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Dermatopathology, Volume 8, Issue 2 (June 2021) – 6 articles

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Open AccessReview
Ichthyoses—A Clinical and Pathological Spectrum from Heterogeneous Cornification Disorders to Inflammation
by , and
Dermatopathology 2021, 8(2), 107-123; https://0-doi-org.brum.beds.ac.uk/10.3390/dermatopathology8020017 (registering DOI) - 07 May 2021
Abstract
Ichthyoses are inborn keratinization disorders affecting the skin only (non-syndromic) or are associated with diseases of internal organs (syndromic). In newborns, they can be life-threatening. The identification of the gene defects resulted in reclassification and a better understanding of the pathophysiology. Histopathologic patterns [...] Read more.
Ichthyoses are inborn keratinization disorders affecting the skin only (non-syndromic) or are associated with diseases of internal organs (syndromic). In newborns, they can be life-threatening. The identification of the gene defects resulted in reclassification and a better understanding of the pathophysiology. Histopathologic patterns include orthohyperkeratosis with a reduced or well-developed stratum granulosum, hyperkeratosis with ortho- and parakeratosis with preserved or prominent stratum granulosum, and epidermolytic ichthyosis. Another pattern features “perinuclear vacuoles and binucleated keratinocytes”, which is associated with keratin mutations. Some ichthyoses are histologically defined by psoriasis-like features, and distinct subtypes show follicular hyperkeratosis. In addition to histological and immunohistochemical methods, these patterns allow a better histopathologic diagnosis. Full article
(This article belongs to the Special Issue New Insights in Pediatric Dermatopathology)
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Open AccessCase Report
Bilateral Nipple Enlargement as a Secondary Effect of Anabolic Drugs: A Histopathological Mimicker of Smooth Muscle Hamartoma
Dermatopathology 2021, 8(2), 103-106; https://0-doi-org.brum.beds.ac.uk/10.3390/dermatopathology8020016 - 15 Apr 2021
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Abstract
Smooth muscle hamartoma are usually solitary and congenital, may affect the genital area and nipples. Histopathologically, they are characterized by the presence of mature smooth muscle bundles. We present a 40 year-old male with bilateral nipple enlargement excised with clinical suspicion of bilateral [...] Read more.
Smooth muscle hamartoma are usually solitary and congenital, may affect the genital area and nipples. Histopathologically, they are characterized by the presence of mature smooth muscle bundles. We present a 40 year-old male with bilateral nipple enlargement excised with clinical suspicion of bilateral leiomyoma. Skin biopsy shows mature, irregularly arranged smooth muscle bundles and lactiferous ducts between them. Immunohistochemistry is positive for smooth muscle actin, desmin and fumarase, but negative for estrogen and progestogen receptors. The presence of lactiferous ducts excludes bilateral leiomyomas. Even when, histopathologically, this can be interpreted as the nipple-type of muscular hamartoma of the breast, clinical history favors an anabolic drug-induced lesion. Bodybuilders present gynecomastia and nipple enlargement as frequent problems, but we have not found any histopathological description of these nipple lesions. We consider that dermatologists should be aware of the presence of them and dermatopathologists should know their histopathological features to avoid misdiagnosis as neoplasms. Full article
(This article belongs to the Section Clinico-Pathological Correlation in Dermatopathology)
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Open AccessReview
Pleomorphic Hyalinizing Angiectatic Tumor (PHAT): Review of the Literature with Case Presentation
Dermatopathology 2021, 8(2), 97-102; https://0-doi-org.brum.beds.ac.uk/10.3390/dermatopathology8020015 - 04 Apr 2021
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Abstract
Pleomorphic hyalinizing angiectatic tumor (PHAT) is a very rare entity of soft tissue considered a “neoplasm of uncertain behaviour of connective or other soft tissue” by the World Health Organization (2020). It develops in subcutaneous tissue of the lower extremities, more frequently in [...] Read more.
Pleomorphic hyalinizing angiectatic tumor (PHAT) is a very rare entity of soft tissue considered a “neoplasm of uncertain behaviour of connective or other soft tissue” by the World Health Organization (2020). It develops in subcutaneous tissue of the lower extremities, more frequently in the region of the ankle and foot, and rarely as a deep-seated soft tissue mass in locations such as the perineum, buttock, arms, head and neck, and viscera. Although inconsistent cytogenetic data have been reported on PHAT so far, there are potential morphological and genetic overlaps with hemosiderotic fibrolipomatous tumor (HFLT) and myxoinflammatory fibroblastic sarcoma (MIFS). Here we report a case of PHAT at the level of the upper third of the right thigh in a 48-year-old patient and we also focus on the differential diagnoses of these entities and conduct a literature review of reported cases. Full article
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Open AccessArticle
Role of CD123 (+) Plasmacytoid Dendritic Cells in Etiologically Different Variants of Erythema Multiforme: A Monocentric Retrospective Study
Dermatopathology 2021, 8(2), 89-96; https://0-doi-org.brum.beds.ac.uk/10.3390/dermatopathology8020014 - 03 Apr 2021
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Abstract
Plasmacytoid dendritic cells (pDCs) constitute a subset of dendritic cells known to be the “professional” interferon type I (IFN-I) producers. pDCs play an important role in antiviral immunity, as well as linking innate and adaptive immunity. Under normal conditions pDCs are not present [...] Read more.
