Dermatopathologist accuracy in classifying angioinvasive fungal infections using virtual microscopy.

BACKGROUND: Due to perceived difficulty in the categorization of angioinvasive fungal infections based on histopathology, variation exists in dermatopathology reporting. METHODS: This study characterized the diagnosis of angioinvasive fungal infections by light microscopy at a single academic institution over an 11-year period. Subsequently, the accuracy of blinded reclassification by virtual microscopy was measured. RESULTS: Seventy-six specimens with hematoxylin-eosin slides were obtained from 33 patients. The mean diagnostic accuracy of dermatopathologists in differentiating mucormycosis, hyalohyphomycosis, and phaeohyphomycosis based on blinded reclassification via virtual microscopy was 74%, with a range of 65%-91%. CONCLUSIONS: While there was a range in diagnostic accuracy, the highest score of 91% and the identification of common sources of error suggest that histopathologic categorization of angioinvasive fungal infections can frequently be performed. However, accurate identification is not always possible given common pitfalls in diagnosis. In addition, standardized and clinically useful reporting should be considered.

Non-uremic Calciphylaxis With Possible Initial Misdiagnosis as Erythema Multiforme/Toxic Epidermal Necrolysis Successfully Treated as Inpatient.

Non-uremic calciphylaxis (NUC) is a rare, high-mortality disease, and it can be easily misdiagnosed as other ulcerative dermatologic conditions. A female in her late 30s with a medical history of alcoholic liver cirrhosis and obesity who previously underwent gastric bypass surgery presented with an 11-month history of worsening bilateral lower extremity wounds following the initiation of spironolactone. A wound biopsy at the time of initial presentation favored erythema multiforme/toxic epidermal necrolysis (EM/TEN). She initially responded to systemic steroids, but her wounds later worsened, prompting her to seek representation a few months later. The initial suspicion was for a superimposed bacterial infection; however, her wounds did not improve following antibiotics. A repeat skin biopsy revealed calciphylaxis, for which she had multiple risk factors, including severe vitamin D deficiency causing secondary hyperparathyroidism. A multidisciplinary approach was successful in achieving a satisfactory response with pain control, wound care, skin grafting, and mitigation of risk factors in addition to the use of sodium thiosulfate. Upon our review, the initial biopsy did not demonstrate features of EM/TEN but did demonstrate features suspicious for calciphylaxis. The exposure to systemic corticosteroids due to the presumed diagnosis of EM/TEN may have worsened her condition since this is a known risk factor for calciphylaxis. Our case highlights the importance of clinicopathologic correlation as well as the place for calciphylaxis in the clinical and histopathologic differential diagnosis of ulcerated, necrotic lesions on the lower extremities in the absence of renal disease.

Cutaneous Necrosis of the Skin Secondary to Methicillin-Resistant Staphylococcus aureus (MRSA) and Bacteroides With Superimposed Herpes Simplex Virus Type II (HSV-II) in the Setting of Infective Endocarditis.

Herpes simplex virus type II (HSV-II) with superimposed bacterial skin infection is an uncommon presentation of cutaneous necrosis in the setting of infective endocarditis. This case reflects a unique presentation of an immunosuppressed patient with infective endocarditis complicated by septic emboli and cutaneous skin lesions attributable to HSV-II and superimposed bacterial skin infection. The patient presented from an outside hospital with symptoms consistent with acute onset heart failure and skin lesions. Transthoracic and transesophageal echocardiography performed there demonstrated focal thickening of the anterior mitral valve leaflet with severe mitral regurgitation. The patient then underwent extensive infectious work-up and was put on broad-spectrum antibiotics. Further work-up demonstrated greater than three DUKE minor criteria and reiterated the focal thickening of the anterior leaflet of the mitral valve, making infective endocarditis the most likely etiology. Biopsies of the skin lesions were performed which stained positive for HSV-II and grew methicillin-resistant Staphylococcus aureus and Bacteroides fragilis. The cardiothoracic surgery service ultimately decided not to perform any surgical intervention to the mitral valve during her hospitalization as she was deemed to be too high of a risk due to her thrombocytopenia and significant comorbidities. She was later discharged in hemodynamically stable condition on long-term intravenous antibiotics with repeat echocardiography demonstrating significant reduction in the mitral regurgitation and the focal thickening of the anterior leaflet of the mitral valve.

