Anthony P. Fernandez

FISH for MYC amplification and anti-MYC immunohistochemistry: useful diagnostic tools in the assessment of secondary angiosarcoma and atypical vascular proliferations.

Background: Secondary angiosarcoma and benign but microscopically atypical vascular proliferations (herein referred to as atypical vascular lesion or AVL) are rare consequences of radiation therapy and/or chronic lymphedema most commonly seen in breast cancer patients. Differentiating angiosarcoma from AVL can be difficult due to overlapping clinical and microscopic features. Recently, amplification of MYC has been associated with 55–100% of secondary angiosarcomas but is reportedly absent in AVL. We examined a series of secondary angiosarcoma and AVL for MYC amplification by fluorescence in situ hybridization (FISH) and expression by immunohistochemistry to investigate the diagnostic utility for discriminating angiosarcoma from AVL.

The Role of the Immune Response in Merkel Cell Carcinoma

Merkel cell carcinoma (MCC) is an aggressive neuroendocrine skin cancer. The Merkel cell polyomavirus (MCPyV) is implicated in its pathogenesis. Immune mechanisms are also implicated. Patients who are immunosuppressed have an increased risk. There is evidence that high intratumoral T-cell counts and immune transcripts are associated with favorable survival. Spontaneous regressions implicate immune effector mechanisms. Immunogenicity is also supported by observation of autoimmune paraneoplastic syndromes. Case reports suggest that immune modulation, including reduction of immune suppression, can result in tumor regression. The relationships between MCPyV infection, the immune response, and clinical outcome, however, remain poorly understood. Circulating antibodies against MCPyV antigens are present in most individuals. MCPyV-reactive T cells have been detected in both MCC patients and control subjects. High intratumoral T-cell counts are also associated with favorable survival in MCPyV-negative MCC. That the immune system plays a central role in preventing and controlling MCC is supported by several observations. MCCs often develop, however, despite the presence of humoral and cellular immune responses. A better understanding on how MCPyV and MCC evade the immune response will be necessary to develop effective immunotherapies.

Psoriasis improvement after bariatric surgery

BACKGROUND Psoriasis is a chronic inflammatory skin disease known to be associated with obesity and metabolic syndrome. Single case reports and small series suggest remission or improvement after bariatric surgery, hypothetically through a GLP-1 mediated mechanism. The objective of this study was to investigate on the effect of bariatric surgery on the clinical behavior of psoriasis in obese patients. METHODS A total of 33 morbidly obese individuals with psoriasis who were on active medical treatment were identified. Demographic characteristics and follow-up data were extracted from our database. Medication usage and percentage of affected body surface area (%ABSA) were recorded preoperatively and at least 6 months after bariatric surgery. RESULTS Nine (27.2%) patients were on systemic therapy at baseline. At a mean follow-up time of 26.2±20.3 months, a mean excess weight loss (EWL) of 48.7± 26.6% was achieved. This was associated with improvement of psoriasis based on downgrade of medication and %ABSA in 30.3% and 26.1% of patients, respectively. In total, 13 of 33 patients (39.4%) had improvement based on either criteria. Eight (24.2%) patients were not on any psoriasis medication at the latest follow-up (P = .001). Older age at the time of surgery (54.8±8.1 versus 48.1±10.4 years, P = .047), Roux-en-Y gastric bypass versus nonbypass procedures (52.4% versus 16.7%, P = .043), and greater EWL (64.2±26.0% versus 43.4± 23.6%, P = .036) predicted improvement. Only 1 (3%) patient experienced worsening after surgery. CONCLUSION Almost 40% of our cohort showed improvement of psoriasis several months after bariatric surgery. Improvement is directly related to the degree of postoperative weight loss and is associated with the Roux-en-Y configuration.

Microvenular hemangioma: a clinicopathologic review of 13 cases.

Microvenular hemangioma is an uncommon benign vascular tumor that can potentially be mistaken for malignancy.