Plasmacytoid dendritic cells (pDCs) constitute a subset of dendritic cells known to be the “professional” interferon type I (IFN-I) producers. pDCs play an important role in antiviral immunity, as well as linking innate and adaptive immunity. Under normal conditions pDCs are not present in skin. They are shown to be a part of the inflammatory infiltrate in different skin conditions including erythema multiforme (EM). This condition is considered to be a cell-mediated immune reaction to a wide variety of agents, most commonly herpes simplex virus. Nevertheless, the pathophysiology of EM still remains unclear. In this study, we grouped 32 biopsies from 30 patients diagnosed with EM, based on their etiology and analyzed the density and distribution of CD123 positive pDCs. In all cases we observed a greatly increased number of pDCs in the dermal inflammatory infiltrate. Virally-induced EM (by herpes simplex virus (HSV) and other viruses) was more likely to have a significantly higher number of pDCs compared to non-virally associated EM. Hence, we think that pDCs play a key role in the pathogenesis of EM independent of etiology and may play an increased role in virally-associated cases. Further studies on pDCs would clarify their importance in EM and improve our understanding of the pathophysiology of this disease. Full article
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Open AccessArticle
Inter-Observer and Intra-Observer Variations in the Assessment of Epithelial Dysplasia in Oral Lichenoid Diseases
Dermatopathology 2021, 8(2), 84-88; https://0-doi-org.brum.beds.ac.uk/10.3390/dermatopathology8020013 - 01 Apr 2021
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Abstract
Oral lichen planus (OLP) and oral lichenoid lesions (OLL) can both present with histological dysplasia. Despite the presence of WHO-defined criteria for the evaluation of epithelial dysplasia, its assessment is frequently subjective (inter-observer variability). The lack of reproducibility in the evaluation of dysplasia [...] Read more.
Oral lichen planus (OLP) and oral lichenoid lesions (OLL) can both present with histological dysplasia. Despite the presence of WHO-defined criteria for the evaluation of epithelial dysplasia, its assessment is frequently subjective (inter-observer variability). The lack of reproducibility in the evaluation of dysplasia is even more complex in the presence of a lichenoid inflammation. We evaluated dysplasia in 112 oral biopsies with lichenoid inflammation in order to study the inter-observer and the intra-observer variability. Full article
(This article belongs to the Section Clinico-Pathological Correlation in Dermatopathology)
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Open AccessArticle
Modulation of Gene Expression in a Sterile Atopic Dermatitis Model and Inhibition of Staphylococcus aureus Adhesion by Fucoidan
Dermatopathology 2021, 8(2), 69-83; https://0-doi-org.brum.beds.ac.uk/10.3390/dermatopathology8020012 - 25 Mar 2021
Viewed by 767
Abstract
Atopic dermatitis is a multifactorial pathology that includes perturbations of gene expression and increased adhesion of Staphylococcus aureus. Fucoidans are seaweed-derived sulfated fucose-rich polysaccharides that are known to be anti-inflammatory and may inhibit adhesion of pathogens. Fucoidan was assessed for effects on gene [...] Read more.
Atopic dermatitis is a multifactorial pathology that includes perturbations of gene expression and increased adhesion of Staphylococcus aureus. Fucoidans are seaweed-derived sulfated fucose-rich polysaccharides that are known to be anti-inflammatory and may inhibit adhesion of pathogens. Fucoidan was assessed for effects on gene expression of an in vitro 3D model of atopic dermatitis. It was also assessed for inhibitory effects on the adhesion of bacteria onto 3D reconstructed skin. Fucoidan significantly altered gene expression in the atopic dermatitis model, and there was a trend to reduce periostin levels. Fucoidan significantly inhibited the adhesion of Staphylococcus aureus and Cutibacterium acnes but did not affect the adhesion of Staphylococcus epidermidis. Fucoidan may be a useful topical agent to assist in the management of atopic dermatitis. Full article
(This article belongs to the Section Molecular Dermatopathology)
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