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Lymphomatoid papulosis (LyP) is a rare, chronic CD30+ cutaneous lymphoproliferative disorder characterized by recurrent, self-healing papulonodular lesions. Despite its benign clinical course, LyP histologically resembles malignant lymphomas, necessitating careful differentiation. A 42-year-old woman presented with a 3-year history of recurring ulcerated papulonodular lesions on her index finger. Histopathological examination revealed atypical CD30+ lymphoid proliferation, confirming LyP type A. Immunohistochemical analysis was positive for CD2, CD4, CD30, and MUM-1, while systemic malignancy was excluded. The patient was treated with low-dose methotrexate (15 mg/week), leading to symptom improvement. LyP is classified into five histological subtypes (A-E) and is often misdiagnosed due to its overlap with inflammatory and neoplastic conditions. While the condition typically resolves spontaneously, it is associated with an increased risk of secondary lymphomas, including mycosis fungoides and primary cutaneous anaplastic large cell lymphoma. Accurate diagnosis relies on clinical presentation, histopathological evaluation, and immunophenotyping. Awareness of LyP's clinical and pathological features is essential for appropriate management and surveillance.

To evaluate the association of anti-IFI16 antibodies with peripheral vasculopathy and the predictive value of anti-IFI16 antibodies for the development or persistence of digital ulcers (DPDU) in SSc. A total of 42 SSc patients and 42 age- and sex-matched healthy controls were enrolled. Anti-IFI16 antibodies were examined by ELISA. Nailfold videocapillaroscopy (NVC) and power Doppler ultrasound (PDUS) were used to assess the micro- and macro-vascular involvement in SSc. All patients were followed up for 6 months to evaluate DPDU. Potential risk factors for DPDU were analyzed by a Firth's penalized logistic regression model. Of the 42 SSc patients enrolled, 19.05% patients were positive for anti-IFI16 antibodies, compared to a significantly low positivity rate of 4.76% in healthy controls (p < 0.001). SSc patients who were positive for anti-IFI16 antibodies manifested higher ulnar artery resistance index than anti-IFI16 negative patients (p = 0.018). Within a 6-month follow-up, 14 (33.3%) patients suffered from DPDU, and the prevalence of anti-IFI16 antibodies in patients with DPDU was 42.9%, remarkably higher than 7.1% in those without DPDU (p = 0.012). Additionally, patients with DPDU were more likely to have digital ulcers (DUs) at enrollment and manifest lower finger pulp blood flow, lower ulnar artery (UA) flow velocity, lower UA resistance index, and higher UA resistance index at baseline in comparison to patients without DPDU. Multivariate analysis further identified DUs at enrollment (OR 5.81; 95% CI 1.09-30.86; p = 0.046) and the positivity of anti-IFI16 antibody (OR 8.64; 95% CI 1.05-70.87; p = 0.045) as independent risk factors for DPDU. Presence of anti-IFI16 antibody is associated with higher UA resistance index in SSc. Multivariate analysis further identified anti-IFI16 antibody as a predictive marker for DPDU in SSc. Key Points • SSc patients who were positive for anti-IFI16 antibodies manifested higher ulnar artery resistance at baseline. • The prevalence of anti-IFI16 antibodies in patients with DPDU during the 6-month follow-up was remarkably higher than those without DPDU. • Multivariate analysis identified DUs at enrollment and anti-IFI16 antibody positivity as independent risk factors for DPDU. • Anti-IFI16 antibody is associated with peripheral vasculopathy in SSc. Multivariate analysis further identified anti-IFI16 as a predictive biomarker for the development of DUs., Competing Interests: Declarations. Disclosures: None., (© 2024. The Author(s), under exclusive licence to International League of Associations for Rheumatology (ILAR).)

