Sluzevich, Jason C.

Abstract Background: Cutaneous T-cell neoplasms display markedly heterogeneous clinical behavior despite often similar pathologic features. For example, transformed mycosis fungoides (tMF) has a poor prognosis and requires intensive therapy whereas lymphomatoid papulosis (LyP) is an indolent lymphoproliferative disorder with spontaneously resolving lesions. These entities have overlapping histologic features and no molecular tools currently exist to aid in subclassification. Methods: We identified 11 T-cell neoplasms with histologic appearances distinct from previously described cutaneous T-cell neoplasms but with features overlapping those of both tMF and LyP. The phenotypic and genetic characteristics of the tumors were studied using immunohistochemistry and fluorescence in situ hybridization, respectively. Clinical data on presentation, treatment, evolution of the lesions, and follow-up were obtained. Results: All patients were older adults (mean age, 75 yr; range, 67-88 yr; 9M:2F). Clinical presentations were similar, with papulonodular skin lesions (0.3-1.0 cm) restricted to a single body area in all but two patients. Histologically there was an extensive atypical lymphoid infiltrate in the epidermis and a dermal tumor containing large transformed cells with numerous mitotic figures and apoptotic bodies. All tumors had a T-cell phenotype and expressed CD30 in both dermal and epidermal components. No case expressed ALK. Most cases had a high proliferative rate (>80%) by Ki67 staining. FISH analysis demonstrated rearrangements of the DUSP22-IRF4 locus on 6p25.3 in all cases. No patient had evidence of systemic disease on imaging studies. In 4 untreated patients, papules began to regress spontaneously a few weeks after presentation, with complete resolution within a month or two. Seven patients were treated with local excision (with or without cryotherapy) or radiotherapy. All non-excised lesions ultimately regressed. All patients were alive at last follow-up (median, 21 mo; range, 7-150 mo). Cutaneous recurrences occurred in 5 patients. At last follow-up only 1 patient had active lesions, which were confined to the presenting site. The remaining 10 patients had been disease-free from 2 to 32 months. Conclusions: Despite pathologic features mimicking an aggressive lymphoma, the benign clinical course observed in all 11 patients suggests these cases represent a newly recognized subtype of LyP. These neoplasms are characterized by 6p25.3 rearrangements, representing the first recurrent genetic abnormality identified in LyP. In contrast, we previously have shown absence of these rearrangements in tMF. Distinction of LyP from tMF is critical in this older patient population unlikely to tolerate intensive therapy. Thus, detection of 6p25.3 rearrangements represents a new tool for molecular classification of cutaneous T-cell neoplasms. Citation Format: Andrew L. Feldman, Laszlo J. Karai, Marshall E. Kadin, Eric D. Hsi, Jason C. Sluzevich, Rhett P. Ketterling, Ryan A. Knudson. Discovery of a previously undescribed cutaneous T-cell neoplasm with chromosomal rearrangements of 6p25.3. [abstract]. In: Proceedings of the 104th Annual Meeting of the American Association for Cancer Research; 2013 Apr 6-10; Washington, DC. Philadelphia (PA): AACR; Cancer Res 2013;73(8 Suppl):Abstract nr 1208. doi:10.1158/1538-7445.AM2013-1208

AbstractChronic cutaneous varicella zoster virus (VZV) infection has not been previously reported or characterized as a complication of dermatomyositis. Two patients with non‐malignancy‐associated dermatomyositis, treated with long‐term prednisone and methotrexate, developed persistent, painless ulcers ultimately established to be secondary to chronic VZV. The absence of pain or a history suggestive of acute VZV, and the lack of characteristic histopathology, resulted in a lengthy delay in diagnosis. Polymerase chain reaction and tissue immunohistochemistry were positive for VZV, and treatment with valacyclovir resulted in complete clearance. Diagnostic testing for VZV should thus be considered in the evaluation of ulcerative lesions in patients with dermatomyositis. The increased incidence of acute VZV in combination with the nature and duration of immunosuppressive treatment in this patient population may be contributory.

AbstractBackgroundVirtual microscopy is increasingly being used in dermatopathology educational settings.ObjectiveThe objective is to evaluate diagnostic accuracy and attitudes between virtual microscopy and traditional glass slide microscopy among dermatology residents.MethodsA 48‐question dermatopathology examination was administered to 35 dermatology residents at three different dermatology residency training sites during the 2011–2012 academic year with half (n = 24) of the questions using the gold standard of glass slide microscopy and half (n = 24) using whole, scanned virtual slides. Correct number of questions using glass slides and virtual slides was evaluated. Participants were surveyed regarding previous experience with digital slide imaging; quality, ease of use, and speed of slide review; and overall microscopy preferences.ResultsOverall, diagnostic accuracy was better with glass slides than virtual slides (p = 0.01). However, no statistically significant difference was found in diagnostic accuracy of first‐year trainees (p > 0.99) or trainees with exposure to virtual microscopy greater than two times per month (p = 0.27). There was no overall personal preference for glass slide vs. virtual microscopy.LimitationsDifferent cases and questions were used for glass slides and virtual microscopy.ConclusionsDiagnostic accuracy with virtual microscopy is dependent on year of residency training and prior experience with virtual microscopy.