Morphologic mimickers of Cryptococcus occurring within inflammatory infiltrates in the setting of neutrophilic dermatitis: a series of three cases highlighting clinical dilemmas associated with a novel histopathologic pitfall

A neutrophil‐predominant inflammatory infiltrate in a cutaneous biopsy can be associated with a broad spectrum of diseases. Here we describe three cases showing a neutrophil‐predominant dermal infiltrate admixed with abundant acellular bodies surrounded by capsule‐like vacuolated spaces, which strikingly mimicked Cryptococcus. Two cases occurred within the settings of underlying hematologic malignancies; the third case was associated with immune dysregulation. Two patients were acutely ill in the medical intensive care unit. Fungal work‐up, including cultures and multiple stains were negative. Because of clinical deterioration in these patients, transmission electron microscopy was pursued to definitively rule out fungal infection. In both cases, characteristics most compatible with autolysing human cells, not Cryptococcus, were identified. Chemotherapy and high‐dose steroids were given, but both patients eventually succumbed to their diseases. To the best of our knowledge, these represent the first reported cases of autolysing human cells mimicking Cryptococcus organisms within neutrophilic infiltrates. They highlight the therapeutic dilemmas arising with histopathologic mimics, as well as the importance of thorough investigation to distinguish mimickers from true infectious organisms. We believe recognition of this microscopic pitfall will be useful to dermatopathologists faced with similar findings in the future, and may prevent unnecessary delay of appropriate therapy in acutely ill patients.

Langerhans cell collections, but not eosinophils, are clues to a diagnosis of allergic contact dermatitis in appropriate skin biopsies

Langerhans cell collections (LCCs) and eosinophils are traditionally considered histologic clues to allergic contact dermatitis (ACD), but rigorous histologic analyses are limited. We correlated the presence of LCCs and eosinophils in skin biopsies with patch test results in patients evaluated for ACD.

Fully automated dual-color dual-hapten silver in situ hybridization staining for MYC amplification: a diagnostic tool for discriminating secondary angiosarcoma

MYC amplification occurs in post‐radiation and chronic lymphedema‐associated secondary angiosarcoma and some primary angiosarcomas. In this study, we tested the ability of automated dual‐color dual‐hapten in situ hybridization (DISH) staining to discriminate secondary angiosarcoma from radiation‐associated atypical vascular lesions (AVL), and to correlate with fluorescence in situ hybridization (FISH) for MYC amplification.

Clinically amyopathic dermatomyositis: clinical features, response to medications and malignancy-associated risk factors in a specific tertiary-care-centre cohort.

Clinically amyopathic dermatomyositis (CADM) is a subset of dermatomyositis (DM) characterized by the typical DM cutaneous manifestations but without myositis. There is a relative paucity of characterized cases of CADM in the peer‐reviewed medical literature.

Intravenous immunoglobulin for treatment of dermatomyositis-associated dystrophic calcinosis

REFERENCES 1. Lee WJ, Jung JM, Won CH, et al. Cutaneous extranodal natural killer/T-cell lymphoma: a comparative clinicohistopathologic and survival outcome analysis of 45 cases according to the primary tumor site. J Am Acad Dermatol. 2014;70:1002-1009. 2. Kim YH, Willemze R, Pimpinelli N, et al, ISCL and the EORTC. TNM classification system for primary cutaneous lymphomas other than mycosis fungoides and S ezary syndrome: a proposal of the International Society for Cutaneous Lymphomas (ISCL) and the Cutaneous Lymphoma Task Force of the European Organization of Research and Treatment of Cancer (EORTC). Blood. 2007;110:479-484. 3. Au WY, Weisenburger DD, Intragumtornchai T, et al, International Peripheral T-Cell Lymphoma Project. Clinical differences between nasal and extranasal natural killer/T-cell lymphoma: a study of 136 cases from the International Peripheral T-cell Lymphoma Project. Blood. 2009;113:3931-3937. 4. Choi YL, Park JH, Namkung JH, et al. Extranodal NK/T-cell lymphoma with cutaneous involvement: ‘‘nasal’’ vs ‘‘nasal-t ype’’ subgtroups—a retrospective study of 18 patients. Br J Dermatol. 2009;160:333-337. 5. Mraz-Gernhard S, Natkunam Y, Hoppe RT, LeBoit P, Kohler S, Kim YH. Natural killer/natural killer-like T-cell lymphoma, CD561, presenting in the skin: an increasingly recognized entity with an aggressive course. J Clin Oncol. 2001;19:2179-2188.

Kasabach-Merritt phenomenon in an adult man with a tufted angioma and cirrhosis responding to radiation, bevacizumab, and prednisone

Tufted angioma (TA) is a benign cutaneous vascular tumor that most commonly occurs in young children. TAs have rarely been reported in adults and/or arising within port‐wine stains (PWS). Kasabach–Merritt phenomenon (KMP) is a life‐threatening consumptive coagulopathy hypothesized to occur specifically in young children who develop either a TA or kaposiform hemangioendothelioma.