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Dear Editor, Extramammary Paget disease (EMPD) is a rare intraepithelial adenocarcinoma that usually affects areas with abundant apocrine glands, such as anogenital or axillary skin. To our knowledge, EMPD has never before been diagnosed in a patient with comorbid hidradenitis suppurativa (HS) or on adalimumab therapy. We present a case of EMPD diagnosed incidentally in excised skin from a patient who underwent surgical management of extensive HS of the groin. [Extracted from the article]

Background and Aims Metastatic Crohn's disease is an extraintestinal cutaneous manifestation characterised by non-specific inflammatory lesions anatomically separate from the gut; genital involvement is rare. We conducted a systematic review of anogenital Crohn's disease and granulomatosis, to provide a synthesis of epidemiology, clinical features, and treatment outcomes. Methods A systematic search of the literature was conducted via MEDLINE, EMBASE, and the Cochrane database from inception to December 1, 2020. Two investigators extracted and analysed study data. Response and remission were defined as partial improvement or complete resolution of symptoms and examination findings, respectively. Results Of 9381 screened studies, 185 articles, [410 cases: 273 female, 137 male] were included. The predominant clinical features were oedema, ulcers, fissures, and hypertrophic lesions. Adults and children present similarly. Luminal Crohn's disease was diagnosed in nearly 80% of cases including 45–80% patients without gastrointestinal symptoms (time to inflammatory bowel disease [IBD] from anogenital Crohn's disease diagnosis [range] –43 to 11 years). Antibiotics, corticosteroids, thiopurines, and anti-tumour necrosis factor [TNF] therapy were the most frequently prescribed agents. At final follow-up, non-response, response, and remission rates were 37/304 [12%], 267/304 [88%], and 114/304 [38%], respectively. Oedema was associated with a poor response to topical therapy. Greater response rates to anti-TNF therapy were seen in patients prescribed concomitant immunomodulation [24/25, 96% vs 67/90, 74%, p = 0.02]. Conclusions We provide an illustrative summary of the clinical presentation and treatment effectiveness of this rare, under-recognised condition, and a proposed algorithm for approach and management. Prospective studies with longer follow-up are required to define optimal treatment strategies. [ABSTRACT FROM AUTHOR]

Background: The differential diagnosis of atypical dermal nonepidermotropic CD8+ lymphocytic infiltrates includes a heterogeneous spectrum of lymphoproliferations with overlapping histological and phenotypic features, but divergent clinical manifestations and prognoses. As these neoplasms are rare, more data on their clinicopathological presentation and course are needed. Objectives: To assess the clinical, histological and immunophenotypic features; outcomes of; and differences between dermal CD8+ lymphoproliferations. Methods: Retrospective analysis of a series of 46 patients and biopsies by the international EORTC Cutaneous Lymphoma Group. Results: The dermal CD8+ lymphoproliferations (n = 46) could be assigned to one of three groups: (i) cutaneous acral CD8+ T‐cell lymphoma (n = 31), characterized mostly by a solitary nodule arising at acral sites, a monotonous dermal infiltrate of small‐to‐medium‐sized CD8+ lymphocytes with a characteristic dot‐like pattern of CD68, a low proliferation rate and an excellent prognosis; (ii) primary cutaneous CD8+ peripheral T‐cell lymphoma, unspecified/NOS (n = 11), presenting with one or multiple rapidly evolving tumours, mostly medium‐sized pleomorphic CD8+ tumour cells with expression of several cytotoxic markers, and high proliferative activity; and (iii) cutaneous CD8+ lymphoproliferations (n = 4), associated with congenital immunodeficiency syndromes in two patients with persisting localized or disseminated violaceous to brownish plaques on the extremities, a histiocyte‐rich infiltrate of mostly small CD8+ lymphocytes with subtle atypia and a protracted course; and papular CD8+ eruptions in two patients with acquired immunosuppression. Conclusions: A constellation of distinct clinical, histopathological and phenotypic features allows discrimination and assignment of dermal CD8+ infiltrates into distinct disease entities. Primary cutaneous acral CD8+ lymphoma, assigned a provisional category in current lymphoma classifications, is a distinct and reproducible entity. A correct diagnosis is essential to avoid unnecessarily aggressive treatment for indolent CD8+ lymphoproliferations and to identify cases with underlying immuno‐deficiency or potential for dismal outcome. [ABSTRACT FROM AUTHOR]