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Malignant melanoma in-situ, lentigo maligna (MMIS-LM) can be successfully treated with several different surgical techniques; however, the literature is inconsistent in defining them. To comprehensively define and describe the national guideline recommended surgical techniques used to treat MMIS-LM to help clarify and standardize this terminology to ensure compliance with the guidelines. A targeted literature review was performed from 1990 to 2022 focusing on articles that discussed the national guideline recommended surgical techniques of wide local excision, Mohs micrographic surgery (MMS), modified Mohs surgery, and staged excision/Slow-Mohs for MMIS-LM, as well as the related methods of tissue processing. National Comprehensive Cancer Network and American Academy of Dermatology guidelines were reviewed to identify how the techniques need to be employed to be compliant with guideline recommendations. We describe the various surgical and tissue processing techniques and discuss advantages and disadvantages of each. This paper was styled as a narrative review defining and clarifying terminology and technique and does not investigate these topics more broadly. Understanding the methodology and terminology for these surgical procedures and tissue processing methods is critical so that both general dermatologists and surgeons can employ these techniques effectively for optimal patient care. [ABSTRACT FROM AUTHOR]

Risk factors for a primary cutaneous squamous cell carcinoma (CSCC) are well-established; however, the host and primary tumor risk factors for subsequent CSCC have not been fully explored. We performed a retrospective chart review of patients diagnosed with CSCC in an academic dermatology clinic in Rhode Island from 2016-2019. Logistic regression was used to evaluate the associations between host factors and multiple CSCC and between primary tumor characteristics and the risk of subsequent CSCC. Adjusted odds ratios (aORs) and 95% CIs were calculated. A total of 1312 patients with CSCC diagnoses were included. Host risk factors significantly associated with multiple CSCCs included: aged >80 years (aOR, 2.18; 95% CI, 1.46-3.31); history of: solid organ transplant (aOR, 2.41; 95% CI, 1.20-4.80); skin cancer (aOR, 1.96; 95% CI, 1.52-2.54); other cancer (aOR, 1.49; 95% CI, 1.11-2.00); family history of skin cancer (aOR, 1.36; 95% CI, 1.03-1.78); and actinic keratosis (aOR, 1.52; 95% CI, 1.18-1.95). Tumor location, diameter, histologic differentiation, and treatment were not significant predictors of subsequent CSCCs. Study patients were predominantly White and from a single institution, limiting the generalizability of results. Certain host characteristics were associated with the development of subsequent CSCC, which may inform clinical guidelines for follow-up. [ABSTRACT FROM AUTHOR]

Background: Assessing medical students on core skills related to melanoma detection is challenging in the absence of a well-developed instrument.Objective: We sought to develop an objective structured clinical examination for the detection and evaluation of melanoma among medical students.Methods: This was a prospective cohort analysis of student and objective rater agreement on performance of clinical skills and assessment of differences in performance across 3 schools.Results: Kappa coefficients indicated excellent agreement for 3 of 5 core skills including commenting on the presence of the moulage (k = 0.87, 95% confidence interval 0.77-0.96), obtaining a history for the moulage (k = 0.84, 95% confidence interval 0.74-0.94), and making a clinical impression (k = 0.80, 95% confidence interval 0.68-0.92). There were no differences in performance across schools with respect to 3 of 5 core skills: commenting on the presence of the moulage (P = .15), initiating a history (P = .53), and managing the suspicious lesion (P value range .07-.17). Overall, 54.2% and 44.7% of students commented on the presence of the moulage and achieved maximum performance of core skills, respectively, with no difference in performance across schools.Limitations: Limitations include overall sample size of students and schools.Conclusion: The Skin Cancer Objective Structured Clinical Examination represents a potentially important instrument to measure students' performance on the optimal step-by-step evaluation of a melanoma. [ABSTRACT FROM AUTHOR]

