Wait and see: a case of EBV + cutaneous extranodal NK/T-type lymphoma with indolent behaviour.

Extranodal NK/T-cell lymphoma (ENKTL) is a rare lymphoma subtype associated with Epstein-Barr virus (EBV) infection, portending a poor prognosis despite systemic chemotherapy. We present the unusual case of an 85-year-old man receiving ibrutinib for mantle cell lymphoma, who developed a erythematous, subcutaneous nodule on the forehead, featuring a proliferation of pleomorphic CD8 + /CD56 - /EBV + cells. Given the negative staging and comorbidities, a watchful waiting strategy was performed, experiencing a benign course with self-resolution and complete remission over a 4-year follow-up. The literature on primary cutaneous ENKTL has been discussed, with particular attention to clinical and histological prognostic factors.

The Bowel-Associated Arthritis-Dermatosis Syndrome (BADAS): A Systematic Review.

Bowel-associated arthritis-dermatosis syndrome (BADAS) is a rare neutrophilic dermatosis that was first described in 1971 in patients who underwent bypass surgery for obesity. Over the years, the number of reported cases associated with medical gastroenterological conditions, particularly inflammatory bowel disease (IBD), has progressively increased. To date, there are no systematic reviews in the literature on BADAS. The design of an a priori protocol was based on PRISMA guidelines, and a search of PubMed and Scopus databases was conducted for articles published between 1971 and 2023 related to the topic. Fifty-one articles including 113 patients with BADAS were analyzed in this systematic review. Bariatric surgery and IBD were the most frequently reported causes of BADAS, accounting for 63.7% and 24.7% of all cases, respectively. A total of 85% of cases displayed the typical dermatological presentation, including urticarial maculopapular lesions centered by a vesicopustule, with the majority of lesions located on the upper limbs (73.5%). Polyarthralgia or localized arthritis were always present. Atypical presentations included cellulitis-like, erythema-nodosum-like, Sweet-syndrome-like and pyoderma-gangrenosum-like manifestations. Gastrointestinal symptoms were frequently observed in IBD-related cases (67.9%). The histopathology showed a neutrophilic infiltrate (96.6%). The most commonly used treatment regimens consisted of systemic corticosteroids, metronidazole and tetracyclines, either alone or in combination. A relapsing-remitting course was observed in 52.1% of patients. In conclusion, BADAS is a neutrophilic dermatosis that presents with a wide variety of cutaneous manifestations, both typical and atypical. Gastrointestinal symptoms are frequently observed, particularly in cases related to IBD. The histopathology is clear but not specific compared with other neutrophilic dermatoses. The diagnosis can be challenging, but the relapsing-remitting course and the strong association with polyarthralgia and gastrointestinal disease can aid in the diagnosis.

Pseudoresistance to permethrin in scabies.

Reports of permethrin resistance of Sarcoptes scabiei var. hominis are emerging. We propose that this may be pseudoresistance. The resistance is due to inadequate counselling by physicians, incorrect treatment (insufficient quantity of permethrin; too short length of treatment), and poor adherence and compliance by patients. Other reasons include single application of permethrin, suggestion to apply the drug for 6-8 hours, failed application on subungual folds, irritant contact dermatitis, in particular on genitals, for which some patients stop the treatment, and unexplainable use of permethrin in post-scabies prurigo. Thus, we believe that several cases of resistance to permethrin are actually cases of pseudoresistance.

Mpox: an updated review of dermatological manifestations in the current outbreak.

Mpox is a disease caused by a double-stranded DNA orthopoxvirus discovered in 1958. In 2022, an outbreak on an unprecedented scale marked its transition from neglected, zoonotic disease circulating almost exclusively within African borders to sexually transmitted infection (STI) of international concern. Although phylogenetic evidence suggests progressive evolution from the strain associated with the 2018 outbreak in Nigeria, epidemiological links with previous cases have still not been completely elucidated. Clinically, mpox presents with systemic symptoms, such as fever, headache, malaise and a characteristic cutaneous eruption, similar to that of cognate viruses (e.g. smallpox). Mpox pseudopustules evolve through several stages, including umbilication and crusting, and resolve in the span of 2-3 weeks. The hallmarks that set the 2022 outbreak apart from classic mpox were a disproportionate number of cases occurring in men who have sex with men, an often localized cutaneous picture and a significant burden in terms of concomitant STIs. Investigations into the disease pathogenesis, related immune response, clinical and dermoscopic features, in addition to studies aimed at defining novel management strategies, have advanced mpox knowledge considerably. Herein, recent findings on mpox are reviewed, with a keen focus on dermatological manifestations and their implications in the current diagnostic scenario, reinforcing the pivotal role of dermatologists in managing suspect cases and preventing further spread of the contagion.

Koebner Phenomenon in Kaposi's Sarcoma: A Large Single-Center Case Series and Review of Current Knowledge.

BACKGROUND: occasional case reports have described the appearance of Kaposi's sarcoma (KS) on previously unaffected skin after incidental or accidental injury, but the association is probably under-reported. OBJECTIVES: to present a large case series of patients suffering from Koebner phenomenon (KP) in KS and describe their main epidemiological, clinical, and therapeutic features. METHODS: we have retrospectively analyzed our clinical and photographic records of 524 patients who had been diagnosed with KS between 2009 and 2021. RESULTS: 31 of 524 (6%) KS patients developed KP. Among these 31 patients, 24 (77%) had KS lesions after surgery, 4 (13%) after electrochemotherapy, laser therapy and cryotherapy, and 3 (10%) on areas affected by bullous diseases. CONCLUSIONS: trauma, including surgery or other medical procedures, can trigger KS, underlying the importance of treatment options which cause the least injury to the skin.

Brunsting-Perry pemphigoid: a systematic review.

BACKGROUND: Brunsting-Perry pemphigoid (BPP) is a rare, autoimmune bullous skin disorder classified within the spectrum of mucous membrane pemphigoid (MMP). MATERIALS AND METHODS: An a priori protocol was designed based on PRISMA guidelines. PubMed and Scopus databases were searched for English-language articles concerning BPP published between 1950 and July 2021. RESULTS: Thirty-six articles including 63 BPP patients were analyzed. The mean age at diagnosis was 62.9 years (range: 27-86). BPP was shown to be characterized by vesiculobullous lesions (46/63, 73.0%) on an erythematous base, erosions or ulcerations (27/63, 42.9%), atrophic scars (49/63, 77.8%), and milia (4/63, 6.3%). Exclusive oral mucosal involvement was documented in 22.2% of cases, usually manifesting after the cutaneous onset of the disease. Subepidermal blistering was a constant finding, often with an eosinophil-rich inflammatory infiltrate (21/58, 36.2%). Positive direct immunofluorescence was found in 92.0% of patients, almost always with linear IgG ± C3 deposits along the basement membrane (43/46, 93.5%). BP180 (12/15, 80.0%), BP230 (5/15, 33.3%), and laminin 332 (3/15, 20.0%) were the most frequently identified target antigens. CONCLUSIONS: BPP nosologic position remains uncertain, given the overlap with other autoimmune bullous diseases, such as MMP, bullous pemphigoid, and epidermolysis bullosa acquisita, particularly in its BPP-like variant. Nonpredominant oral mucosal lesions may appear during the course of the disease, generally after cutaneous manifestations. Positivity of DIF and anti-BP180/230 autoantibodies detected on ELISA/immunoblotting in the absence of anticollagen VII antibodies may provide guidance in diagnosing BPP.