Coexistence of large cell transformed mycosis fungoides and diffuse large B-cell lymphoma in one patient.

Diffuse large B-cell lymphoma (DLBCL) is the most common and aggressive subtype of non-Hodgkin lymphoma. The overall risk of developing DLBCL is increased in patients with other lymphomas, such as mycosis fungoides (MF). In this report, we present an 81-year-old female with early-stage MF who simultaneously progressed to tumor stage, large-cell transformed (LCT) MF and developed a primary DLBCL in a lymph node (LN). She presented with a tumor on her leg and new lymphadenopathy in her right axilla. Skin biopsy of the tumor revealed infiltration of large atypical CD3+, CD4+, and CD30+ cells, and a smaller portion of CD8+ cells in the dermis, consistent with LCT MF. Biopsy of the axillary LN revealed diffuse sheets of CD20+, BCL-2+, c-MYC+, and CD10- cells, highly suggestive of double expressor DLBCL. High-throughput sequencing revealed monoclonal T cells in the skin tumor and a monoclonal B-cell population in the LN. The above findings led to simultaneous diagnoses of LCT MF and nodal double expressor DLBCL. Our case demonstrates the importance of performing a full pathological workup in cutaneous T-cell lymphoma patients presenting with lymphadenopathy.

Apocrine cystadenoma: A long-standing apocrine hidrocystoma with an adenomatous proliferation.

BACKGROUND: Apocrine cystadenoma is a rare, benign adenomatous cystic neoplasm, the pathogenesis of which is not fully understood. We sought to characterize the clinical, dermatoscopic, and histopathologic features of apocrine cystadenoma and its relationship to hidrocystoma. METHODS: We retrospectively analyzed cases of apocrine cystadenoma and hidrocystoma retrieved from the dermatopathology laboratory information system. RESULTS: Of the 350 cases apocrine cystic lesions, 13 cases of apocrine cystadenomas met the inclusion criteria. The age ranged from 20 to 84 years with an average of 64 years. They were long-standing (duration 3-15 years), slow-growing, large tumors usually found on the scalp. Dermatoscopy accentuated translucent light to dark blue color and prominent vessels that were present more at the periphery. All lesions were multilocular with columnar to cuboidal lining and decapitation secretion. A large portion of the lesion consisted of a simple nonproliferative epithelial lining, identical to that observed in apocrine hidrocystomas, while the proliferative adenomatous component made up a smaller portion with two patterns: (1) tubular proliferation, which either protruded into the cystic cavity or expanded outward peripherally, or (2) papillary projections, which were multiple layers thick with fibrovascular core, sometimes accompanied by tubular proliferation. Immunohistochemical stains showed strong staining for p40 and a sparse number of cells stained for Ki-67 and p53. CONCLUSIONS: The long duration of the lesion and the large areas of simple apocrine epithelial lining suggest that apocrine cystadenomas arise from long-standing apocrine hidrocystomas. However, the retrospective nature of the study from a single institution is a limitation.

Advances in melanoma: epidemiology, diagnosis, and prognosis.

Unraveling the multidimensional complexities of melanoma has required concerted efforts by dedicated community of researchers and clinicians battling against this deadly form of skin cancer. Remarkable advances have been made in the realm of epidemiology, classification, diagnosis, and therapy of melanoma. The treatment of advanced melanomas has entered the golden era as targeted personalized therapies have emerged that have significantly altered the mortality rate. A paradigm shift in the approach to melanoma classification, diagnosis, prognosis, and staging is underway, fueled by discoveries of genetic alterations in melanocytic neoplasms. A morphologic clinicopathologic classification of melanoma is expected to be replaced by a more precise molecular based one. As validated, convenient, and cost-effective molecular-based tests emerge, molecular diagnostics will play a greater role in the clinical and histologic diagnosis of melanoma. Artificial intelligence augmented clinical and histologic diagnosis of melanoma is expected to make the process more streamlined and efficient. A more accurate model of prognosis and staging of melanoma is emerging based on molecular understanding melanoma. This contribution summarizes the recent advances in melanoma epidemiology, classification, diagnosis, and prognosis.

