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A 12-year-old female with oligoarticular juvenile inflammatory arthritis developed an atrophic linear plaque involving the left medial forearm and proximal arm 7 months after intra-articular triamcinolone injection for arthritis. The plaque spontaneously resolved without treatment over approximately one year. It is important to recognize this rare complication of intra-articular steroid injection in order to avoid potential misdiagnosis as linear scleroderma and subsequent immunosuppressive treatment.

Pityriasis rubra pilaris (PRP) is a rare, chronic, heterogeneous, papulosquamous inflammatory dermatosis of unknown etiology. Although erythematous scaly patches characterize the classic presentation of PRP, a broad range of clinical presentations has been reported. Histologically, PRP is characterized by psoriasiform acanthosis with alternating orthokeratosis and parakeratosis and rarely small acantholytic foci. In this article, we report a patient who presented with diffuse erythroderma and extensive acantholysis mimicking pemphigus vulgaris histologically.

Cutaneous Rosai-Dorfman disease (RDD) can be difficult to distinguish from other non-Langerhans cell histiocytoses, particularly xanthogranuloma (XG). Pathologists use S100 immunoreactivity, abundant plasma cells, and the presence of emperipolesis to distinguish RDD from XG. However, S100 expression has been reported in XG and, in practice, we have occasionally observed emperipolesis in cases that were otherwise clinically and pathologically consistent with XG. We present 10 cases of XG with emperipolesis and variable S100 immunoreactivity. Histologically, 7 cases were most in keeping with XG, and a histologic differential of XG versus RDD was raised in the remaining 3 cases. All 10 cases were clinically consistent with XG. Notably, none of these cases showed abundant plasma cells. Nine cases showed variable S100 immunostaining, ranging from focal/weak expression, to focal/strong, diffuse/moderate, and diffuse/strong expression. Histiocytes in all cases were CD68 positive and CD1a negative. We conclude that emperipolesis and S100 expression in a skin biopsy cannot reliably distinguish XG from cutaneous manifestations of RDD. Clinical correlations are essential, as are histologic clues to a diagnosis of classic XG that include an abundance of foamy mononuclear cells, Touton giant cells, and an absence of pale-stained histiocytes, abundant plasma cells, fibrosis, or vascular proliferation.

Not Applicable

Painless Skin Nodule on the Finger of an 18 Year Old Andrea Olofson;Shaofeng Yan;Konstantinos Linos; The American Journal of Dermatopathology

Benign cutaneous plexiform hybrid tumor of perineurioma and cellular neurothekeoma (BCPHTPCN) is a recently described entity that presents as a solitary papule in the perioral area. As implied by its name, BCPHTPCN displays microscopic features of both perineurioma and cellular neurothekeoma arranged in a plexiform pattern. We report a case of nonplexiform benign cutaneous biphasic hybrid tumor of perineurioma and cellular neurothekeoma in a 36-year-old woman, who presented with a 4-year history of a firm, flesh-colored left ankle nodule. Histologically, there was a biphasic, well-circumscribed unencapsulated dermal mesenchymal proliferation with no connection to the epidermis, which exhibited mild acanthosis with slightly pigmented basal keratinocytes and overlying parakeratosis. The proliferation consisted of uniform bland spindle cells with bipolar cytoplasmic processes arranged in whorls with interspersed islands of epithelioid cells. Immunohistochemically, the spindle cell component was positive for CD34, EMA, and GLUT-1, consistent with perineurial differentiation, whereas the epithelioid nests were positive for NKI/C3 and MiTF, as expected in neurothekeoma. Stains for S100 protein, SOX10, desmin, claudin, pan-melanoma markers, and NSE were negative. We believe this case expands the histopathologic spectrum of BCPHTPCN showing that it can be grown in a nonplexiform pattern, and we suggest the term benign cutaneous biphasic hybrid tumor of perineurioma and cellular neurothekeoma as a more precise name. It is also, to the best of our knowledge, the first case reported outside the head and neck area.

