Idiopathic Calcinosis Cutis of the Hand.

Calcinosis cutis is a disease process characterized by calcified lesions in the skin. Although there are five subtypes of calcinosis, idiopathic calcinosis cutis is a rare disease process with no clear etiology. It has been described in many parts of the body; however, there are only five reported cases specifically involving the hands. We describe the presentation and successful treatment of a case of idiopathic calcinosis cutis in a 65-year-old man with lesions on his bilateral hands. We believe that surgical excision of symptomatic lesions is a safe and effective treatment for idiopathic calcinosis cutis of the hands.

Cutaneous hydrophilic polymer emboli following endovascular repair of an abdominal aortic aneurysm: a case and review of literature.

Hydrophilic polymer embolism (HPE) is a rare iatrogenic complication of the use of polymer-coated intravascular devices, which may affect several organ systems including the skin. Herein, we present a patient who developed a cutaneous eruption with associated neurologic manifestations secondary to localized HPE. This is a potentially underdiagnosed, life-threatening complication and physicians should consider HPE when evaluating skin eruptions in patients who have undergone endovascular procedures.

Complete response of Merkel cell carcinoma with talimogene laherparepvec (TVEC) monotherapy.

Merkel cell carcinoma (MCC) is a rare neuroendocrine neoplasm, warranting surgical excision with sentinel lymph node biopsy. In later stages, adjuvant chemotherapy and radiation are required owing to its aggressive malignant behavior. We describe a 62-year-old woman who presented with multifocal recurrence of MCC and was not a candidate for immunotherapy or surgery. The patient underwent four treatments of intratumoral talimogene laherparepvec (TVEC) and demonstrated a complete response with no histologic evidence of remaining MCC on four scouting biopsies. Although TVEC therapy is currently approved for the treatment of advanced stage melanoma, it is still being investigated in MCC. This case supports the use of TVEC as monotherapy in select patients with locally advanced MCC who are not candidates for surgery or systemic immunotherapy.

Surgical Margin Mapping of Melanoma In Situ Using In Vivo Reflectance Confocal Microscopy Mosaics.

BACKGROUND: Melanoma in situ (MIS) can have poorly defined borders and subclinical extension that makes margin control challenging. Reflectance confocal microscopy (RCM) is a promising noninvasive technique that can be used to assess subclinical spread. OBJECTIVE: To optimize surgical margins of histology-proven MIS using RCM mosaics. MATERIALS AND METHODS: Prospective review of 22 patients with histology-proven MIS who underwent RCM margin mapping prior to staged excision, between August 1, 2018, and August 13, 2020, at the Department of Dermatology, University of New Mexico, School of Medicine. RESULTS: Twenty patients (91%) had tumor clearance on the first stage using a 3-mm surgical margin after confocal margin mapping. CONCLUSION: Reflectance confocal microscopy margin mapping using the mosaic device tends to clear MIS in one stage, and the use of the handheld device may improve the accuracy for difficult anatomic areas. Current Procedural Terminology codes for RCM do not reflect the time required and complexity of the procedure. Reflectance confocal microscopy margin mapping prior to excision has the potential to decrease the number of stages needed for melanoma removal, reduce treatment time, and cost.

Dermatologists are more likely than oncologists to prescribe skin-directed therapies for early-stage cutaneous T-cell lymphoma: a retrospective review.

Early-stage cutaneous T-cell lymphoma (CTCL) is managed effectively with skin-directed therapies such as topical medications, phototherapy, and local ionizing radiation. Patients with CTCL often seek care from both dermatologists and oncologists. Our study aimed to compare the frequency that skin-directed treatments were prescribed to patients managed by each of these specialties. Overall, we found there was a statistically detectable relationship between the presence or absence of oncologist involvement and the likelihood that a patient would be prescribed skin-directed therapies (P=0.0003). Of the oncologists included in the study, 66% opted for management revolving around systemic rather than skin-directed therapies. However, when a dermatologist and oncologist worked together in a patient's care, the number of patients receiving skin-directed therapies increased to 100%. Our study suggests that patients with early stage CTCL may benefit from having a dermatologist involved in their care.

Digital Whole Slide Imaging Compared With Light Microscopy for Primary Diagnosis in Surgical Pathology.

CONTEXT.­: The adoption of digital capture of pathology slides as whole slide images (WSI) for educational and research applications has proven utility. OBJECTIVE.­: To compare pathologists' primary diagnoses derived from WSI versus the standard microscope. Because WSIs differ in format and method of observation compared with the current standard glass slide microscopy, this study is critical to potential clinical adoption of digital pathology. DESIGN.­: The study enrolled a total of 2045 cases enriched for more difficult diagnostic categories and represented as 5849 slides were curated and provided for diagnosis by a team of 19 reading pathologists separately as WSI or as glass slides viewed by light microscope. Cases were reviewed by each pathologist in both modalities in randomized order with a minimum 31-day washout between modality reads for each case. Each diagnosis was compared with the original clinical reference diagnosis by an independent central adjudication review. RESULTS.­: The overall major discrepancy rates were 3.64% for WSI review and 3.20% for manual slide review diagnosis methods, a difference of 0.44% (95% CI, -0.15 to 1.03). The time to review a case averaged 5.20 minutes for WSI and 4.95 minutes for glass slides. There was no specific subset of diagnostic category that showed higher rates of modality-specific discrepancy, though some categories showed greater discrepancy than others in both modalities. CONCLUSIONS.­: WSIs are noninferior to traditional glass slides for primary diagnosis in anatomic pathology.

