Arteriovenous malformation with hair collar sign.

We report the case of a newborn who was noted at birth to have an occipital scalp nodule presenting with a hair collar sign (HCS). The nodule had enlarged since birth. An MRI revealed a soft tissue mass on the occipital scalp without deep extension or cranial bone involvement. A biopsy of the nodule led to a diagnosis of arteriovenous malformation (AVM). A vascular malformation with HCS has not been reported before in North America. This case highlights the complexity of diagnosing a lesion with a hair collar sign.

Nonosseous Periocular Manifestations of Langerhans Cell Histiocytosis: A Case Report and Systematic Review.

PURPOSE: Langerhans cell histiocytosis (LCH) is a disorder of dendritic cell proliferation that typically involves bone. It can be diagnostically challenging when LCH presents without bony involvement, leading to delays in diagnosis and treatment. In this study, the periocular manifestations of LCH in cases where the underlying orbital bones are not involved are described through a systematic review. METHODS: A systematic review of the literature was performed to capture all cases of LCH that involved the periocular region but not the underlying orbital bones. These included LCH cases that involved the periocular skin, the ocular surface, and the orbital tissue. The authors also highlight an additional case where LCH presented with periocular edema and multifocal, nodular conjunctival lesions. RESULT: This review illustrates that LCH rarely presents with periocular infiltration without orbital bone involvement. In these atypical cases, LCH can present as an eyelid mass, a chalazion-like lesion, generalized periocular swelling, ocular surface lesions, or infiltration of any orbital structure. Ocular surface LCH has a higher rate of recurrence than other periocular LCH. Orbital LCH can involve any tissue including extraocular muscles, the lacrimal gland, or indistinct areas within the orbit. CONCLUSIONS: LCH is a clinicopathologic diagnosis. Although most cases involve the bone, any soft tissue can be involved. Biopsy is required to confirm the diagnosis of this heterogeneous disease.

Histopathological features of iatrogenic occlusive vasculopathy caused by intra-articular hyaluronic acid injection for osteoarthritis.

Occlusive vasculopathy may rarely occur after intra-articular injection with hyaluronic acid. The associated histological changes are not well described. Herein, we would like to present representative histology of this phenomenon.

Calciphylaxis: how specific are the pathological features: avoiding false-positives and false-negatives.

Calciphylaxis is considered a critical inflammatory dermatosis with potentially devastating clinical consequences. Skin biopsies are expedited for evaluation and are often considered as a gold standard for diagnostic confirmation and exclusion of other conditions. The key histopathological features include a combination of vascular and extra-vascular calcifications, intravascular microthrombi, and changes related to resulting ischemia. The pathological diagnosis of calciphylaxis is not always a straightforward process as it can be influenced by a number of factors. The specificity of pathological diagnosis of calciphylaxis has been questioned and a systematic approach with multidisciplinary collaboration is required to avoid potential errors.

Diagnostic and treatment challenges of a case of primary cutaneous signet-ring cell/histiocytoid carcinoma of the eyelid.

BACKGROUND: Primary cutaneous signet-ring cell/histiocytoid carcinoma of the eyelid is an extremely rare but aggressive neoplasm diagnosed primarily in elderly men. Until now there are 32 published cases of signet-ring cell carcinoma or histiocytoid carcinoma of the eyelid. We report the clinical, radiographic and histological features of the 33rd reported case of PCSRCC in the eyelid of a 73-year-old male, and review diagnostic and treatment challenges of this rare entity. CASE PRESENTATION: Our case highlights a 73-year-old male who was referred for surgical correction of right eye ptosis that was present for 2 years. Upon assessment, he was noted to have an upper lateral orbital rim mass. Computed tomography (CT) noted ill-defined soft tissue thickening anterior to the right globe, predominantly pre-septal but with slight post-septal extension. The pathology revealed diffusely and deeply infiltrating tumour cells extending through the dermis, subcutis, orbicularis muscle bundles and nerve fibers; the tumour cells were noted to have a monotonous histiocytoid appearance with foamy granular eosinophilic cytoplasm. At high magnification, intracytoplasmic vacuoles and occasional intermixed signet ring cells were identified. Immunohistochemical staining revealed the tumour cells to be AE1/AE3, CK7, GCDFP-15, E-cadherin, androgen receptor stain and GATA3 positive. Final pathology report confirmed the diagnosis of primary cutaneous signet-ring cell/histiocytoid carcinoma. Further imaging failed to identify a distant primary malignancy or metastatic disease. The decision was made to attempt surgical excision of the tumor. After the bulk of the grossly apparent tumor was removed, intraoperative frozen sections were sent. Superficial biopsies of the right periorbital region were performed, which revealed extension significantly further than the gross disease. Thereafter, the patient underwent a wide orbital exenteration with reconstruction using a temporary split-thickness skin graft. Due to positive margins on final permanent sections, the patient underwent further wide resection with free muscle-skin flap reconstruction followed by adjuvant radiation treatment. CONCLUSION: Our case represents the 33rd case of primary signet-ring cell/histiocytoid carcinoma of the eyelid in a 73-year-old male, the first documented case with GATA3 positivity and the second documented case with androgen receptor stain positivity.