Painful ulcerations developed in a 33-year-old woman with anti-NXP-2-positive dermatomyositis in the facial and trunk areas and a 67-year-old woman with TIF1-gamma-positive dermatomyositis on the hands, while undergoing systemic therapy with azathioprine or low-dose methylprednisolone and cyclic administration of intravenous immunoglobulins (IVIG), respectively. In the laboratory workup, the anti-MDA‑5 antibody status remained negative and the creatine kinase (CK) normal in both patients, while histopathological examinations were nonspecific. Intensive topical class 4 corticosteroid therapy and continuation of the immunosuppressive or immunomodulating therapy led to healing of the ulcerative skin lesions. This report aims to raise awareness of the rare occurrence of cutaneous ulceration in anti-NXP-2- or anti-TIF1-gamma-positive dermatomyositis, as ulcerations may be associated with a severe disease course. In both of our patient cases, it is exclusively a dermal manifestation without other complications., Competing Interests: Einhaltung ethischer Richtlinien. Interessenkonflikt: C. Wald, J.-C. Simon und R. Treudler geben an, dass kein Interessenkonflikt besteht. Für diesen Beitrag wurden von den Autor/-innen keine Studien an Menschen oder Tieren durchgeführt. Für die aufgeführten Studien gelten die jeweils dort angegebenen ethischen Richtlinien. Für Bildmaterial oder anderweitige Angaben innerhalb des Manuskripts, über die Patient/-innen zu identifizieren sind, liegt von ihnen und/oder ihren gesetzlichen Vertretern/Vertreterinnen eine schriftliche Einwilligung vor., (© 2025. The Author(s).)

Marjolin's ulcer (MU) is a rare, aggressive skin tumor. There are numerous case reports but large long-term studies are lacking, necessitating further exploration of its treatment. This study aimed to summarize and analyze the characteristics, treatment methods, and prognosis of MU. We retrospectively analyzed the clinical data of 126 patients with MU, treated between January 2013 and January 2023 at the burn center. Demographic data, clinical characteristics, treatment, and prognosis were statistically analyzed. Of the 126 included patients, 104 were followed up for 0.1-10.2 years. The most common cause of the primary injury was flame burn (50.8%). Lesions were commonly observed on the lower limbs (47.6%). The predominant histopathological type was squamous cell carcinoma (92.8%). Among the 126 patients, 35 (27.8%) presented with bone invasion, 37 (29.4%) presented with enlarged lymph nodes, and 9 (7.1%) had lymph node metastasis. Extensive local excision (83.3%) was the most common surgical procedure; the defect was repaired using skin grafting (41.9%), free flaps (37.1%), and local flaps (21.0%). Multivariate analysis revealed that bone invasion and lymph node involvement were risk factors for postoperative recurrence. Survival analysis showed that age, latency period, pathological type, and recurrence were significant risk factors for survival. Extensive local resection is necessary to eradicate tumors, and patient follow-up should be more frequent within 1 year postoperatively. As MU is preventable, it is essential to reach a quick diagnosis and avoid delayed management before the occurrence of deadly metastases., (© The Author(s) 2024. Published by Oxford University Press on behalf of the American Burn Association. All rights reserved. For commercial re-use, please contact reprints@oup.com for reprints and translation rights for reprints. All other permissions can be obtained through our RightsLink service via the Permissions link on the article page on our site—for further information please contact journals.permissions@oup.com.)

Granulomatosis with polyangiitis (GPA) is a small-to-medium vessel vasculitis that can affect the skin, respiratory tract, kidneys and other organs. A rare cutaneous manifestation of GPA is pyoderma gangrenosum (PG)-like ulcerations, which can have debilitating and disfiguring consequences. We report the case of a man in his 40s with refractory PG-like ulcerations secondary to GPA, not responsive to conventional immunosuppression, who was successfully treated with rituximab. This case emphasises the need for early recognition of this rare manifestation to allow for prompt initiation of treatment., Competing Interests: Competing interests: None declared., (© BMJ Publishing Group Limited 2025. No commercial re-use. See rights and permissions. Published by BMJ Group.)