Kaposi Sarcoma as a Cutaneous Vasculitis Mimic: Diagnosis and Treatment.

Painful, palpable purpura usually indicate underlying vasculitis. We report a case of systemic vasculitis treated with immunosuppression that developed painful, vasculitis-like purpuric lesions that progressed rapidly to fulminant Kaposi sarcoma (KS). These purpuric, tumorous lesions resolved completely following the suspension of immunosuppression; however, without immunosuppression, the underlying autoimmunity recurred. This case highlights the potential for early KS to present as a vasculitis mimic or pseudovasculitis that clinicians should keep in mind when purpuric, vasculitis-like lesions develop in an immunosuppressed patient with vasculitis. It is important to recognize these pseudovasculitis lesions as KS rather than recurrent vasculitis so that immunosuppression can be withdrawn.

H syndrome: 5 new cases from the United States with novel features and responses to therapy.

BACKGROUND: H Syndrome is an autosomal recessive disorder characterized by cutaneous hyperpigmentation, hypertrichosis, and induration with numerous systemic manifestations. The syndrome is caused by mutations in SLC29A3, a gene located on chromosome 10q23, which encodes the human equilibrative transporter 3 (hENT3). Less than 100 patients with H syndrome have been described in the literature, with the majority being of Arab descent, and only a few from North America. CASE PRESENTATION: Here we report five pediatric patients from three medical centers in the United States who were identified to have H syndrome by whole exome sequencing. These five patients, all of whom presented to pediatric rheumatologists prior to diagnosis, include two of Northern European descent, bringing the total number of Caucasian patients described to three. The patients share many of the characteristics previously reported with H syndrome, including hyperpigmentation, hypertrichosis, short stature, insulin-dependent diabetes, arthritis and systemic inflammation, as well as some novel features, including selective IgG subclass deficiency and autoimmune hepatitis. They share genetic mutations previously described in patients of the same ethnic background, as well as a novel mutation. In two patients, treatment with prednisone improved inflammation, however both patients flared once prednisone was tapered. In one of these patients, treatment with tocilizumab alone resulted in marked improvement in systemic inflammation and growth. The other had partial response to prednisone, azathioprine, and TNF inhibition; thus, his anti-TNF biologic was recently switched to tocilizumab due to persistent polyarthritis. Another patient improved on Methotrexate, with further improvement after the addition of tocilizumab. CONCLUSION: H syndrome is a rare autoinflammatory syndrome with pleiotropic manifestations that affect multiple organ systems and is often mistaken for other conditions. Rheumatologists should be aware of this syndrome and its association with arthritis. It should be considered in patients with short stature and systemic inflammation, particularly with cutaneous findings. Some patients respond to treatment with biologics alone or in combination with other immune suppressants; in particular, treatment of systemic inflammation with IL-6 blockade appears to be promising. Overall, better identification and understanding of the pathophysiology may help devise earlier diagnosis and better treatment strategies.

"Blueberry muffin" rash and large right thigh mass: a unique presentation of Langerhans cell histiocytosis.

Langerhans cell histiocytosis (LCH) is a clonal proliferation of bone marrow derived antigen-presenting cells that can involve a spectrum of cutaneous findings, with or without internal organ involvement. Neonatal LCH almost always presents with skin findings, usually petechial papules and/or erosions in a seborrheic distribution, with or without extracutaneous involvement. Previously described as varying entities, LCH is now considered a single disease process demonstrating a spectrum of clinical findings. We report a unique case of neonatal LCH presenting with a "blueberry muffin" rash in conjunction with a large soft tissue tumor.

Mitotic rate in cutaneous melanomas ≤1 mm in thickness: a prospective study.

The mitotic rate (MR) of malignant melanoma (MM) refers to the number of mitoses per square millimeter. Studies have suggested that it is an independent prognostic variable predicting survival in patients with MM, and it was recently included in the American Joint Committee on Cancer (AJCC) recommendations for diagnosis and treatment of MM. The AJCC melanoma staging committee recommends using the "hot-spot" approach to determine the MR, whereby it is reported as the maximum dermal mitotic figures identified in a 1-mm area of the melanoma. The AJCC has recommended that the MR be determined in all melanomas, irrespective of Breslow depth or other features. We aimed to quantify the MR in MM ≤1 mm in thickness and to identify statistical associations between the MR, Breslow depth, and Clark level. In addition, we hoped to identify practical issues in determining the MR via the hot-spot technique. We conducted a prospective study to determine the MR, Breslow depth, and Clark level in MM ≤1 mm in thickness. Seven melanomas were identified with epidermal mitoses only (7.4%). Sixteen melanomas had dermal mitoses (16.8%); of these, the majority (75.0%) contained only one mitotic figure. Seventy-nine melanomas had no dermal mitoses (83.2%). Seven lesions (7.4%) demonstrated multiple mitoses; 4 with ≥2 dermal mitoses/mm and 3 with multiple epidermal mitoses. We conclude that thin MM with >1 mitosis/mm is rare and discuss practical and theoretical issues with determining the MR using the hot-spot approach.