Nevus sebaceus can give rise to multiple neoplasms, some of which have the potential to become malignant. We describe the occurrence of a previously unreported combination of a cutaneous horn proliferating from a trichilemmoma overlying a multilocular pilar cyst and nevus sebaceus. An elderly woman with a several years’ history of multiple scalp subcutaneous nodules and an enlarging cutaneous horn presented to our clinic. The nodule underlying the large exophytic horn was tender and occasionally bled. The lesion was removed by excisional biopsy due to concern for malignancy. Histologically, a verrucous trichilemmoma was identified overlying irritated pilar cysts and a nevus sebaceus. Phosphatase and tensin homolog (PTEN) mutations are frequently found in trichilemmomas and HRAS mutations in nevus sebaceus. Mammalian target of rapamycin (mTOR) is a part of the final common pathway for HRAS and PTEN raising the therapeutic question if rapamycin can be used to treat these neoplasms.

CIC-rearranged sarcomas comprise a recognized subset of the so called “undifferentiated small round cell sarcomas” that lack the traditional EWSR1 fusion to one of the ETS family members.1 A smaller percentage have demonstrated BCOR gene rearrangements.2 The CIC gene is most commonly fused with the DUX4 or DUX4L10 genes through t(4;19)(q35;q13) or t(10;19)(q26;q13). An alternative fusion partner, the FOXO4 (Xq13.1) gene, has recently been identified.3 CIC-rearranged sarcomas have a distinct gene signature compared to classic Ewing sarcomas with upregulation of ETS transcription factors and WT1. They are more common in adults, in soft tissue locations as opposed to bone, and they have a mixed response to neo/adjuvant traditional Ewing sarcoma chemotherapy protocols.4 This article is protected by copyright. All rights reserved.

We present a case of a patient with long-standing hyperpigmented macules and erythematous papules over his chest, abdomen, back and arms, suggestive of Dowling-Degos disease (DDD). In addition, there were hyperkeratotic papules, alternating red and white nail-bed discolouration, and V-shaped nail notching consistent with Darier disease (DD). Histology showed findings consistent with DDD and DD on separate specimens. The lack of acantholysis in areas of filiform hyperpigmented rete ridges ruled out Galli-Galli disease (GGD). DDD results from mutations in the genes encoding keratin 5 (KRT5), protein O-glucosyltransferase 1 (POGLUT1) or protein O-fucosyltransferase 1 (POFUT1), while DD results from mutations in the ATP2A2 gene. Both genes are present on chromosome 12. In this case, the patient presented with features of both DDD and DD, which suggests that either a cooperating mutation or a mutation in an unrelated gene locus may underlie the findings in this patient.

Background Appropriate use criteria (AUC) provide physicians guidance in test selection, can affect health care delivery, reimbursement policy, and physician decision-making. Objectives The American Society of Dermatopathology (ASDP), with input from the American Academy of Dermatology (AAD) and the College of American Pathologists (CAP), sought to develop AUC in dermatopathology. Methods The RAND/UCLA appropriateness methodology, which combines evidence-based medicine, clinical experience and expert judgment, was used to develop AUC in dermatopathology. Results With the number of ratings predetermined at 3, AUC were developed for 211 clinical scenarios (CS) involving 12 ancillary studies (AS). Consensus was reached for 188 (89%) CS, with 93 (44%) considered "usually appropriate", 52 (25%) "rarely appropriate", and 43 (20%) "uncertain appropriateness". Limitations The methodology requires a focus on appropriateness without comparison between tests and irrespective of cost. Conclusions The ultimate decision of when to order specific test rests with the physician and is one where the expected benefit exceeds the negative consequences. This publication outlines the recommendation of appropriateness - AUC for 12 tests used in dermatopathology. Importantly, these recommendations may change considering new evidence. Results deemed "uncertain appropriateness" and where consensus was not reached may benefit from further research.