Background: Knowledge of the skin cancer examination (SCE) and its practice remain relevant competency gaps among medical students. Objective: We elaborate on a method of SCE known as the Integrated Skin Exam and discuss the development of an instructional film that illustrates its principles. We assess the tool’s effect on knowledge, attitudes, and perceptions related to the SCE. Methods: Second-year students among 8 randomized schools viewed the film and completed pre-post questionnaires. Results: After viewing The Integrated Skin Exam film, students demonstrated improved melanoma knowledge, including identification of high-risk demographic groups (61% vs 42.9%, P < .001), high-risk anatomic sites in women (88.6% vs 46.5%, P < .001) and men (92.1% vs 34.8%, P < .001), and the ABCDEs of melanoma (98.4% vs 91.2%, P < .001). Students demonstrated increased confidence in the SCE (66.93% vs 16.40%, P < .001) and augmented intentions to practice it (99.05% vs 13.9%, P < .001). A greater proportion (70.4% vs 41.9%, P < .001) of students thought less than 3 minutes were required to integrate SCE into the routine examination. Limitations: Longitudinal impact of the film was not assessed. Conclusion: The Integrated Skin Exam film introduces an integrated approach to the SCE that addresses knowledge gaps, mitigates perceived barriers, and augments intention related to practice of the SCE. [Copyright &y& Elsevier]

Cutaneous metastases occur in 0.7% to 9% of all malignancies. In women, cutaneous metastases occur most often in breast cancer, followed in order by colorectal carcinoma, melanoma and ovarian carcinoma. Of the squamous cell carcinomas (SCC) that do metastasize, many are exceedingly difficult to differentiate from primary SCC of the skin and are often found in the advanced stages with well-established primary tumor and lymph node involvement. This is an important distinction because metastatic cutaneous SCC is associated with a much poorer prognosis than primary SCC. Cutaneous metastases from vulvar cancers are even less common and have been reported in only 8 cases. We report a rare case of epidermotropic metastatic squamous cell vulvar cancer in a 77-year-old woman. [ABSTRACT FROM AUTHOR]

Background: Merkel cell carcinoma (MCC), an aggressive malignancy that has been increasing in incidence, rarely presents with an epidermotropic pattern. Objective: We conducted an immunohistochemical evaluation of 6 previously unpublished cases of epidermotropic MCC, focusing particularly on the staining characteristics of epithelial membrane antigen and cytokeratin-20 in the hope of providing insight into the mechanism of epidermotropism in MCC. Methods: This study is a retrospective evaluation using light microscopy and immunohistochemistry. Results: Forty cases of MCC with pathology at Rhode Island Hospital and the Miriam Hospital in Providence, RI, from 1983 through 2009 were reviewed. Following exclusion criteria, 6 patients (5 men, 1 woman) with a mean age of 82.5 years (range, 72-92) demonstrated epidermotropism. Three of 6 patients had MCC of the eyelid. In cases 1, 3, and 6, the perinuclear dot pattern observed with cytokeratin-20 in the epidermotropic MCC cells was less pronounced than the pattern observed in the dermis, and in all 6 of the tumors, the epidermal staining pattern observed with epithelial membrane antigen was not more or less prominent than the staining observed in the dermis. Limitations: The small total number of cases of epidermotropic MCC is a limitation. Conclusion: The data presented reinforce the differential diagnosis of tumors with an epidermotropic growth pattern and the importance of immunohistochemical staining in the histologic workup of such tumors: squamous cell carcinoma in situ, melanoma, mycosis fungoides, eccrine porocarcinoma, sebaceous carcinoma of the eyelid, mammary and extramammary Paget disease, MCC, and epidermotropic metastases. It is notable that 3 of 6 identified tumors were located on the eyelid; further study of epidermotropic MCC may shed more light on this finding, either as an unusual coincidence or a finding with unexplained significance. [Copyright &y& Elsevier]