Impact of alternative histopathology protocols in the dermatopathology laboratory: A prospective quality improvement study.

BACKGROUND: Histopathology protocols for processing dermatopathology specimens vary among laboratories. OBJECTIVE: To determine an optimal histopathology protocol to minimize cost and turnaround time (TAT) for biopsy specimens in a dermatopathology laboratory. METHODS: A prospective, 4-month study compared the mean cost and TAT of producing one versus two initial H&E slides, and zero versus three unstained slides that could be used for frequently used special or immunohistochemical (IHC) stains. RESULTS: For all cases, cost was lower for one versus two initial H&E slides, with an insignificant increase in TAT. Producing three vs zero unstained slides incurred higher cost, with no reduction in TAT. In a subset of cases in which frequently used special or IHC stains were performed, cost and TAT were optimized by producing one initial H&E and three unstained slides. CONCLUSION: A protocol of one initial H&E slide and zero unstained slides optimizes cost and TAT in our dermatopathology laboratory. Pigmented lesions and inflammatory dermatoses may benefit from the addition of unstained slides. Further study is needed to quantify this benefit and evaluate for other cases for which an alternative protocol is advantageous.

Stevens-Johnson Syndrome (SJS) As the Initial Presentation in a Patient With a New Diagnosis of HIV.

A woman in her 40s presented to the emergency department with a diffuse rash consistent with Stevens-Johnson syndrome (SJS). There was no identifiable inciting factor. However, she was newly diagnosed with human immunodeficiency virus (HIV) during that same hospital admission. The leading theory for why she developed SJS given her lack of classic precipitating factors is an immune dysregulation as a result of HIV. Most cases of SJS/toxic epidermal necrolysis (TEN) in patients with HIV are related to highly active antiretroviral therapy and prophylaxis with trimethoprim-sulfamethoxazole. There is a lack of literature regarding SJS as the initial presentation of HIV without known underlying etiology or inciting factors.

Lichenoid granulomatous dermatitis revisited: A retrospective case series.

BACKGROUND: Lichenoid granulomatous dermatitis (LGD) is an uncommon reaction pattern for which clinical correlates can be difficult to establish. LGD combines vacuolar degeneration with variable types of granulomas. OBJECTIVE: To determine clinical correlates of LGD. METHODS: The laboratory information systems at the University of Florida, the Medical College of Wisconsin, and Inform Diagnostics Research Institute were queried to identify 56 cases of LGD. Cases were reviewed for information regarding eosinophils, plasma cells, deep perivascular infiltrates, granuloma subtype, parakeratosis, epidermal atrophy, psoriasiform epidermal changes, pseudoepitheliomatous hyperplasia, periadnexal inflammation, vasculitis, and red blood cell extravasation. RESULTS: The most common clinical correlates were drug eruption (39.3%, n = 22) and lichenoid keratosis (19.6%, n = 11). Tattoo reaction, postherpetic dermatitis, and scabies or postscabietic dermatitis each accounted for 7.1% (n = 4) of cases. Pigmented purpuric dermatosis and lichen striatus each accounted for 5.4% (n = 3) of cases. Dermal eosinophils (P = .005) and psoriasiform epidermal changes (P = .055) were associated with drug hypersensitivity. Perineural (P = .049) and perifollicular (P = .003) inflammation were associated with tattoo reaction and postherpetic dermatitis. Red blood cell extravasation was helpful in cases of pigmented purpuric dermatosis (P = .049). LIMITATIONS: This study is limited by its retrospective nature and statistical power. CONCLUSION: Dermal eosinophilia, psoriasiform epidermal changes, periadnexal inflammation, and red blood cell extravasation might aid in the clinical diagnosis of patients with LGD.

Adult Onset Still's Disease Associated with Mycoplasma pneumoniae Infection and Hemophagocytic Lymphohistiocytosis.

