than 20 cases reported in the medical literature. It presents as asymptomatic to mildly pruritic, hyperpigmented macules and/or patches involving intertriginous and flexural areas and skin folds in light-skinned individuals. The unique pattern of skin involvement, clinical features, and histology are distinctive. The combination of hyperpigmented lesion(s) isolated to non-sun exposed, intertriginous, and flexural areas with lichenoid histology is unique and separates it from other similar entities, such as lichen planus actinus and erythema dyschromicum perstans/ashy dermatosis. The case well highlights this unusual condition and represents the first case reported in the United States.

Cutaneous granulomatous vasculitis associated with chronic lymphocytic leukemia: case report and review of literature of a rare entity.

Cutaneous granulomatous vasculitis associated with lymphoproliferative disorders is a rare entity, with only 14 cases previously reported in the English literature. Patients generally present with nodules or ulcers involving the extremities, which can appear months or years before or after onset of systemic disease. Granulomatous vasculitis has a poor prognosis when associated with underlying lymphoproliferative disorders, with the majority of reported cases fatal. Knowledge of this unusual entity is important to allow for proper clinical evaluation, follow-up, and therapy. We report a 77-year-old female with chronic lymphocytic leukemia and granulomatous vasculitis, which highlights the features of this association, and expands the clinical data.

Onychomatricoma: case report of an unusual subungual tumor with literature review.

Onychomatricoma is rare subungual tumor of nail matrix origin. We report a 40-year-old white man with an onychomatricoma of the left index finger that highlights the characteristic clinical and pathological features and expands the known case material. We also review the published medical literature on the subject.

Interobserver and intraobserver variability of a two-tier system for grading ovarian serous carcinoma.

Although grading has been demonstrated to be an important prognostic factor in ovarian serous carcinoma, there is no system universally used to perform this task. A few years ago, we proposed a two-tier system for grading ovarian serous carcinoma that is based primarily on the assessment of nuclear atypia (uniformity vs. pleomorphism) in the worst area of the tumor. Tumor grade in this two-tier system is correlated with survival. After being used by numerous pathologists and trainees at The University of Texas M.D. Anderson Cancer Center (MDACC) for 15 years, we have observed that this system is user-friendly and reproducible. We undertook this study to evaluate the interobserver and intraobserver variability among a group of 7 gynecologic pathologists and 2 general surgical pathologists using this grading system. A total of 80 cases of ovarian serous carcinoma, 40 low-grade and 40 high-grade, were circulated twice among these pathologists. Slides with examples of low-grade and high-grade serous carcinoma were sent with the unknowns. A website was used to provide diagnostic criteria, images of examples of ovarian low-grade and high-grade carcinoma, and a log form to facilitate data entry. Statistical analysis demonstrated an overall kappa statistic among the different observers of 0.909. The intergrader kappa's ranged from 0.717 to 1.000 in the first round of the review and from 0.701 to 1.000 in the second round. Eight of the participants had an intragrader kappa ranging from 0.775 to 1.000 (excellent agreement), whereas a single participant had an intragrader kappa of 0.725 (good agreement). This study demonstrates that the two-tier grading system (the MDACC grading system) for ovarian serous carcinoma on the basis of the assessment of nuclear atypia is easy to learn and is highly reproducible. These findings would support its universal use, which would be beneficial for the standardization of clinical trials and protocols, thus facilitating the understanding of this disease and investigation into the treatment of patients affected by these tumors.

Fine-needle aspiration of renal angiosarcoma.

Angiosarcoma of the kidney is an unusual neoplasm, and primary renal angiosarcoma is exceedingly rare, with fewer than 11 well-documented cases reported to date. To our knowledge, no publication to date has correlated the fine-needle aspiration cytologic findings in renal angiosarcoma with the gross, histologic, and immunohistochemical findings. A 50-year-old man presented with a left kidney mass and multiple liver and pulmonary nodules. Computed tomography-guided fine-needle aspiration biopsies of the renal mass and a hepatic nodule were performed and demonstrated malignant spindle cells consistent with angiosarcoma. The diagnosis was confirmed at autopsy through histologic examination and associated ancillary studies. This case presents the fine-needle aspiration cytologic findings in renal angiosarcoma and correlates these findings with the gross pathologic, histologic, and immunohistochemical findings.

Dermatologic complications from levamisole-contaminated cocaine: a case report and review of the literature.

Levamisole is a veterinary anthelmintic drug with immunomodulatory properties in humans. It has become increasingly common as a contaminant in cocaine and is now detected in the majority of cocaine seized in the United States. A variety of adverse reactions have been reported in association with levamisole, the most severe being agranulocytosis, vascular occlusive disease, and thrombotic vasculopathy, with or without vasculitis. The combination of rapidly progressive cutaneous ecchymosis and purpura leading to necrosis, often affecting the ears and cheeks; neutropenia or agranulocytosis; serologic autoantibodies; and thrombotic vasculopathy, with or without associated vasculitis, in a patient who has recently used cocaine is characteristic of exposure to contaminant levamisole. We report the case of a 54-year-old man who presented with the clinical findings of levamisole-contaminated cocaine use and review the literature regarding cutaneous reactions associated with levamisole. Our case highlights this important public health issue and represents a clinical course that is unusually severe.