Staged margin-controlled excision (SMEX) for lentigo maligna melanoma in situ.

BACKGROUND: No consensus exists regarding the best surgical strategy to achieve clear surgical margins while minimizing tissue excision when definitely excising lentigo maligna melanoma in situ (LM). The staged margin controlled excision (SMEX) technique is a modification of the spaghetti technique that allows surgeons to minimize margins and ensure complete excision of LM. OBJECTIVES: Our objectives were twofold: a) to evaluate the effectiveness of SMEX for treatment of LM and b) detail the SMEX technique. METHODS: A retrospective chart review of adult patients who underwent the SMEX technique for treatment of LM from 2011 to 2016 was conducted. RESULTS: Twenty-four patients were identified with predominantly facial lesions. The mean defect size was 12.1 cm2 . A mean number of two SMEX procedures, with an average margin of 9 mm, were required to obtain complete excision of the LM. Using SMEX, we achieved 100% clearance of LM over a median follow up period of 18 months, with a range of 1-63 months. CONCLUSIONS: SMEX offers a reliable surgical excision method that ensures complete excision of LM in a cosmetically sensitive manner. The recurrence outcomes of SMEX are comparable, if not better, than those of alternative excision techniques in the literature.

Clinical and Dermoscopic Features of Pigmented Disseminated Superficial Actinic Porokeratosis: Case Report and Literature Review.

INTRODUCTION: Porokeratosis is a benign hyperkeratotic skin tumour due to a clonal proliferation of keratinocytes and is characterised by a telltale annular threadlike configuration along the border of a skin-colored to erythematous papule that can expand centrifugally. CASE PRESENTATION: We are presenting a clinical and dermoscopic case of pigmented disseminated superficial actinic porokeratosis (DSAP) limited to the upper trunk of a white man with sun-damaged skin. Literature Review and Conclusion: A thorough review of PubMed failed to identify any previous reports on the dermoscopic appearance of pigmented porokeratosis. On dermoscopy, the presence of black dots limited to the periphery of the lesions is due to pigment incontinence and melanophages within the superficial papillary dermis limited to the area below the cornoid lamella. Pigmented DSAP is a unique morphological presentation of porokeratosis, and it is essential to be familiar with its clinical and dermoscopic presentation.

Spontaneous Regression of a Congenital Melanocytic Nevus by Sclerosis.

Congenital melanocytic nevi (CMNs) naturally evolve throughout life, growing proportionately with the child, darkening, and exhibiting textural or surface changes (e.g., papillomatous, verrucous, cerebriform), hypertrichosis, and, later in life, lightening of pigmentation. We report the case of a 5-year-old child with complete resolution of a medium-sized CMN involving the distal left leg and foot via sclerosis and in the absence of any halo phenomenon. Spontaneous regression of CMN via sclerosis is rare, and it is thought that an immunologic mechanism different from the mechanism that the halo phenomenon induces mediates this regression. We reviewed the literature on this phenomenon and discuss how it might lead to regression of the nevus.

Sweet syndrome with panniculitis, arthralgia, episcleritis, and neurologic involvement precipitated by antibiotics.

BACKGROUND: Sweet syndrome is an uncommon skin condition, often idiopathic in origin although it may be reactive to various systemic conditions, recent infections, underlying malignancies, and medications. OBJECTIVE & METHOD: To present a case highlighting a rare clinical presentation and to review the causes of Sweet syndrome with an emphasis on drug-induced etiologies. RESULTS: We describe a 45-year-old woman who developed Sweet syndrome while receiving nitrofurantoin and ciprofloxacin for a urinary tract infection. Her course of disease was complicated by arthralgias, episcleritis, headaches, and erythema nodosum-like subcutaneous involvement. There was marked improvement with discontinuation of the inciting antibiotics and initiation of systemic steroids. CONCLUSION: This case illustrates Sweet syndrome related to nitrofurantoin and/or ciprofloxacin. This is the second report of Sweet syndrome related to these antibiotics and the first associated with ocular, joint, and neurologic involvement.