BACKGROUND Dermatofibrosarcoma protuberans (DFSP) is a tumor of intermediate malignancy, which in selected circumstances can pose difficulty in diagnosis. Clear cell sarcoma (CCS) is a very rare aggressive soft tissue sarcoma that can be difficult to distinguish histologically from melanoma. METHODS The current literature on t(17;22) COL1A1-PDGFB fluorescence in situ hybridization (FISH) assay in DFSP was reviewed. Also reviewed was the current literature on dual color break-apart EWSR1 FISH assay in CCS. Finally, the current utilization patterns of these tests was assessed in attendees of the American Society of Dermatopathology annual meeting (Chicago, 2016). RESULTS The literature indicates that (17;22) COL1A1-PDGFB FISH assay has limited value for classic DFSP, where the diagnosis can be established by routine morphology and immunohistochemistry. Given the high specificity of the EWSR1 FISH assay and significant complexity in the diagnosis of CCS, this ancillary study is helpful in distinguishing CCS from melanoma. CONCLUSIONS In attendees, t(17;22) COL1A1-PDGFB FISH testing for classic cases of DFSP is appropriately not being used by respondents. However, the literature sustains that it is useful in selected circumstances in which a definitive diagnosis is challenging. The majority of respondents are utilizing the EWSR1 FISH assay to distinguish CSS from melanoma as is supported by the literature.

Myopericytoma is a benign mesenchymal neoplasm thought to comprise part of a spectrum of perivascular myoid cell neoplasms with myofibroma, angioleiomyoma, and glomus tumor. We describe 2 such neoplasms involving the urinary tract: 1 incidentally identified in the kidney of a 59-year-old woman and 1 in the urinary bladder of a 52-year-old woman who presented with urinary frequency and dysuria. Histologically, the bladder tumor was composed of numerous blood vessels surrounded by plump perivascular myoid cells, as in subcutaneous myopericytoma. The renal tumor showed similar morphology centrally and a symplastic glomus tumor-like growth pattern at the periphery. Immunohistochemically, both tumors were reactive for markers of smooth muscle differentiation, such as smooth muscle actin and caldesmon/calponin but negative for CD34, cathepsin K, and S100 protein. Both patients are free of disease 14 and 39 months after resection, respectively. Our findings broaden the morphologic spectrum of myopericytoma.

Primary mucinous carcinoma (PCMC) is a rare, slow-growing mucinous variant of sweat gland tumors. Clinically, PCMC presents with a variety of morphologies and most commonly affects the eyelid or brow followed by the neck and scalp.1 According to a recent meta-analysis by Kamalpour et al,1 approximately 215 cases of PCMC have been reported from 1952 to 2010, with an increased prevalence in whites, elderly, and men. Most of these cases (136 of 159) were treated with traditional surgical excision with 1-cm minimum margins, and only a limited number (15 of 159) were treated with MMS. Of the patients treated with Mohs micrographic surgery (MMS), 2 (13%) had recurring disease with no reported metastases. In contrast, 46 patients (34%) either had recurrence of disease or disease that metastasized when treated with wide local excision. Overall, the rate of PCMC metastasis was low (6.1%), and the rate of local recurrence was high (19.6%). Mean follow-up time was 30.1 (standard deviation, 39.4) months for patients treated with excision and 23.1 (standard deviation, 17.5) months for patients treated with MMS.1 We describe a patient with PCMC of the eyelid treated with MMS who has shown no evidence of recurrence for a longer follow-up period than currently reported in the literature.

To characterise clinicopathological features and clinical outcomes of the genitourinary tract solitary fibrous tumours, incorporatingThe presence of the molecular hallmarkEleven solitary fibrous tumours were diagnosed in eight male patients and three female patients with a mean age of 46 years (range: 11-64 years). Four of the tumours had malignant histological features, and three were considered moderate risk for metastasis. With a mean follow-up time of 61 months, 1 recurred locally and 2 presented at distant metastatic sites. Using a break-apart FISH probe cocktail, we found theA subset of solitary fibrous tumours of the genitourinary tract behaved aggressively. Using a break-apart FISH probe cocktail, we found the

Nodular fasciitis is a self-limiting benign fibroblastic/myofibroblastic proliferation, which typically presents as a rapidly growing mass resembling an aggressive lesion clinically. It can also mimic a sarcoma histologically, hence the frequent characterization as "pseudosarcoma." We describe a case of a 53-year-old man who presented with a posterior chest wall mass that on imaging showed erosion into the adjacent ribs. After resection, the diagnosis of nodular fasciitis was rendered. Bone erosion by nodular fasciitis is extremely rare and can resemble a malignant neoplasm radiologically.

