Blaschkoid melanotic cutaneous lupus erythematosus with "melanocytic nests".

Melanotic cutaneous lupus erythematosus (LE) is a newly described clinical variant of chronic cutaneous LE, presenting with localized or diffuse brownish or grayish macular and reticulated pigmentation in the absence of erythema, scaling, atrophy, scarring, or telangiectasia. The diagnosis is based upon histopathology, which demonstrates the characteristic features of LE with an interface vacuolar dermatitis with melanophages, and a superficial and deep, perivascular and periadnexal lymphocytic infiltrate with mucin deposition. Herein, we describe a case of a 61-year-old White male presenting with melanotic cutaneous LE with a blaschkoid distribution on his face in which the histopathological phenomenon of "true melanocytic nests" in the setting of a lichenoid pattern was seen. We want to highlight how nests of cellular aggregates at the dermoepidermal junction labeling with melanocytic markers may occur in the setting of an interface tissue reaction. This benign reactional pattern may mimic atypical melanocytic proliferations, especially on sun-damaged skin. Clinicopathological correlation and careful microscopic examination using a panel of multiple melanocytic markers is crucial for making an accurate final diagnosis. All the cases of melanotic cutaneous LE reported in the literature are also reviewed.

Lichen Planus Pigmentosus with True Melanocytic Nests: A Case Report with a Comprehensive Literature Review.

Lichen Planus Pigmentosus (LPP) is an uncommon variant of lichen planus characterized by the development of dark greyish-brown macules and patches primarily affecting sun-exposed areas. Histologically, it presents with lichenoid interface dermatitis with many melanophages. In select cases, the presence of melanocytic nests or pseudomelanocytic nests within LPP lesions has been documented, posing a diagnostic challenge. We present a detailed case report of a 32-year-old Eritrean woman with a longstanding history of hyperpigmented macules, alongside an in-depth review of the existing literature on lichenoid dermatoses featuring melanocytic or pseudomelanocytic nests. This paper delves into the clinical presentation, histopathological features, differential diagnosis, and potential mechanisms underlying this intriguing phenomenon.

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.

Clinical, Dermoscopic, Ultrasonographic, and Histopathologic Correlations in Kaposi's Sarcoma Lesions and Their Differential Diagnoses: A Single-Center Prospective Study.

(1) Background: Kaposi's sarcoma (KS) is an angioproliferative neoplasm typically appearing as angiomatous patches, plaques, and/or nodules on the skin. Dermoscopy and ultrasonography have been suggested as an aid in the diagnosis of KS, but there is little evidence in the literature, especially regarding its possible differential diagnoses. Our aim is to describe and compare the clinical, dermoscopic, and ultrasonographic features of KS and KS-like lesions. (2) Methods: we conducted a prospective study on 25 consecutive patients who were first referred to our tertiary care center from January to May 2021 for a possible KS. (3) Results: 41 cutaneous lesions were examined by means of dermoscopy, Doppler ultrasonography, and pathology, 32 of which were KS-related, while the remaining 9 were lesions with clinical resemblance to KS. On dermoscopy, a purplish-red pigmentation, scaly surface, and the collarette sign were the most common features among KS lesions (81.3%, 46.9%, and 28.1%, respectively). On US, all 9 KS plaques and 21 KS nodules presented a hypoechoic image. Dermoscopic and Doppler ultrasonographic findings of KS-like lesions, such as cherry angioma, venous lake, glomus tumor, pyogenic granuloma, and angiosarcoma were also analyzed. (4) Conclusions: dermoscopy and Doppler ultrasonography can be useful to better assess the features of KS lesions and in diagnosing equivocal KS-like lesions.

Kaposi's sarcoma, biologics and small molecules: Navigating the complex interplay between host immunity and viral biology. A case series with focused review of the literature.

The consequences of cytokine-specific immune modulation in the development and course of Kaposi's sarcoma (KS) are poorly understood. A retrospective chart review of patients treated with biologic/small molecule drugs and followed at the dedicated KS outpatient service of our Dermatology Unit was performed. The literature on biologic and small molecule drug use in KS patients was also reviewed. Data concerning 12 KS patients treated with biologic/small molecule drugs were collected. After a median delay of 6 months following biologic or small molecule drug introduction, nine patients experienced either KS onset or reactivation. Drugs associated with KS onset or flaring were: rituximab, infliximab, ruxolitinib apremilast (1), mirikizumab, abatacept (1). After a median follow-up of 25 months, all cases achieved persistent complete response through culprit drug discontinuation or drug withdrawal plus treatment. No effect on KS course was recorded with tocilizumab and vedolizumab. Based on our experience with the largest case series reported to date as well as the available literature, tocilizumab and ustekinumab seem to exert an overall neutral effect on KS. On the other hand, rituximab, infliximab, and ruxolitinib have been associated with the development or worsening of pre-existing KS and should be carefully pondered before use. Due to limited and partly controversial evidence, no definitive conclusions can be drawn on vedolizumab, apremilast, mirikizumab, abatacept.