Histopathologic Evaluation of Atypical Fibroxanthoma or Pleomorphic Dermal Sarcoma Debulk Specimen from Mohs Surgery: A Requirement for Their Proper Distinction.

Pleomorphic dermal sarcomas can be clinically aggressive, with a higher tendency to cause local recurrence, metastasis, and death. Atypical fibroxanthoma and pleomorphic dermal sarcoma are histopathologically similar, and their distinction requires a systematic examination of the entire excised tumor. Since Mohs micrographic surgery is commonly utilized to treat atypical fibroxanthoma, a histopathologic evaluation of debulk specimens by permanent pathology is prudent to avoid underdiagnosing pleomorphic dermal sarcoma. This approach can improve risk assessment and treatment decisions, ultimately enhancing patient outcomes. Also, the proper distinction will facilitate the future development of accurate staging criteria and additional treatment modalities.

Direct Immunofluorescence of Skin and Oral Mucosa: Guidelines for Selecting the Optimum Biopsy Site.

Direct immunofluorescence is a vital diagnostic test for assessing vesiculobullous disorders, vasculitides, and connective tissue diseases. It is a robust and valuable technique that offers essential diagnostic information for many critical dermatoses. Dermatopathologists depend heavily on the data obtained from direct immunofluorescence evaluation to confirm final diagnoses. Selecting the most appropriate biopsy site is necessary for maximizing diagnostic accuracy, and the best site may vary depending on the clinical differential diagnosis. Inaccurate biopsy site selection can significantly impact the accuracy of the results. To optimize the use of direct immunofluorescence studies, this review provides helpful guidelines and some practical tips for selecting the best biopsy site.

A case of disseminated angiosarcoma-like Kaposi sarcoma presenting with unusual vesiculobullous lesions.

Angiosarcoma-like Kaposi sarcoma represents a recently delineated rare histomorphologic variant of Kaposi sarcoma that can be difficult to distinguish from other vasoproliferative lesions. Conventional lesions of Kaposi sarcoma encompass patches, plaques, and nodules; however, rarely vesiculobullous lesions have been described. Angiosarcoma-like Kaposi sarcoma has never been previously reported to present with vesiculobullous lesions. Herein, we describe a unique case of disseminated angiosarcoma-like Kaposi sarcoma with vesiculobullous lesions as the initial manifestation of human immunodeficiency virus infection.

Cutaneous Syncytial Myoepithelioma: A Unique Variant Worth Recognizing.

Cutaneous syncytial myoepithelioma is a recently characterized variant of cutaneous myoepithelioma with a distinct histopathological and immunohistochemical profile. It is more common in men and predominately involves upper and lower extremities. Microscopically, it is a dermal tumor with a characteristic solid syncytial growth pattern displaying positivity with EMA and S100 immunohistochemical stains. Lately, EWSR1-PBX3 fusion has been documented in a vast majority. Although it follows a benign clinical course, its histopathological differential diagnosis includes clinically aggressive neoplasia. This contribution summarizes the derivation, clinical presentation, histopathological and immunohistochemical features, molecular genetics, pertinent differential diagnosis, and behavior of this unique cutaneous appendageal tumor.

Normocomplementemic urticarial vasculitis in a patient with multiple sclerosis on glatiramer acetate.

Glatiramer acetate is one of the oldest and safest disease modifying therapies used to treat relapsing-remitting multiple sclerosis. Urticarial vasculitis is a rare complication of treatment with glatiramer acetate, having been reported by only two others previously. Here, we describe a case of normocomplementemic urticarial vasculitis diagnosed on skin punch biopsy in a patient with multiple sclerosis treated with glatiramer acetate for five years. Upon treatment with steroids and an antihistamine along with discontinuation of glatiramer acetate, the urticaria resolved.

Diversity in dermatology: International medical graduates and their role in the diversity of a specialty.