Objectives: To update the 2017 European Alliance of Associations for Rheumatology (EULAR) recommendations for treatment of systemic sclerosis (SSc), incorporating new evidence and therapies., Methods: An international task force was convened in line with EULAR standard operating procedures. A nominal group technique exercise was performed in two rounds to define questions underpinning a subsequent systematic literature review. The evidence derived was discussed and overarching principles, recommendations and future research agenda were iteratively developed with voting rounds., Results: The task force agreed on 22 recommendations covering 8 clinical/organ domains including Raynaud's phenomenon, digital ulcers, pulmonary arterial hypertension, scleroderma renal crisis, skin fibrosis, interstitial lung disease (ILD), gastrointestinal manifestations and arthritis. Most new recommendations are related to skin fibrosis and ILD. These included novel recommendations for the use of mycophenolate mofetil, nintedanib, rituximab and tocilizumab for the treatment of these crucial disease manifestations. The recommendations also included first-line and second-line interventions, providing increased utility for rheumatology practitioners. Important additions to the future research agenda included consideration of novel interventions for the management of vascular, musculoskeletal and gastrointestinal manifestations and calcinosis, as well as for the local management of digital ulcers., Conclusion: These updated recommendations include the first set of synthetic and biological targeted therapies recommended for key fibrotic manifestations of SSc as well as first-line combination treatment for newly diagnosed pulmonary artery hypertension and prioritise a new research agenda for the coming years., (Copyright © 2024 The Author(s). Published by Elsevier B.V. All rights reserved.)

Endovascular treatment (EVT) for peripheral artery disease in patients with chronic limb-threatening ischemia (CLTI) is a common practice in contemporary medicine and its effectiveness is widely acknowledged. However, refractory ulcers can occasionally be encountered, particularly in patients who underwent renal transplantation (RT), even after successful EVT. To date, there have been no data on prognosis reported following EVT for CLTI in RT recipients. We included all RT recipients who underwent EVT in our hospital between 2010 and 2022. We analyzed data from 43 limbs with ischemic ulcerations classified as Rutherford class 5 or 6, which were managed solely with EVT (i.e., no bypass surgery was performed). The primary and secondary outcomes of our study were the incidence of complete wound healing and major adverse limb events (MALE), including clinically driven target vessel revascularization, major amputation, and all-cause death. The median follow-up was 31 months. The mean age of the study population was 64.7 ± 8.7 years, with predominantly male participants (79.1%). The overall wound healing rate was 34.9%. Kaplan-Meier curve revealed that wound healing rates at 1 and 3 years were 33.6% and 40.9%, respectively. The wound healing rates of RT recipients who underwent EVT for CLTI were found to be less than satisfactory., Competing Interests: Declarations. Conflict of interest: The authors have the following disclosures: Masashi Nakao, Hiroyuki Arashi and Junichi Yamaguchi belong to a division (Clinical Research Division for Cardiovascular Catheter Intervention) that is financially maintained by donations from Abbott Vascular, Boston Scientific, Medtronic, and Terumo. The other authors have no competing interests relevant to this study., (© 2024. The Author(s) under exclusive licence to Japanese Association of Cardiovascular Intervention and Therapeutics.)

Background: Radiation ulcers that develop after breast cancer surgery are mainly repaired with pedicled flaps or free flaps rather than local random flaps or skin grafts due to large skin defects and poor wound healing. Complicated surgical techniques and donor site reconstruction increase the risk of failure. We report our experience of using the local random long neck reading man flap (LNRMF) to cover large chest wall radiation ulcers, achieving good outcomes., Methods: Eight patients who developed chest radiation ulcers after breast cancer surgery and were treated between January 2024 and June 2024 were included. The duration of the wounds ranged from 8 to 35 days. Necrotic and ischemic tissues were completely surgically removed, and subsequent wounds were repaired with LNRMFs. The wounds ranged from 6 cm × 8 cm to 15 cm × 16 cm in size. Visual analog scale scores were recorded during therapy., Results: Eight patients healed well after the first stage. The sutures were removed 10-17 days (average, 12.63 days) after surgery, and no infections, necrosis, or necrotic skin flaps were observed. The average Visual analog scale score was 2.5. The follow-up period was 2-6 months (mean, 4.7 months). Slight linear scarring was observed, and no limitations in shoulder elevation were observed in seven patients; however, 1 patient had slightly limited shoulder elevation. The average SCAR scale score was 3.88, and all sensation scores (according to the Medical Research Council scale) were S3+., Conclusions: LNRMFs are easily created and effectively repair secondary wounds that develop after the resection of radiation ulcers that develop after breast cancer surgery and large skin defects. These flaps can be widely used and should be promoted at the grassroots level., Competing Interests: Conflicts of interest and sources of funding: none declared., (Copyright © 2024 Wolters Kluwer Health, Inc. All rights reserved.)