Adult Onset Still's Disease (AOSD) is a systemic inflammatory disorder that can be associated with hemophagocytic lymphohistiocytosis (HLH), a rare but potentially fatal disease of overactive histiocytes and lymphocytes. We present a unique case of AOSD complicated by Mycoplasma pneumonia infection and HLH. A 28-year-old female developed joint pains followed by a diffuse, erythematous, pruritic skin rash that quickly spread throughout the body. The patient deteriorated and developed fever, chills, cough, and dyspnea and had to be intubated. She had hypoalbuminemia, elevated liver enzymes, a very high serum ferritin level, positive anti-Mycoplasma pneumonia IgG and IgM antibodies, and normal rheumatoid factor and anti-nuclear antibodies. The chest X-ray showed diffuse bilateral infiltrates. Bone marrow biopsy revealed hemophagocytosis. The patient was treated with azithromycin, methylprednisolone, and anakinra and was discharged home on cyclosporine and prednisone. This case highlights that patients can develop features of both AOSD and HLH at the beginning of the disease and early diagnosis and treatment increase the likelihood of recovery.

Perceptions of pathology informatics by non-informaticist pathologists and trainees.

BACKGROUND: Although pathology informatics (PI) is essential to modern pathology practice, the field is often poorly understood. Pathologists who have received little to no exposure to informatics, either in training or in practice, may not recognize the roles that informatics serves in pathology. The purpose of this study was to characterize perceptions of PI by noninformatics-oriented pathologists and to do so at two large centers with differing informatics environments. METHODS: Pathology trainees and staff at Cleveland Clinic (CC) and Massachusetts General Hospital (MGH) were surveyed. At MGH, pathology department leadership has promoted a pervasive informatics presence through practice, training, and research. At CC, PI efforts focus on production systems that serve a multi-site integrated health system and a reference laboratory, and on the development of applications oriented to department operations. The survey assessed perceived definition of PI, interest in PI, and perceived utility of PI. RESULTS: The survey was completed by 107 noninformatics-oriented pathologists and trainees. A majority viewed informatics positively. Except among MGH trainees, confusion of PI with information technology (IT) and help desk services was prominent, even in those who indicated they understood informatics. Attendings and trainees indicated desire to learn more about PI. While most acknowledged that having some level of PI knowledge would be professionally useful and advantageous, only a minority plan to utilize it. CONCLUSIONS: Informatics is viewed positively by the majority of noninformatics pathologists at two large centers with differing informatics orientations. Differences in departmental informatics culture can be attributed to the varying perceptions of PI by different individuals. Incorrect perceptions exist, such as conflating PI with IT and help desk services, even among those who claim to understand PI. Further efforts by the PI community could address such misperceptions, which could help enable a better understanding of what PI is and is not, and potentially lead to increased acceptance by non-informaticist pathologists.

Polymorphous sweat gland carcinoma: a report of two cases.

Polymorphous sweat gland carcinoma (PSGC) is a rare adnexal neoplasm with characteristic variegated histopathologic findings and low-grade clinical behavior. First described in 1994, only 11 cases have been reported in the literature. It is named for the multiplicity of architectural patterns that may be present: solid, tubular, trabecular, pseudopapillary and cylindromatous. Owing to the multiple architectural patterns, the differential diagnosis is broad, including metastatic adenocarcinoma and other adnexal neoplasms with ductular differentiation. We present two new cases of PSGC and review the literature on this rare tumor.

Colocalization of vitiligo and alopecia areata presenting as poliosis.

Vitiligo and alopecia areata are two cutaneous diseases believed to be primarily autoimmune in pathogenesis. While the coexistence of the two conditions in the same patient has been well-described, reports of the two disease processes occurring in the same location are rare. We report the case of a 10-year-old male with an unremarkable past medical history who presented with a single localized area of poliosis with depigmented underlying skin on the frontal scalp. The hair in the affected area was relatively decreased in density. A punch biopsy of the depigmented patch demonstrated features consistent with both vitiligo and alopecia areata. The decreased number of large hair follicles and a focal peribulbar lymphocytic infiltrate around an anagen follicle were suggestive of alopecia areata. A panel of melanocyte-specific stains revealed absent melanocytes in the epidermis, consistent with vitiligo. Loss of microphthalmia-associated transcription factor-positive root sheath cells was seen, suggestive of loss of melanocyte stem cells. The combination of clinical and histopathologic findings supports the theory of a common pathogenesis of alopecia areata and vitiligo.