Paraneoplastic Pemphigus/Paraneoplastic Autoimmune Multiorgan Syndrome: Part II. Diagnosis and Management.

In the second part of this Continuing Medical Education article on paraneoplastic pemphigus/paraneoplastic autoimmune multiorgan syndrome (PNP/PAMS), its diagnostic criteria, investigative work-up, and management are reviewed. PNP/PAMS is a rare autoimmune blistering disorder associated with high morbidity and mortality. Recognizing PNP/PAMS's key features and its diagnostic criteria is critical in initiating appropriate work-up. Evaluating PNP/PAMS requires knowledge of its findings on histopathology, direct immunofluorescence, indirect immunofluorescence, and enzyme-linked immunosorbent assay. Lastly, treatments for PNP/PAMS are reviewed with suggestions based on case reports and expert opinions in the literature. LEARNING OBJECTIVES: After completing this learning objective, the reader will be able to identify the criteria necessary for diagnosing paraneoplastic pemphigus (PNP/PAMS), learn how to work-up a diagnosis of PNP/PAMS, and understand important principles in the management of PNP/PAMS.

Paraneoplastic pemphigus/paraneoplastic autoimmune multiorgan syndrome: Part I. clinical overview and pathophysiology.

Paraneoplastic pemphigus/paraneoplastic autoimmune multiorgan syndrome (PNP/PAMS) is a highly fatal autoimmune blistering disease. The condition occurs in patients with underlying benign or malignant neoplasms, most commonly lymphoproliferative disorders. Both humoral and cell-mediated immunities contribute to the pathogenesis, and autoantibodies against plakin family proteins are characteristic. Patients typically present with severe stomatitis and polymorphous skin lesions, which are often resistant to treatment. Bronchiolitis obliterans (BO) is a frequent complication which contributes to the high mortality rate of PNP/PAMS. Given the rarity of this disorder and heterogeneity of clinical presentation, clinicians should maintain a high index of suspicion for PNP/PAMS to avoid delayed diagnosis. In this first part of a two-part continuing medical education (CME) series, risk factors, pathogenesis, and clinical features of PNP/PAMS are discussed.

Errors encountered in the diagnostic pathway: A prospective single-institution study.

BACKGROUND: Biopsy specimens go through a diagnostic pathway before a pathology report is rendered for the clinician. Errors can occur at any step in this pathway. METHODS: A 1-year prospective study was conducted at a single academic institution to identify and characterize errors that occurred in the diagnostic pathway from the clinic to the dermatopathology lab. RESULTS: A total of 25 662 specimens were processed and 190 errors were recorded (an error rate of 0.7%). The most common errors were an incorrect biopsy site (n = 65), incorrect data entry of a correct diagnosis (n = 25), and specimen mix-up (n = 23). There were 17 diagnostic errors. Errors most often occurred in the pre-analytical phase (n = 128). The clinician was responsible for 34.2% of errors, the dermatopathologist for 23.7%, and the histotechnician for 18.9%. Slips were the most common type of human error (n = 156). CONCLUSION: The most common error involved an incorrect biopsy site at the clinical stage. Over two-thirds of errors occurred before the slide reached the dermatopathologist. Diagnostic errors (analytical phase) rarely occurred, and when they did occur, the clinician was most likely to discover the error. Examining and addressing common laboratory errors help to reduce their incidence and lead to quality improvement in dermatopathology.

Exogenous Ochronosis With Ocular Involvement From Chronic Use of Teavigo.