Background and Aim: Werner syndrome (WS) is an autosomal recessive, adult-onset, progeroid syndrome caused by WRN mutations. As refractory skin ulcers significantly affect the quality of life of patients with WS, this study identified ulcer risk factors and assessed prevention methods., Methods: We analyzed the data of 51 patients with WS enrolled in the Japanese Werner Syndrome Registry between 2016 and 2022. A cross-sectional analysis was performed to determine the association with skin ulcers at baseline. Statistical analyses were conducted, including Welch's and Pearson's chi-square tests. Age was adjusted using a logistic regression model., Results: The mean patient age was 48.8±7.6 years, and 66.7% of patients presented with skin ulcers. Univariate analysis showed that patients with skin ulcers were older than those without ulcers. Systolic blood pressure (SBP) was higher in patients with skin ulcers. Patients without skin ulcers received metformin and pioglitazone treatment significantly more often than those with ulcers. Logistic regression analysis adjusted for age showed that higher SBP remained a significant risk factor for skin ulcers. Patients administered pioglitazone had lower ulcer morbidity., Conclusions: Age and SBP are risk factors for skin ulcers in patients with WS. Moreover, pioglitazone treatment may prevent skin ulcers.

Mohs' hydrophilic cream is a formulation with improved convenience by changing the base material of the conventional Mohs' paste to a hydrophilic cream. We report a case in which Mohs' hydrophilic cream was effective in treating bleeding from breast cancer with cancerous skin ulcers. The patient was a 68-year-old woman. She was admitted to our hospital because of severe anemia due to repeated bleeding from a massive right breast cancer. We used Mohs' hydrophilic cream to control bleeding. It was easy to apply the cream along the shape of the tumor, and after fixation for about 20 minutes, it was easily removed by washing with warm water. The procedure was completed within 60 minutes. After 3 times use of the cream, the surface of the tumor was fixed, and bleeding was controlled. During the treatment period, the cream could be used repeatedly without hardening. Bleeding and exudation were alleviated, so she could receive home care as she wished until she died 7 months later. Compared to the conventional Mohs' paste, Mohs' hydrophilic cream was easier to treat and dispense, just by changing of the base material, and showed good fixation comparable to the conventional paste. We concluded that Mohs' hydrophilic cream is useful for both patients and medical staff.

Eccrine porocarcinoma (EPC) is a rare skin adnexal malignancy with a high potential for metastases. The most common metastatic sites are the lymph nodes and lungs. CCutaneous metastasis is extremely rare, particularly the zosteriform variant, with fewer than 5 cases reported in the literature. Here, we report a unique case of EPC in a 71-year-old male, clinically presenting with multiple clusters of ulcerated nodules distributing as a zosteriform pattern throughout his upper left limb, along with draining lymphatic metastases and lymphedema., (© 2024 the International Society of Dermatology.)

In this CME, we review 2 specific categories of ulcers: inflammatory (where inflammation is the primary pathologic process leading to ulceration) and vaso-occlusive (where occlusion is the primary process). Inflammatory ulcers include pyoderma gangrenosum and vasculitides, whereas livedoid vasculopathy, calciphylaxis, and Martorell ulcers are vaso-occlusive ulcers. Determining the causes of ulcers in these conditions may require laboratory evaluation, biopsy, and imaging., Competing Interests: Conflicts of interest Dr Fett is on the board of the Medical Dermatology Society and serves as the education cochair for the MDS. Dr Fett is an author and editor for UpToDate vasculitis, lichen sclerosus, and pyoderma gangrenosum topics. Dr Alavi serves as the board member of HS foundation and as principal investigator for BI (Boehringer Ingelheim) and Processa and consultant for Abbvie, BI, InflaRx, Novartis, UCB. Dr Ortega is the current President of the Pacific Dermatology Association and serves as an associate editor for Dermatology (Karger). Additionally, he is a consultant for Genentech and Guidepoint and an advisor to Bristol Meyer Squibb, Boehringer Ingelheim and Janssen. Dr Ortega has received research grants from the Lilly, Janssen, Incyte and Pfizer. He is supported by NIH NIAMS R01 AR083110. The other authors have no conflicts of interests to disclose., (Copyright © 2024 American Academy of Dermatology, Inc. Published by Elsevier Inc. All rights reserved.)