We present the case of a male infant with violaceous bullae on the scalp that were initially thought to be bullous aplasia cutis but at 3 months of age were diagnosed as a kaposiform hemangioendothelioma. This diagnosis should be considered when evaluating newborns with bullous plaques on the scalp that do not heal in the first 2-3 weeks of life. Kaposiform hemangioendothelioma (KHE) is a rare vascular tumor that typically presents as a violaceous to purpuric plaque at birth or early infancy. It may be associated with Kasabach-Merritt phenomenon (KMP), a potentially life-threatening consumptive coagulopathy.

A micropapillary variant of prostatic acinar adenocarcinoma has not been reported in the literature. Herein, we report a case of a 50-year-old patient who presented with an elevated prostate-specific antigen concentration and was subsequently diagnosed with prostatic acinar adenocarcinoma on biopsy. Radical prostatectomy specimen revealed prostatic carcinoma with Gleason score 4 + 5 = 9/10, with micropapillary component constituting 80% of tumor volume. Immunohistochemical studies of the prostate carcinoma showed a homogeneously positive prostate-specific antigen and α-methylacyl-CoA racemase, high-molecular-weight cytokeratin, and p63 protein cocktail pattern of staining in both micropapillary and conventional components. Pelvic lymph nodes were negative for metastatic disease. In contrast to the aggressive behavior of micropapillary carcinomas of other organs, the disease in our patient has thus far followed a more benign course, with low stage on presentation and a 2-year follow-up free of disease. However, prognostic correlation should be established on large series in order to assign this variant to a grade category within the Gleason scheme.

We report a case of an extra nuchal-type fibroma in a 51-year-old male suspected to have attenuated familial adenomatous polyposis (Gardner's syndrome), who presented with a longstanding buttock mass excised due to enlargement and pain. Histopathologically, lobules of haphazard, hypocellular, hyalinized collagen bundles replaced the dermis and subcutis and entrapped nerve bundles, mimicking Morton neuroma. Ramifying nerve twigs found around larger nerve fascicles showed the co-existence of traumatic neuroma. Elastic tissue stain revealed elastosis characterized by large, arborizing fibers lying between and within the hyalinized collagen bundles. Modified Masson's trichrome stain showed light blue staining of collagen bundles producing the hyalinized nodules with irregular, light red staining of collagen bundles at their periphery and within tumor collagen. Compression and/or degeneration of collagen and secondary elastosis with later entrapment by tumor collagen could explain this microscopic phenotype. By immunohistochemistry, tumor spindle cells expressed nuclear β-catenin and cyclin D1, mostly within regions of fibrosis implicating activation of the adenomatous polyposis coli (APC)-Wnt pathway. Genetic analysis showed a missense mutation in APC gene (c.7504G>A, p.G2502S in exon 15) and a functional homozygous polymorphism in the MUTYH gene (c.36+325G>C, (IVS1+5G/C)). Nuchal-type fibroma has been associated with Gardner's syndrome and trauma. In this patient, genetic predisposition coupled with repetitive, localized trauma and collagen degeneration may have provided the stimulus for the development of extra nuchal-type fibroma.

Summary Parkinson's disease (PD) is a common neurodegenerative disorder, occurring in at least 1% of patients older than 65 years of age and characterized by bradykinesia, rigidity, and tremor. Parkinsonism is a broad term for patients with PD and Parkinson's like disorders. Due to the aging population, the number of patients admitted to the intensive care unit (ICU) with PD or disorders with Parkinsonism is expected to rise in the future. For optimal ICU care, PD co-morbidities of memory impairment, aspiration, and dysautonomia require special attention. Withdrawal of dopaminergic medications in the perioperative state or ICU can lead to worsened PD symptoms, chest wall rigidity complicating ventilator management, and a severe hyperpyrexia, rigid, encephalopathic state termed neuroleptic malignant syndrome (NMS). PD patients are also sensitive to antipsychotic and antiemetic medications, which can exacerbate rigidity and aspiration. Elderly patients with PD are more sensitive to sedative medications and more susceptible to develop post-operative or ICU delirium, which has increased morbidity and mortality. PD patients are at increased risk of increased perioperative aspiration pneumonia and its associated morbidity and mortality. Specialized ICU management strategies for patients with PD are described.