Background: The clinical condition generalized epidermolytic hyperkeratosis, also known as bullous congenital ichthyosiform erythroderma, is an autosomal dominant disorder and presents as a bullous disease of the newborn followed by an ichthyotic skin disorder throughout life. Clinical epidermolytic hyperkeratosis (cEHK) has characteristic histopathologic findings. Mosaic cEHK, which occurs without a family history, is a sporadic condition that clinically resembles epidermal nevi but demonstrates histopathologic findings similar to the generalized disorder; when a postzygotic mutation involves the germ line, the disease can occur in subsequent generations as generalized cEHK. Ichthyosis bullosa of Siemens (IBS) is similar histopathogically, but is clinically distinct from generalized cEHK, presenting with more superficial bullae. Objectives: It is well established that the clinical diagnoses generalized cEHK, mosaic cEHK, and IBS have similar histopathologic findings of epidermolysis with hyperkeratosis. We sought (1) to characterize the spectrum of histopathologic features and (2) to assess whether there were histopathologic differences between these clinically distinct disorders. Methods: One hundred seventeen skin biopsy slides from the National Registry for Ichthyosis and Related Skin Disorders were reviewed, with those reviewers blinded to clinical information. All slides were systematically evaluated for a variety of features, including differences in the pattern of the epidermolysis and hyperkeratosis. Clinical predictions of whether the biopsy specimen was obtained from patients with generalized cEHK, mosaic cEHK, or IBS were made on the basis of histologic pattern of the epidermolysis and hyperkeratosis. Results: Eighteen of the 117 slides revealed features sufficient to make a histologic diagnosis of epidermolytic hyperkeratosis (hEHK). One additional slide, for which a definitive histologic diagnosis was not possible, had features of both hEHK and acantholytic dyskeratosis. Two distinct patterns of the histopathologic changes were observed within the 18 slides diagnostic of hEHK: (1) continuous involvement of the entire horizontal epidermis and (2) focal involvement revealing skip areas of normal-appearing epidermis along the horizontal epidermis. Upon clinical correlation, all 12 of the slides with continuous involvement were from patients with generalized cEHK. One slide was from acral skin and had continuous involvement; this was from a patient with Vorner''s palmoplantar keratoderma. Of the remaining 5 slides with focal involvement, two patterns were observed: focal involvement of both granular and spinous layers and focal involvement of only the granular layer. The 3 slides with focal involvement of the granular and spinous layers were from patients with mosaic cEHK. Of the two slides with focal involvement confined to the granular layer, one was from a patient with IBS and the other from a patient with generalized cEHK. Limitation: The sample pool is biased by who was enrolled in the Registry and therefore may not represent the full spectrum of the disease. Conclusion: The pattern of histologic involvement may be a useful predictor of the clinical phenotype of cEHK. [Copyright &y& Elsevier]

Blue nail dyschromia may represent melanocytic, vascular, or other etiologies. A case of a subungual blue nodule is presented, with a pseudo-clubbed nail. On histopathologic examination, there was a combined subungual blue nevus, with features of a common blue nevus and a pigmented epithelioid melanocytoma. This unusual presentation is reviewed, with a discussion of blue nail dyschromia and subungual blue nevi. [Copyright &y& Elsevier]

Primary cutaneous adenoid cystic carcinoma is a rare, slow-growing malignancy first described by Boggio in 1975. This tumor characteristically consists of basophilic cells with a distinct adenoid or cribriform pattern in the mid to deep reticular dermis. Modified myoepithelial cells with prominent basement membrane material often surround true lumina. Definitive diagnosis relies on the characteristic histologic features and the exclusion of metastatic disease. We describe two patients who presented with painful papules of the scalp and were successfully treated with wide local excision. [Copyright &y& Elsevier]

Nail dyschromia, including melanonychia and erythronychia, encompasses a wide range of possible diagnoses. While the majority of these lesions are benign, malignancies of the nail unit represent a sinister, and potentially life-threatening, cause of nail dyschromia. Unfamiliarity with tumors of the nail apparatus can lead to a delay in diagnosis. A case is presented of a patient with two separate and concurrent malignant neoplasms of the nail unit, on different nails on the same hand, each featuring an unusual clinical presentation: amelanotic melanoma presenting as longitudinal erythronychia and squamous cell carcinoma in situ presenting as longitudinal melanonychia. This presentation underscores the need for a low threshold for biopsy in the presence of nail dyschromia of uncertain etiology. [Copyright &y& Elsevier]

Heterotopic salivary gland tissue (HSGT) consists of salivary tissue outside of the major and minor salivary glands and typically presents as a draining sinus and/or asymptomatic nodule of the neck along the lower anterior sternocleidomastoid muscle. There are a limited number of case series exploring this rare entity. To further delineate the clinicopathologic characteristics of this lesion, we present 11 cases of HSGT in the neck, many with cutaneous involvement. Anatomic pathology files from Lifespan-affiliated hospitals (Rhode Island Hospital and Miriam Hospital) were retrospectively reviewed for all cases meeting criteria for HSGT from 1983 through 2005, and 11 patients were identified: 4 female and 7 male, ages 5 months to 64 years, with 8 patients younger than 18 years; 7 patients presented with a draining sinus. Of note, 8 of 11 cases occurred on the right side. In general, microscopic examination revealed mucinous and serous salivary glandular structures with an associated duct; no cytologic atypia was observed. All cases stained positive for smooth muscle actin and calponin, highlighting a myoepithelial layer similar to that seen in minor and major salivary glands. As in our series, most cases of cervical HSGT occur near the anterior sternocleidomastoid muscle with a right-sided predilection. Cases generally present by early childhood, although 3 cases in our series were diagnosed in adulthood. Although cancers arising in these lesions are fairly uncommon, most clinically apparent foci of HSGT are excised. This entity should be considered in the dermatologist''s differential diagnostic considerations for a draining sinus and a lateral, especially right-sided, neck mass. [Copyright &y& Elsevier]

