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Dermatopathology, Volume 7, Issue 2 (September 2020) – 4 articles

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Open AccessCase Report
Emaciation, Congestive Heart Failure, and Systemic Amyloidosis in Severe Recessive Dystrophic Epidermolysis Bullosa: Possible Internal Complications Due to Skin-Derived Inflammatory Cytokines Derived from the Injured Skin
Dermatopathology 2020, 7(2), 41-47; https://0-doi-org.brum.beds.ac.uk/10.3390/dermatopathology7020007 - 14 Sep 2020
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Abstract
Inherited epidermolysis bullosa (EB) is a rare genetic skin disorder characterized by epithelial tissue fragility. Recessive dystrophic epidermolysis bullosa (RDEB) is the most severe form, characterized by the presence of blisters, erosion, and ulcer formation, leading to scarring and contraction of the limbs. [...] Read more.
Inherited epidermolysis bullosa (EB) is a rare genetic skin disorder characterized by epithelial tissue fragility. Recessive dystrophic epidermolysis bullosa (RDEB) is the most severe form, characterized by the presence of blisters, erosion, and ulcer formation, leading to scarring and contraction of the limbs. RDEB is also associated with extra-cutaneous complications, including emaciation, congestive heart failure, and systemic amyloidosis. The main cause of these clinical complications is unknown; however, we hypothesized that they are caused by elevated circulating inflammatory cytokines overproduced by injured keratinocytes. We addressed this phenomenon using keratin-14 driven, caspase-1 overexpressing, transgenic (KCASP1Tg) mice in which injured keratinocytes release high levels of IL-1α and β. KCASP1Tg showed severe spontaneous dermatitis, as well as systemic complications, including aberrant weight loss, cardiovascular disease, and extensive amyloid deposition with organ dysfunction, resembling the complications observed in severe EB. These morbid conditions were partially ameliorated by simultaneous administration of anti-IL-1α and β antibodies. The skin not only constitutes a physical barrier, but also functions as the largest immune organ. We suggest a novel role for IL-1 in the pathogenesis of EB and the use of anti-IL-1 antibodies as a potential therapy for EB complications. Full article
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Open AccessCase Report
Pseudolymphomatous Granuloma Annulare: A Case Report
Dermatopathology 2020, 7(2), 38-40; https://0-doi-org.brum.beds.ac.uk/10.3390/dermatopathology7020006 - 08 Sep 2020
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Abstract
Granuloma annulare is an idiopathic granulomatous condition. Clinical variants of granuloma annulare include classical and localized, large erythematous patch, generalized, perforating, and subcutaneous/deep forms. Rarely, granuloma annulare shows a prominent lymphoid infiltration. This form is called pseudolymphomatous granuloma annulare. Here, we describe a [...] Read more.
Granuloma annulare is an idiopathic granulomatous condition. Clinical variants of granuloma annulare include classical and localized, large erythematous patch, generalized, perforating, and subcutaneous/deep forms. Rarely, granuloma annulare shows a prominent lymphoid infiltration. This form is called pseudolymphomatous granuloma annulare. Here, we describe a new case of pseudolymphomatous granuloma annulare. Full article
(This article belongs to the Section Clinico-Pathological Correlation in Dermatopathology)
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Open AccessReview
Clear Cell Acanthoma: A Review of Clinical and Histologic Variants
Dermatopathology 2020, 7(2), 26-37; https://0-doi-org.brum.beds.ac.uk/10.3390/dermatopathology7020005 - 25 Aug 2020
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Abstract
Degos and Civatte first described clear cell acanthoma (CCA) in 1962 and later in a review article found that, in most instances, the lesion was a solitary red-brown dome-shaped papule that involved the distal lower extremity. The first morphologic variant of CCA was [...] Read more.
Degos and Civatte first described clear cell acanthoma (CCA) in 1962 and later in a review article found that, in most instances, the lesion was a solitary red-brown dome-shaped papule that involved the distal lower extremity. The first morphologic variant of CCA was reported as a “giant form of the acanthoma of Degos” which measured 45 × 40 mm, about twice the size of the largest CCA documented earlier. Since then, many variants of CCA have been described, including polypoid, pigmented and atypical. Herein, we describe a new variant of CCA and add another example of the polypoid variant to the literature. The new variant exhibits cellular features of trichilemmoma but architecturally differs from it. We also attempt to broaden the list of CCA variants summarized by Tempark and Shwayder by adding ours and a few more examples of CCA. The new variants of CCA include verrucous, linear, subungual and trichilemmal. Full article
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Open AccessCase Report
Allopurinol-Induced Oral Lichenoid Drug Reaction with Complete Regression after Drug Withdrawal
Dermatopathology 2020, 7(2), 18-25; https://0-doi-org.brum.beds.ac.uk/10.3390/dermatopathology7020004 - 12 Aug 2020
Viewed by 1055
Abstract
Background: Lichen planus is a chronic mucocutaneous inflammatory disease. Oral manifestations are common, and may remain exclusive to the oral mucosa without involvement of the skin or other mucosae. A differential diagnosis includes oral lichenoid drug reactions. Allopurinol, which is the first [...] Read more.
Background: Lichen planus is a chronic mucocutaneous inflammatory disease. Oral manifestations are common, and may remain exclusive to the oral mucosa without involvement of the skin or other mucosae. A differential diagnosis includes oral lichenoid drug reactions. Allopurinol, which is the first line hypo-uricemic treatment, is often quoted as being a possible offending drug, though oral reactions have rarely been reported. Case presentation: We describe a 59-year-old male gout patient, successfully treated with allopurinol, who developed acute onset of oral lichenoid lesions, involving bilaterally the buccal mucosa, the tongue and the labial mucosa. Histopathology was consistent with a lichen planus or a drug-induced lichenoid reaction. Improvement of the patient’s condition after withdrawal of allopurinol confirmed the lichenoid nature of the lesion. Remission was complete after a few weeks. Discussion: Although unusual, allopurinol may induce a lichenoid drug reaction. These reactions may mimic clinically and histopathologically idiopathic lichen planus. Improvement or complete regression of the lesions may be attempted to confirm the diagnosis. According to the latest WHO recommendations, these lesions have a potential for malignant transformation. Full article
(This article belongs to the Section Clinico-Pathological Correlation in Dermatopathology)
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