Brain death commonly occurs after a devastating central nervous system (CNS) injury from head trauma or stroke. Severe brain injury and brain death create a variety of extra-cerebral organ manifestations including neuroendocrine hormone deficiencies, systemic inflammatory response syndrome (SIRS), neurogenic pulmonary oedema (NPE), myocardial stunning, as well as electrolyte and immunologic derangements. Spinal-mediated reflexes after brain death may be alarming to medical staff and families alike, and proper recognition and counselling to the family is necessary to prevent further emotional turmoil and doubt against the diagnosis of brain death. The challenge for the intensive care unit (ICU) physician is maintaining adequate organ perfusion and metabolism in brain-dead patients who eventually become organ donors. Due to the potential benefit from organ donation, maximal ICU management strategies should be employed to maintain viable organs until brain death is confirmed and the patient's wishes for organ donation can be expressed through family or documentation. Optimal ICU management of brain-dead patents who become organ donors increases the opportunity for organ transplantation and provides donation of organs previously not spared.

Summary Weakness is a common problem in patients requiring intensive care unit management. Critical illness polyneuromyopathy is a useful term to describe the acquired neuropathy and/or myopathy that develops in the setting of critical illness. There is a broad differential diagnosis and although most cases can be diagnosed clinically, a range of investigations may be required. The principal risk factors are systemic inflammatory response syndrome (particularly due to severe sepsis), corticosteroid and neuromuscular blocking drug administration and hyperglycaemia. Treatment remains largely supportive, emphasizing the importance of preventative strategies. This article will answer common questions pertaining to critical illness weakness.

To the Editor:Recently, Kazakov et al1 performed a clinicopathologic and genotypic analysis on a series of 16 patients suffering from multiple (familial) trichoepitheliomas (MFT: MIM 601606) and found mutations in the CYLD gene in 46%. Based on the frequent presence of CYLD mutations in MFT, it, alo

Pigment alterations of the vulva are quite common and due to either hypopigmentation or hyperpigmentation caused by a wide range of inflammatory, infectious, and neoplastic disorders. Because of this broad range of etiologies, accurate clinical diagnosis is difficult; therefore, clinicians must have a low threshold for biopsy when the clinical features are ambiguous. In this chapter, we will review the common causes of vulvar disorders of pigmentation

Eruptive disseminated porokeratosis (EDP) is a disease that presents clinically with sudden onset of erythematous papules and plaques, with a ridge-like border histologically represented by a cornoid lamella. We report a case of EDP occurring in a 39-year-old woman 3 days after completion of a 2-week course of oral corticosteroid therapy for an acute asthma exacerbation. The patient was treated with emollients and sun protection. Unlike the more chronic disseminated superficial (actinic) porokeratosis, EDP secondary to immunosuppression from corticosteroid therapy has very rarely been reported in the dermatological literature.

Typical myofibromas are biphasic tumors composed of a central zone of immature spindled to rounded cells arranged in a pericytic pattern and a peripheral zone of myoid nodules. Central necrosis is occasionally seen. A small but undefined subset of myofibromas displays atypical features that may lead to a misdiagnosis of sarcoma. To more completely characterize these tumors and define their behavior, we analyzed our experience with myofibromas having 1 or more atypical features including hypercellularity, absent or inconspicuous, poorly demarcated myoid nodules, infiltrative growth pattern, and perineural invasion. Of 266 cases of myofibromas, 24 cases were retrieved on the basis of pathology reports in which atypical features were mentioned. The tumors presented in 16 male and 8 female individuals (mean age 17 y; range, 2 wk to 62 y) as masses of variable size (mean 3.0 cm; range, 1.5 to 6.5 cm). Fourteen cases arose on the head and neck and 10 cases on the limbs. The referring or suspected diagnosis was sarcoma in 8 cases. The tumors were typically more cellular than ordinary myofibroma with levels of cellularity similar to that expected in fibrosarcoma (22/24). In addition, they displayed inconspicuous, loosely cohesive (22/24) or absent myoid nodules (2/24), infiltrating borders (19/24), intravascular growth (5/24), and perineural invasion/nerve entrapment (6/24). The mean mitotic rate was 5 mitoses/10 high-power fields, but no tumor showed significant cytologic atypia. The tumors were positive for actins (11/11) and CD34 (2/8). Follow-up in 14 patients revealed no distant metastases. We conclude that a small subset of myofibromas shows atypical features that complicate the diagnosis but do not adversely affect outcome.