Sweet''s syndrome–like (Sweet''s-like) neutrophilic panniculitis is usually idiopathic, but is frequently associated with hematologic, inflammatory, and immunologic disease. Drug-related cases of Sweet''s syndrome have been reported. Of relevance, chemotherapy with the retinoid all-trans-retinoic acid (ATRA) infrequently induces Sweet''s-like neutrophilic panniculitis that occurs concomitantly with neutrophilic differentiation. The pathologic features are limited to the adipose tissue or include both the dermis and the subcutaneous fat; the neutrophilic infiltrate can be observed in the lobules, the septae, or both. We present this case because of the unusual subcutaneous neutrophilic infiltrate consistent with Sweet''s-like neutrophilic lobular panniculitis in a patient with acute promyelocytic leukemia receiving ATRA chemotherapy. This case highlights the important connection between ATRA and Sweet''s syndrome. [Copyright &y& Elsevier]

Multicentric reticulohistiocytosis (MRH) is a rare histocytic disease characterized by destructive arthritis in association with classic skin findings. Although MRH is not strictly a paraneoplastic disease, one quarter of cases are malignancy related. We report a case of MRH with an initial remission followed by an acute exacerbation several years later heralding the clinical presentation of endometrial carcinoma. During this flareup a skin biopsy specimen revealed a diffuse dermal infiltrate composed of histiocytes with ground-glass cytoplasm and multiple atypical mitoses. Approximately 40% of the cells stained with the proliferation marker Ki-67. Treatment of endometrial carcinoma resulted in improvement of skin and joint symptoms, and a repeat biopsy specimen no longer demonstrated mitotic figures. These findings support a reactive and proliferative cause of MRH. [Copyright &y& Elsevier]

Adenomas in the anogenital region are uncommon. There has been debate about the origin, including ectopic breast tissue, cutaneous apocrine gland, and most recently anogenital mammary-like gland. An anogenital mass in a 36-year-old woman was excised, and histopathologic examination and immunostaining were performed. Microscopic tissue sections showed a morphologic pattern similar to that of a mammary fibroadenoma, and immunostaining demonstrated the presence of estrogen receptors and progesterone receptors. The possibility of adenomas of anogenital mammary-like glands should be considered when evaluating patients with a mass in this area with confirmation by tissue biopsy or aspiration cytology. [Copyright &y& Elsevier]

Nephrogenic systemic fibrosis/nephrogenic fibrosing dermopathy is an idiopathic fibrosing disorder recently described in patients with renal disease. The typical histology in nephrogenic systemic fibrosis consists of haphazardly arranged dermal collagen bundles with clefting, collagenous change which extends into the subcutaneous septa, mucin deposition, fibroblast proliferation, and increased elastic fibers with minimal inflammation. We present a 65-year-old female with classic clinical features whose biopsy demonstrated unique histologic features of septal pannicilitis with lymphocytic aggregates and Miescher''s radial granulomas mimicking erythema nodosum. [Copyright &y& Elsevier]

Porokeratotic eccrine ostial and dermal duct nevus and a similar condition, porokeratotic eccrine and hair follicle nevus, are rare disorders of keratinization with eccrine and hair follicle involvement. We describe the clinical features in 5 patients, all of whom had widespread skin involvement following the lines of Blaschko. Two patients presented with erosions in the newborn period as the initial manifestation of their disease; one had an associated structural anomaly, unilateral breast hypoplasia; and one adult had malignant transformation in the nevus with development of multifocal squamous cell carcinomas. Three patients had histologic involvement of both acrosyringia and acrotrichia. Based on the observation of overlapping histologic features, we propose the name “porokeratotic adnexal ostial nevus” to incorporate the previously described entities porokeratotic eccrine ostial and dermal duct nevus and porokeratotic eccrine and hair follicle nevus. [Copyright &y& Elsevier]