COVID-19 Vaccination in Patients with Classic Kaposi's Sarcoma.

The novel coronavirus disease 2019 (COVID-19) has represented an overwhelming challenge for worldwide health systems. Patients with cancer are considered at higher risk for severe COVID-19 and increased mortality in case of infection. Although data on the novel severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) vaccination in patients with cancer are limited, there is enough evidence supporting anti-infective vaccination in general in patients with active cancer, or with history of previous malignancy. Subjects with classic Kaposi's sarcoma (KS) represent a small subset of cancer patients, which should be considered at heightened risk for infections due to several factors including age, and impaired immune function status. Several cases of human herpesviruses reactivation among critically ill COVID-19 patients have been described. Moreover, in case of severe infection and treatment with immunomodulating agents, patients with CKS are exposed at significant risk of viral reactivation and disease progression. Considering the baseline clinical risk factors of patients with CKS, and the complex interplay of the two viral agents, SARS-CoV-2 vaccination should be strongly recommended among patients with KS. KS represents an interesting field to study the interactions among chronic viral infections, SARS-CoV-2 and the host's immune system. Prospective observational studies are needed to provide more insights on vaccine activity and safety among patients with cancer, optimal vaccine schedules, potential interactions with antineoplastic therapies, and other comorbidities including chronic viral infections.

Mucosal Kaposi's sarcoma in HIV-negative patients: a large case series from a single, tertiary referral center in Italy.

BACKGROUND: Mucosal involvement in HIV-negative Kaposi's sarcoma (KS) is uncommon but has potentially serious repercussions on patient care. Evidence regarding its epidemiology and optimal management is limited. Invasive endoscopic staging at diagnosis and periodically during follow-up is currently recommended by major guidelines. MATERIALS AND METHODS: We reviewed the clinical records of 1,308 HIV-negative KS patients followed at our dedicated KS outpatient service. Demographics, clinical characteristics, and treatment outcomes for cases with biopsy proven mucosal lesions were collected. RESULTS: Mucosal involvement was documented in 53 patients (4.1% of our cohort), being present at diagnosis in 28 (52.8%) and occurring at a later time in the remaining 25 (47.2%) patients, with a mean latency of 8 years (±7.7). Oral cavity (43.4%) and glans penis (39.6%) were the most frequently involved anatomical sites. Of those with available treatment response data, complete response (CR) of mucosal KS was appreciated in 41 cases (93.2%), while partial response (PR) and stable disease (SD) were documented in one (2.3%) and two cases (4.5%), respectively. Same-site recurrences were noticed in seven patients (17.1%). CONCLUSION: Mucosal involvement in HIV-negative KS is rare, and its recurrence, if properly treated, appears to be infrequent. Thus, routine invasive monitoring in this setting may be unnecessary. We propose a tailored approach based on the clinical manifestations of each patient, limiting the indication of invasive procedures to the first evaluation and in case of significant clinical worsening or to monitor known mucosal localizations.

Iatrogenic Kaposi's sarcoma in myasthenia gravis: learnings from two case reports.

INTRODUCTION: Myasthenia gravis (MG) is an autoimmune neuromuscular disease whose treatment encompasses acetylcholinesterase inhibitors, oral steroids, and other immunosuppressants. Kaposi's sarcoma (KS) is a lymphangioproliferative disease associated with human herpesvirus 8 (HHV-8) infection and immunodeficiency or immunosuppression, mainly corticosteroids. CASE REPORTS: We present two cases of MG patients treated with oral steroids who developed KS. Patient 1 was diagnosed with three oral KS lesions. Prednisone was discontinued with lesion regression and stabilization, while azathioprine and pyridostigmine prompted control of MG. Patient 2 developed KS lesions on the trunk and lower limbs while taking prednisone and azathioprine. Steroid tapering was started but new oral and lymph nodal lesions appeared. Paclitaxel therapy was introduced and the patient experienced pulmonary embolism and developed sensitive neuropathy. Complete remission of KS lesions was achieved and maintained with azathioprine and pyridostigmine as MG medications. CONCLUSIONS: KS is an uncommon but clinically relevant adverse event (AE) often induced by steroid therapy. It can be controlled by steroid withdrawal but could necessitate chemotherapy, which associates with further potential AEs. Skin evaluation should be performed in all patients with chronic steroid therapy. Steroid-sparing strategies, including new drugs, could reduce KS and other steroid-related comorbidities. HHV-8 testing should be considered before starting chronic immunosuppression.

Diagnosis and treatment of classic and iatrogenic Kaposi's sarcoma: Italian recommendations.

Kaposi's sarcoma (KS) is a lymphangioproliferative disorder associated with Human herpesvirus 8 (HHV8) infection. Four clinical subtypes are recognized: classic, endemic, epidemic (HIV-related) and iatrogenic. KS diagnosis is based on clinical features, histopathological assessment, and HHV8 serology. Classic KS is usually skin-limited and has a chronic course, while the iatrogenic variant may show mucosal, nodal or visceral involvement. Clinical staging is fundamental to guide the management. Localized disease may be treated with different local therapies, even if there are no randomized trials comparing these different modalities. Aggressive, disseminated KS and cases with visceral involvement usually require systemic chemotherapy, most commonly vinblastine, bleomycin or paclitaxel. Iatrogenic KS needs immunosuppression tapering/withdrawal and, if possible, switch to m-TOR inhibitors in post-transplant KS. The present work by a panel of Italian experts provides guidelines on KS diagnosis and management based on a critical review of the literature and a long and extensive personal experience.