A diverse medical workforce improves patient care. Dermatology is the second-least diverse medical specialty in the United States, and many recent publications have discussed the different reasons and possible solutions to improve this disparity. A quarter of physicians in the United States are international medical graduates, which directly affects the cultural diversity in health care. Dermatology has the lowest percentage of international medical graduates in its active physician workforce. Among other measures, the inclusion of more international medical graduates in residency programs can help improve the diversity in this specialty and alleviate any disparities in dermatological care delivery in underserved communities.

Ageing population and society: a scientometric analysis.

The ageing population and society (APS) nexus is one of the key grand challenges of this millennium. And yet, the systematic analysis of scholarly literature on the APS nexus has remained under the radar. This study responds to this gap and employs a quantitative approach through a scientometric analysis of literature on the APS nexus to inform policy discussions and guide future research directions. This study adopts quantitative scientometric methods to examine the APS literature (n = 566) between 2011 and 2020 found in the Scopus database. The analysis reveals key research topics and recognizes the most important articles, authors, publication outlets, institutions, and countries in the field. The findings indicate that while issues such as ageing population, gender, quality of life, and socio-economic aspects of ageing have received significant interest, social exclusion of older adults, age diversity, social policy, and the eldercare workforce have received less attention. As challenges associated with the APS nexus will continue to gain currency in the future, this paper discusses the implications of the findings on (a) future research direction and (b) north-south research collaboration. The analysis shown in this paper should be of interest to scholars and policymakers interested in addressing the challenges associated with the APS nexus.

Dermatopathology resident training and education during the COVID-19 pandemic: Challenges faced and lessons learned.

Clinical laboratory services and associated training programs faced unprecedented challenges during the coronavirus disease 2019 pandemic. With the introduction of pandemic related strictly mandated institutional policies of physical distancing, dermatopathology rotations, a key component of both dermatology and pathology residency programs, were impacted. In order to adapt to this new environment, a few modifications in resident training and education were introduced at various institutions. These disruptions initiated a change in the standard teaching approach, with a shift from face-to-face learning to a virtual and online model. These adaptations and innovations are discussed here with their likely benefits and limitations.

Muffin Technique Micrographic Surgery for Non-melanoma Skin Cancer.

Background: Mohs micrographic surgery (MMS) is the gold standard treatment for high-risk facial non-melanoma skin cancer. However, patients' access to MMS is limited by cost. The muffin technique micrographic surgery (MTMS) is an alternative micrographic technique wherein the entire excised margin is evaluated post-operatively by a pathologist using paraffin-embedded material. Herein, we describe the implementation and the preliminary results of MTMS in an academic dermatology center. Objective: To describe the MTMS and outline its efficacy and safety in a real-world clinical academic setting. Methods: A retrospective chart review was conducted of all patients with basal cell carcinoma (BCC) and squamous cell carcinoma (SCC) who underwent MTMS at the University of Alberta Dermatology Center from June 2016 until July 2019. Results: A total of 69 patients were included (64 BCCs and 5 SCCs). 68.1% of surgeries had clear margins following the first incision, 100% after second round re-excisions. There were no observed cases of tumor recurrence after a median 40 months of follow-up. There were no major adverse events or complications. Conclusions: MTMS is a superior alternative to simple excision of skin cancer by providing full margin control and residual tumor mapping.

A diagnosis of Stevens-Johnson Syndrome (SJS) in a patient presenting with superficial keratitis.

PURPOSE: To describe a case of Stevens-Johnson syndrome (SJS) diagnosed in a patient presenting with primarily ocular findings where SJS had not been initially suspected. OBSERVATIONS: A 23-year-old female presented with a 2 day history of bilateral eye pain, conjunctival injection, decreased visual acuity, and photophobia in the context of a 4 day history of fever, headache, and sore throat. She was found to have bilateral superficial keratitis and treated for suspected early infectious keratitis secondary to extended contact lens wear. She returned the next day with worsening visual symptoms, a new macular rash over her upper torso, and new ulcerating lesions over her buccal and perioral tissue. The patient was diagnosed with SJS. She was successfully treated using systemic cyclosporine with antibiotics and steroid eye drops. CONCLUSIONS AND IMPORTANCE: Ophthalmologists may be the first physicians to diagnose SJS, a life-threatening condition that can initially present with non-specific viral prodromal symptoms and ocular signs alone. This case emphasizes the importance of considering a patient's entire clinical history, especially when the presentation is atypical and the diagnosis is not obviously apparent.