In the second part of this CME, we present an approach for the management of inflammatory and vaso-occlusive ulcers and highlight the need for further research in this field. The 3 overarching principles for management are etiology-specific treatment, ulcer care, and consideration of patient comorbidities and risk factors for poor healing. Both etiology-specific treatment and management of patient comorbidities and risk factors often require collaboration with providers from other specialties. Ulcer care is governed by tissue debridement, infection control, management of moisture imbalance, and epithelial edge advancement. As wound healing is a dynamic process, management should be adapted to changes in the status of the ulcer., Competing Interests: Conflicts of interest Dr Fett is on the board of the Medical Dermatology Society and serves as the education cochair for the MDS. Dr Fett is an author and editor for UpToDate vasculitis, lichen sclerosus, and pyoderma gangrenosum topics. Dr Alavi serves as the board member of HS foundation and as principal investigator for BI (Boehringer Ingelheim) and Processa and consultant for Abbvie, BI, InflaRx, Novartis, UCB. Dr Ortega is the current President of the Pacific Dermatology Association and serves as an associate editor for Dermatology (Karger). Additionally, he is a consultant for Genentech and Guidepoint and an advisor to Bristol Meyer Squibb, Boehringer Ingelheim and Janssen. Dr Ortega has received research grants from the Lilly, Janssen, Incyte and Pfizer. He is supported by NIH NIAMS R01 AR083110. The other authors have no conflicts of interests to disclose., (Copyright © 2024 American Academy of Dermatology, Inc. Published by Elsevier Inc. All rights reserved.)

Objectives: To test the hypothesis that photographs (in addition to self-reported data) can be collected daily by patients with SSc using a smartphone app designed specifically for digital lesions, and could provide an objective outcome measure for use in clinical trials., Methods: An app was developed to collect images and patient-reported outcome measures including Pain score and the Hand Disability in Systemic Sclerosis-Digital Ulcers (HDISS-DU) questionnaire. Participants photographed their lesion(s) each day for 30 days and uploaded images to a secure repository. Lesions were analysed both manually and automatically, using a machine learning approach., Results: Twenty-five patients with SSc-related digital lesions consented, of whom 19 completed the 30-day study, with evaluable data from 27 lesions. Mean (s.d.) baseline Pain score was 5.7 (2.4) and HDISS-DU 2.2 (0.9), indicating high lesion- and disease-related morbidity. A total of 506 images were used in the analysis [mean number of used images per lesion 18.7 (s.d. 8.3)]. Mean (s.d.) manual and automated lesion areas at day 1 were 11.6 (16.0) and 13.9 (16.7) mm2, respectively. Manual area decreased by 0.08 mm2 per day (2.4 mm2 over 30 days) and automated area by 0.1 mm2 (3.0 mm2 over 30 days). Average gradients of manual and automated measurements over 30 days correlated strongly (r = 0.81). Manual measurements were on average 40% lower than automated ones, with wide limits of agreement., Conclusion: Even patients with significant hand disability were able to use the app. Automated measurement of finger lesions could be valuable as an outcome measure in clinical trials., (© The Author(s) 2024. Published by Oxford University Press on behalf of the British Society for Rheumatology.)

Martorell hypertensive ulcer (MHU) represents a painful, difficult-to-handle condition associated with peri-pheral, subcutaneous arteriolosclerosis caused by chronic hypertensive disease. Betablockers are effective for and widely used to treat hypertensive disease but are reported to exacerbate peripheral vasoconstriction. The effect of betablockers on pre-existing arteriolosclerosis and the course of MHU is, however, unknown. A retrospective study to assess the effect of betablockers on the course and response to treatment of MHU was conducted. Clinical and histopathological data were collected of patients treated for MHU at the authors' institution between 2014 and 2023 and a side-by-side comparison was performed of patients taking betablockers or not. Analysis focused on MHU severity at presentation, analgesic use, response to therapeutic intervention, and alterations of cutaneous arterioles. The study reports significantly larger ulcers and more frequent use of opioids in patients taking betablockers, while no significant difference was observed in terms of MHU response to treatment. Significantly increased luminal obstruction of peripheral cutaneous arterioles was found in patients taking beta-blockers. Based on these data, betablockers may have a negative effect on the course of MHU and should be carefully assessed in patients with MHU.