Alopecia Areata and Toxic Metals.

Toxic metals are not so rare but are often neglected causes of alopecia areata in men and women. Thallium, arsenic, selenium, and mercury are the most common cause of metals-related alopecia, which is what Vicky Yu and colleagues' found. Other than the presence of thallium, arsenic, mercury, and selenium, cadmium, bismuth, lithium, and copper should also be taken into account when dermatologists are considering toxic metals as a potential cause of alopecia areata in humans.

Bone involvement in classic Kaposi's sarcoma.

BACKGROUND: Classic Kaposi's sarcoma (KS) is a rare angioproliferative neoplasm which typically occurs on the skin of the lower limbs of immunocompetent elderly men. Bone involvement in classic KS has been exceptionally reported. OBJECTIVES: To identify patients with classic KS who developed bone involvement based on a retrospective analysis of our departmental database. MATERIALS AND METHODS: Clinical presentation, diagnostic method, treatment, and outcome were analysed and compared with cases reported in the literature. RESULTS: In total, we identified 1,196 patients with classic KS, three (0.25%) of whom developed bone involvement. The patients were all male and the average age at onset of bone involvement was 81.3 years. All three patients had biopsy-proven anaplastic KS affecting a bone of the lower limb. Bone radiological features were non-specific in one patient, while in all patients magnetic resonance imaging revealed osteolytic lesions and/or a solid tumour. HHV8 genotype analysis was performed in two patients, and subtypes A and C were found. CONCLUSION: Bone involvement should be considered in patients with known KS presenting with bone pain.

Paclitaxel as first- or second-line treatment for HIV-negative Kaposi's sarcoma: a retrospective study of 58 patients.

Background: Paclitaxel has recently been approved for AIDS-related Kaposi's sarcoma (KS) and there is much interest also in HIV-negative KS.Objective: To assess the safety and effectiveness of intravenous paclitaxel in the treatment of non-HIV-associated KS.Method: A retrospective database analysis of our departmental database in histologically proven, HIV-negative KS.Results: Fifty-eight patients treated with intravenous paclitaxel 100 mg weekly were identified. Among these patients, 11 patients underwent paclitaxel as first-line treatment, whereas 47 received paclitaxel after other types of systemic chemotherapy. Fifty-three (94.6%) patients achieved a partial or a complete remission after a mean of 13.5 infusions. Disease progression was observed in two patients and one patient had a stable disease. Thirty-one (58.5%) of 53 responding patients are still stable after a mean of 19.1 months of follow-up, while 22 (41.5%) patients relapsed after a mean of 14 months. Paclitaxel was repeated in relapsed patients obtaining PR/CR in all cases. Tolerance was good except for one patient who discontinued the treatment because of a severe allergic reaction.Conclusion: Paclitaxel is effective for the treatment of non-HIV-related KS, both as first- and as second-line treatment. It is well tolerated and can be repeated without loss of efficacy.

Efficacy of a detergent combined with a moisturizer for the treatment of pruritus associated with xerosis in an elderly population affected by Kaposi's sarcoma.

BACKGROUND: Xerosis is common among patients with Kaposi's sarcoma (KS). The aim of our study was to evaluate the efficacy of a detergent containing dihydroavenanthramide D 5% combined with a moisturizer containing 1% of menthol for the treatment of chronic pruritus associated with xerosis in elderly KS patients. METHODS: We conducted a prospective, open-label, intra-individual, right/left comparative study. During the 4-week treatment study, patients used the test products on the right lower limb, and a basic skin cleanser plus basic cream on their left lower limb, in a predefined protocol. A 10 cm visual analogue scale (VAS), the hydration index (HI) of the stratum corneum and the overall dry skin score (ODSS) were used to assess pruritus and xerosis severity on admission, at 2 and 4 weeks. RESULTS: Thirty patients (24 males, 6 females, mean age: 76.6±6.8 years) were enrolled. At the end of 4 weeks, the mean pruritus VAS score declined from 4.2±2.2 to 1.7±1.4 on the right side, and from 4.2±2.2 to 2.3±1.5 on the left side. The HI score increased from 25.6±15.0 to 46.1±12.3 on the right side, and from 26.0±15.2 to 35.4±12.6 on the left side. Differences between the right and left limbs were significant for VAS score (P=0.0064), HI (P<0.0001) and ODSS values (P=0.0049). There was no adverse reaction to the test products. CONCLUSIONS: Daily use of a detergent containing dihydroavenanthramide D 5% combined with a moisturizer containing 1% of menthol improves chronic pruritus associated with xerosis in elderly adults with KS.