Incidental cutaneous reaction patterns: epidermolytic hyperkeratosis, acantholytic dyskeratosis, and hailey-hailey-like acantholysis: a potential marker of premalignant skin change.

Focal acantholytic dyskeratosis (FAD), epidermolytic hyperkeratosis (EHK), and Hailey-Hailey-like acantholysis (HH) represent unique histology reaction patterns, which can be associated with defined phenotypic and genotypic alterations. Incidental microscopic foci demonstrating these patterns have been identified in skin and mucosal specimens in association with a gamut of disease processes. These changes, when secondary, are of unclear etiology and significance. The following study further analyzes the incidence and association of these histologic patterns in a routine pathology/dermatopathology practice.

A randomized comparison of methylene blue-directed biopsy versus conventional four-quadrant biopsy for the detection of intestinal metaplasia and dysplasia in patients with long-segment Barrett's esophagus.

OBJECTIVES: Methylene blue (MB) selectively stains specialized intestinal metaplasia (SIM) and may assist in surveying a columnar-lined esophagus for Barrett's esophagus associated dysplasia. METHODS: This is a prospective, randomized crossover study comparing 4-quadrant random biopsies (4QB) versus MB-directed biopsies for the detection of SIM and dysplasia in 48 patients with long segment Barrett's esophagus (LSBE). Patients randomly underwent two endoscopies over a 4-wk time period with either 4QB or MB-directed biopsies as their first or second exam. Our aim was to correlate stain intensity with histology. RESULTS: The sensitivity of MB for SIM and dysplasia was 75.2% and 83.1%, respectively. The yield of 4QB for identifying nondysplasia SIM was 57.6% (523/917) and for dysplasia was 12% (111/917). Dark staining was significantly associated with histologic grade (P < 0.007). The final diagnosis was correct in 43 (90%) patients using MB and in 45 (94%) using 4QB. The 4QB technique missed dysplasia in 3 of 21 patients while MB biopsies missed dysplasia in 5 of 21 patients. The discordance between the two techniques was not significant (P= 0.727, McNemar's test). The mean number of biopsies taken during 4QB was 18.92 +/- 6.36 and with MB was 9.23 +/- 2.89 (P < 0.001). CONCLUSION: MB requires significantly fewer biopsies than 4QB to evaluate for SIM and dysplasia. Dark staining correlates more with HGD than LGD in our experience. While MB is not more accurate than 4QB, MB may help to define areas to target for biopsy during surveillance endoscopy in patients with LSBE.

Use of methylene blue for detection of specialized intestinal metaplasia in GERD patients presenting for screening upper endoscopy.

The ability of randomly obtained biopsy specimens to identify intestinal metaplasia in the distal esophagus is low. Use of vital staining has been suggested, as stains are taken up by areas histologically identified as specialized intestinal metaplasia (SIM). This study evaluated the role of methylene blue (MB) staining for identification of SIM in GERD patients undergoing a screening endoscopy. Chromoendoscopy of the distal esophagus using 1% MB was performed on 52 GERD patients presenting for their first endoscopy. Biopsies were obtained from areas that were stained darkly, stained lightly, unstained, or macroscopically abnormal. In patients with no MB staining, four-quadrant biopsy of the distal esophagus was performed. Twenty-seven patients (52%) showed staining with MB, while 25 patients did not. Two hundred sixty-six biopsies were obtained. SIM was detected in 11 (21%) subjects (SIM+) but not in the remaining 41 (SIM-). One hundred sixty-five biopsies were unstained (25 SIM+, 140 SIM-) and 101 were stained (12 SIM+, 89 SIM-). The per-biopsy sensitivity and specificity of MB for detection of SIM were 32.4 and 85%, while the per-patient sensitivity and specificity were 63.3 and 51.2%. MB staining for detection of SIM in GERD patients without a macroscopic appearance suggestive of a columnar-lined esophagus is a poor screening tool for SIM.

Papular spongiotic dermatitis of smallpox vaccination: report of 2 cases with review of the literature.

We report 2 cases of nonspecific postvaccinial dermatitis following smallpox vaccination. The patients presented with diffuse, pruritic, erythematous macules and papules 11 days (case 1) and 7 days (case 2) following routine smallpox vaccination. Biopsies of the lesions demonstrated spongiotic dermatitis without evidence of viral cytopathic changes. One case showed a pityriasis rosea-like histologic pattern. The exanthema resolved without sequelae with symptomatic treatment (case 1). Review of historical literature demonstrated the association of a variety of nonspecific cutaneous complications with vaccinia inoculation, including erythema multiforme, urticaria, and pityriasis rosea. The association of these various dermatitides with smallpox immunization is not well known and is likely underreported.