Microvenular hemangioma (MVH) is a rare, slowly growing, benign vascular tumor that typically presents as a solitary enlarging plaque or nodule on the trunk or the extremities of young to middle-aged adults. A minority of MVH present with multiple lesions that are either gradually or suddenly acquired (eruptive MVH). Herein, we report a case of a 53-year-old woman who progressively developed numerous bilateral MVHs presenting as enlarging, blanching, erythematous to violaceous macules, patches, and plaques over the proximal thighs and axillae. Two biopsies exhibited the irregular branching venules with inconspicuous lumina lacking endothelial atypia and associated with dermal fibrosis characteristic of MVH. Immunophenotypically, the endothelium expressed Wilms Tumor 1, CD31, CD34, and erythrocyte-type glucose transporter protein (GLUT-1) GLUT-1 focally and was negative for Human herpes virus 8 and the lymphatic marker D2-40. In addition, numerous dermal spindle cells expressing CD34 and procollagen, putative fibrocytes, surrounded the thickened dermal collagen bundles and small vessels of MVH implicating a reactive/reparative (proliferative) process due to an unrecognized cutaneous injury. A review of MVH summarizing its clinicopathologic findings and its natural history is presented.

The Chapel Hill Consensus Conference criteria define cryoglobulinemic vasculitis (CV) as “vasculitis, with cryoglobulin immune deposits, affecting small vessels (i.e. capillaries, venules, or arterioles), and associated with cryoglobulins in the serum.” Hepatitis C virus (HCV) infection is the underlying etiologic factor in the majority of cases of CV, but it also occurs secondary to other infections (e.g., hepatitis B), connective tissue diseases (e.g., Sjogren’s syndrome), and hematolymphoid proliferations (e.g., Waldenstrom’s macroglobulinemia). Purpura, arthralgia, and weakness are the most common presenting signs and symptoms but any organ can be affected (skin, kidney, and nerves most frequently). The extent of involvement and disease severity progress with time in the absence of treatment. Recurrent palpable purpura associated with post-inflammatory hyperpigmenation is the most frequent cutaneous manifestation of CV but others include nodules, leg ulcers, livedo racemosa, and digital gangrene, i.e., signs of muscular-vessel vasculitis. Skin biopsy shows a small-vessel neutrophilic vasculitis equally affecting superficial and deep dermal and subcutis vessels, with neutrophilic muscular-vessel vasculitis in a minority of cases. Thrombosis, in some instances by hyaline/ type I cryoglobulin thrombi, contributes to the ischemic damage. By direct immunofluorescence, abundant IgM, and complement deposits are found in the affected vessels, highlighting the immune-complex mediated pathogenesis. Adverse prognostic factors for HCV-related CV include the presence of ulcers and renal insufficiency. Anti-HCV therapy (interferon-α and ribavirin) with or without rituximab is associated with a favorable clinical response, but relapse is common. In some patients, the persistent CV necessitates the administration of immunosuppressive agents.

Trichoblastic fibroma (TBF), also known as trichoblastoma, small nodular type, is a rare benign cutaneous adnexal tumor that shows trichogenic (follicular) differentiation that can be mistaken clinically and histologically for basal cell carcinoma (BCC), a locally aggressive trichogenic carcinoma. As the clinical behaviors of these two follicular neoplasms are opposite from one another and their management is thus different, recognition of TBF is crucial for effective therapy. Although TBF most commonly is first seen on the face, this lesion has not previously been reported in the eyelid. In this report, we document the first case of TBF affecting the ocular adnexa and detail the histologic features that differentiate TBF from BCC.

Routine light microscopy remains a powerful tool to diagnose, stage and prognose melanoma. Although it is very economical and efficient, it requires a significant level of expertise and, in difficult cases the final diagnosis is affected by subjective interpretation. Fortunately, new insights into the genomic aberrations characteristic of melanoma, coupled with ancillary studies, are further refining evaluation and management allowing for more confident diagnosis, more accurate staging and the selection of targeted therapy. In this article, we review the standard of care and new updates including four probe FISH, the 2009 American Joint Commission on Cancer staging of melanoma and mutant testing of melanoma, which will be crucial for targeted therapy of metastatic melanoma.