Exogenous ochronosis refers to accumulation of homogentisic acid metabolites in tissues, manifesting as pigmentation of affected tissues. Phenolic compounds are most commonly implicated, including hydroquinone, quinine, phenol, resorcinol, mercury, and picric acid. The affected connective tissues exhibit brownish discoloration when heavily pigmented and the histopathological appearance is characteristic with "banana-shaped" ochre-colored pigment deposits. Herein, the authors describe a rare case of exogenous ochronosis involving the conjunctiva, sclera and skin, as a result of chronic use of Teavigo (94% epigallocatechin gallate), a polyphenol compound with postulated antioxidant and antiapoptotic activity.

Lichen Sclerosus of the Lip.

In this case report, we outline a case of a 36-year-old woman who presented to the dermatology clinic with a history of a hypopigmented macule on her lip. After conducting hepatitis C antibody testing and a shave biopsy, the patient was diagnosed with lichen sclerosus. Because of the increased risk for squamous cell carcinoma, she underwent an anogenital exam, where no lesions were found.

Granulomatous secondary syphilis: Another diagnostic pitfall for the dermatopathologist.

Syphilis is growing ever more prevalent in the United States with its incidence rising every year. Dermatopathologists need to maintain a high index of suspicion to avoid delayed diagnosis of this treatable disease. Accordingly, it is imperative to be aware of its myriad of presentations-including secondary syphilis with granulomatous inflammation. Most cases show aggregations of epithelioid histiocytes associated with plasma cells. Other patterns include an interstitial granuloma-annulare-like pattern, sarcoidal, and tuberculoid pattern. Immunohistochemical stains for Treponema pallidum may be negative, especially in late secondary or tertiary syphilis. We present a case of nodular secondary syphilis with granulomatous inflammation with negative T. pallidum staining.

An unusual presentation of pigmented purpuric lichenoid dermatitis.

Pigmented purpuric lichenoid dermatitis (PPLD) is a rare subtype of pigmented purpuric dermatosis, which classically presents with a mixture of lichenoid papules and patches on the bilateral lower extremities. Herein, we describe an unusual case of a 47-year-old man with PPLD who presented with 1-3mm discrete papules without the presence of larger patches. The diagnosis of PPLD should be considered for patients presenting with bilateral symmetric discrete papules on the legs.

An unexpected case of non-uremic calciphylaxis in a patient with multiple risk factors.

A 58-year-old woman with a history of morbid obesity, asthma, and prior warfarin use presented to the hospital with shortness of breath and a three-month history of painful, ulcerated ulcers with retiform purpura of her bilateral distal extremities. A punch biopsy specimen demonstrated focal necrosis and hyalinization of the adipose tissue with subtle arteriolar calcium deposition, findings consistent with calciphylaxis. We discuss the presentation of non-uremic calciphylaxis and review the risk factors, pathophysiology, and interdisciplinary management approach of this rare disease.

Diagnosis and treatment of low-risk superficial basal cell carcinoma in a single visit.

INTRODUCTION: Surgical excision remains the most commonly utilized treatment for superficial basal cell carcinoma (sBCC). In the era of cost containment of healthcare, the rising incidence of BCC and the high cost of excision require a continuous search for efficient and cost-effective management. OBJECTIVE: Examine the feasibility of the diagnosis and treatment of low-risk sBCC in a single visit. MATERIALS AND METHODS: Retrospective chart review of sBCCs diagnosed and treated in a single visit. RESULTS: The study identified 151 histologically confirmed sBCCs in 86 patients over a 5-year period, 93 (61.6%) cases of which were diagnosed as low-risk sBCC and treated in a single appointment. The majority of the cases (n = 86) were treated with curettage alone and the rest (n = 7) with a shave removal technique. The average size of the lesion was 0.82 cm located primarily on the trunk and extremities (95.7%). One recurrence on the trunk was observed in the single appointment group. Overall, diagnostic sensitivity was 95.4% and specificity was 92.0%. CONCLUSIONS: Diagnosis and treatment of sBCC in a single visit is an efficient and cost-effective management option for those who are proficient in identifying low-risk sBCC.

Sarcomatoid Dedifferentiated Melanoma: The Diagnostic Role of Next-Generation Sequencing.