Background: Type III hyperlipoproteinemia usually results from an inherited defect in the composition of apolipoprotein E and is associated with atherosclerosis. An acquired form of the type III phenotype may rarely be associated with myeloma and immunoglobulin-lipoprotein complexes. Observation: We present the case of a 72-year-old man with a history of well-controlled, unclassified hypercholesterolemia and hypertriglyceridemia, without evidence of atherosclerotic disease. He subsequently developed refractory dyslipidemia, palmar crease, and tuberous xanthomas. Type III hyperlipoproteinemia was confirmed, and nonclassic defective apolipoprotein E. Common secondary causes of hyperlipidemia were ruled out. A workup for malignancy revealed monoclonal IgA gammopathy. Immunostaining confirmed IgA antibodies complexed to the patient''s very low-density lipoprotein (VLDL) fraction, causing gross impairment of VLDL metabolism. Conventional therapy for type III hyperlipoproteinemia was attempted but ineffective. Thus, chemotherapy was initiated for his myeloma, with subsequent lowering of his IgA, cholesterol, and triglyceride levels, and improvement of his xanthomas. Conclusion: There are several unusual features to this case. Planar xanthomas can be associated with myelomas, but usually in the setting of normal lipids. Type III hyperlipoproteinemias are not usually refractory to standard therapy and are only rarely associated with IgA myeloma. IgA antibodies complexed to the patient''s VLDL caused gross impairment of VLDL metabolism. The patient''s apolipoprotein E genotype (heterozygote E2/E3) is not typical for expression of the heritable type III phenotype (homozygote E2/E2). These features support a causal relationship between this patient''s multiple myeloma and type III hyperlipoproteinemia rather than two independent, coexistent conditions. [Copyright &y& Elsevier]

Localized hyperhidrosis on the left forearm of a 7-year-old girl is described. Biopsy revealed an eccrine nevus. The differential diagnosis of localized hyperhidrosis is discussed.

Elastic fibers in the extracellular matrix are integral components of dermal connective tissue. The resilience and elasticity required for normal structure and function of the skin are attributable to the network of elastic tissue. Advances in our understanding of elastic tissue physiology provide a foundation for studying the pathogenesis of elastic tissue disorders. Many acquired disorders are nevertheless poorly understood owing to the paucity of reported cases. Several acquired disorders in which loss of dermal elastic tissue produces prominent clinical and histopathologic features have recently been described, including middermal elastolysis, papular elastorrhexis, and pseudoxanthoma-like papillary dermal elastolysis, which must be differentiated from more well-known disorders such as anetoderma, acquired cutis laxa, and acrokeratoelastoidosis. Learning objective At the conclusion of this learning activity, participants should have an understanding of the similarities and differences between acquired disorders of elastic tissue that are characterized by a loss of elastic tissue.

Elastic fibers in the extracellular matrix are an integral component of dermal connective tissue. The resilience and elasticity required for normal structure and function of the skin may be attributed to the network of elastic tissue. Advances in our understanding of elastic tissue physiology provide a foundation for studying the pathogenesis of elastic tissue disorders. Many acquired disorders are nevertheless poorly understood due to the paucity of reported cases. Several acquired disorders in which accumulation or elastotic degeneration of dermal elastic fibers produces prominent clinical and histopathologic features have recently been described. They include elastoderma, linear focal elastosis, and late-onset focal dermal elastosis and must be differentiated from better-known disorders, among them acquired pseudoxanthoma elasticum, elastosis perforans serpiginosa, and Favré-Racouchot syndrome. Learning objective At the conclusion of this learning activity, participants should understand the similarities and differences between acquired disorders of elastic tissue that are characterized by an increase in elastic tissue, as well as the spectrum of solar elastotic dermatoses.

Two cases of compound melanocytic nevi with granular cell changes were studied. Histological examination revealed compound melanocytic nevi with a portion of the dermal component exhibiting a sheet like proliferation of nevoid cells with granular cytoplasm. Immunohistochemically, both populations of cells stained positive for S-100 and Melan-A. However, HMB-45 was positive only for the nevus cells with granular cytoplasm. We believe this represents granular cytoplasmic changes of nevus cells and does not denote malignant transformation.

Keratosis lichenoides chronica is a rare dermatosis of unknown etiology with a wide variety of cutaneous manifestations. We present a 47-year-old male with a history of progressive and recalcitrant hyperkeratotic and warty plaques, mimicking verrucous secondary syphilis both clinically and microscopically. We review the clinical manifestations, microscopic features, and treatment modalities for this rare and distinctive dermatosis.