ABSTRACT: Sarcomatoid dedifferentiated melanoma (SDDM) represents a diagnostic challenge as this cutaneous spindle cell melanoma lacks expression of classic melanocytic markers including S100, SOX10, Melan-A, HMB45, and MITF. The expression of the emerging melanoma marker preferentially expressed antigen in melanoma (PRAME) in SDDM is largely unknown. In this article, a case of SDDM arising in association with a nodular melanoma is highlighted. A 65-year-old man presented with a several week history of an ulcerated lesion on the right medial knee. A shave biopsy of the lesion revealed a biphasic neoplasm, which consisted of a centrally located poorly differentiated spindle cell component and an adjacent nodular component consisting of atypical melanocytes arranged in nests and fascicles. While the nodular component stained for S100, SOX10, and Melan-A, the spindle cell component failed to stain for these conventional melanocytic markers, only staining diffusely for CD10 and faintly for CD68. Both components stained for PRAME diffusely albeit less intensely within the spindle cell component. Next-generation DNA sequencing assay of the microdissected biphasic components revealed a shared mutation of NRAS. The results of the PRAME immunohistochemical stain and next-generation DNA sequencing assay facilitated in establishing the diagnosis of SDDM in association with nodular melanoma.

Skin diseases in Asian individuals that you do not want to miss: A selection of unique or relatively more common conditions in Asian populations.

The Asian population currently constitutes a simple majority of the global population, comprising nearly 60%. The percentage of the US population that identifies as Asian is expected to grow to 41 million by the year 2050, making up an eventual 9% of the US population. As the world and US populations of Asian individuals increase, the demand for dermatologic care from this population will increase, requiring dermatologists to become more familiar with the diagnosis and treatment of Asian-specific skin characteristics and diseases. In this contribution, we review skin conditions specific to or relatively more common in Asian patients to help recognition and management of diseases in an increasing Asian patient population. We discuss prurigo pigmentosa, primary cutaneous plasmacytosis, lipodystrophia centrifugalis abdominalis infantilis, Epstein-Barr viru-positive T- and natural killer-cell lymphoproliferative disorders, acquired bilateral nevus of Ota-like macules, and BehÒ«et disease.

Dermatoscopy.

The dermatoscope has gained tremendous popularity among dermatologists as an adjunctive tool to better visualize subsurface structures and identify patterns that may improve the diagnosis of a wide range of skin diseases. Initially, the pigmented lesion experts who were the early adopters promoted the use of the dermatoscope to increase diagnostic accuracy of early melanomas and decrease the harvesting of benign lesions. With current near universal adoption of the diagnostic technique by dermatologists, the dermatoscope is now employed to help identify a wide variety of inflammatory, infectious, and vascular conditions of the skin, hair, and nails, resulting in the emergence of several branches of dermatoscopy-inflammoscopy, trichoscopy, onychoscopy, and entodermoscopy. The future of dermatoscopy will involve incorporation of artificial intelligence that will make the assessment process increasingly objective, more accurate, and universally available. Despite the wide acceptance and adoption of dermatoscopy, the overall impact of its widespread use still remains unclear, whether it has decreased biopsy rates of benign lesions, reduced health care costs, or improved patient outcomes.

Artificial intelligence in dermatology for the clinician.

As medicine enters the era of artificial intelligence (AI)-augmented practice, dermatology is beginning to witness the integration of AI into the daily practice, particularly in the areas of diagnosis, prognosis, and treatment of skin diseases. Many of the current electronic medical records that dermatologists have incorporated provide guidance in billing, a form of AI at work. The recent advances in visual recognition AI make application and integration of the technology particularly suited for perceptual specialties such as radiology and dermatology. In dermatology, AI is poised to improve the efficiency and accuracy of traditional diagnostic approaches, including visual examination, skin biopsy, and histopathologic examination. This review highlights the current progress of AI in dermatology and provides a basic overview of the technology.