A saree is a garment worn by women in the Indian subcontinent. It is usually wrapped around the body and secured over an ankle-length skirt (a petticoat). The petticoat is usually tied very tightly around the waist with a cord. The tight cord of the petticoat often leads to dermatoses related to chronic friction and maceration, resulting in dermatitis and depigmentation. In rare cases, chronic friction may lead to the development of squamous cell carcinoma. In the literature, these skin lesions, including malignant transformation, have been described as 'saree cancers', but it is the tightness of the cord tied around the waist that results in chronic inflammation that may result in malignant transformation. We report two cases of squamous cell carcinoma in women associated with tight petticoat cord ties-'petticoat' cancer., Competing Interests: Competing interests: None declared., (© BMJ Publishing Group Limited 2024. No commercial re-use. See rights and permissions. Published by BMJ.)

Allogeneic hematopoietic stem cell transplantation (alloSCT) is the cornerstone treatment for various hematopoietic disorders, but its utility is often compromised by chronic graft-versus-host disease (cGvHD), affecting skin integrity and leading to ulcer formations. Traditional treatments, including systemic and topical therapies, frequently fail in severe cases. This study retrospectively examines three patients with therapy-resistant ulcers due to cGvHD post-alloSCT treated at the University Hospital of Regensburg in 2023. We evaluated the therapeutic impact of human amniotic membrane (hAM) transplantation-a novel approach utilizing hAM's anti-inflammatory, anti-microbial, and anti-fibrotic properties for wound healing. Surgical debridement was followed by hAM application and routine follow-up. HAM transplantation led to complete wound closure in two out of three patients and a significant reduction in local pain and infection rates. The treatment alleviated the need for regular dressing changes within three months in two patients, demonstrating the hAM's efficacy in fostering rapid and sustained healing. The utilization of hAM represents a promising alternative for the management of refractory skin ulcers in cGvHD patients, particularly when conventional methods are inadequate., (© 2024. The Author(s).)

Objective: Cutaneous ulcers induced by metastasis present a challenging clinical issue, often resistant to conventional dressing change interventions. In this case, we demonstrates the efficacy of Traditional Chinese Medicine in managing ruptured tumors., Case Presentation: We present a case study of a 40-year-old Chinese male patient diagnosed with advanced tonsillar squamous cell carcinoma with cervical lymph node metastasis and cutaneous ulceration. Despite nearly six months of conventional dressing treatment yielding minimal improvement, significant healing of the ulcerated surface of the neck metastasis was observed after approximately two months of decoction therapy. The cancerous lesion exhibited a reduction of nearly 90 %, with concomitant alleviation of secondly symptoms such as cold intolerance, excessive sweating, anxiety and depression., Conclusions: In this case, we introduces Traditional Chinese Medicine may be an effective alternative therapy for the treatment of cancer-related disease. And it may also be an optional therapeutic approach for healthcare practitioners with cutaneous carcinoma ulceration., (Copyright © 2024 The Author(s). Published by Elsevier Inc. All rights reserved.)

We report an unusual presentation of Cytomegalovirus (CMV) cutaneous perianal ulcerative lesion in a patient with severe immunosuppression. A 43-year-old male presented with perianal ulcer along with bleeding and pain while passing stools. On biopsy, the ulcer showed typical histopathological features of CMV infection with involvement of endothelial cells. The patient was treated with oral valganciclovir for CMV ulcer which showed significant improvement subsequently. After 2 weeks, antiretroviral therapy (ART) was reinitiated with Tenofovir disoproxil fumarate, Lamivudine, and Dolutegravir. A high index of suspicion should always be maintained for CMV cutaneous ulcers in individuals with advanced immunosuppression., (© Journal of the Association of Physicians of India 2024.)

Background and Objectives: Diagnosis and therapy of peristomal skin lesions are challenging for the majority of therapists. Established diagnostic tools have not yet been validated. Our objective was to outline the spectrum of and to identify risk factors for skin lesions in ostomy patients. A focus was set on peristomal ulcerations and their differentiation as peristomal pyoderma gangrenosum., Methods: In a retrospective analysis, frequency and character of peristomal skin lesions in patients presenting at two departments were analysed. Patients suffering from peristomal ulcerations were subjected to a more detailed analysis including application of the PARACELSUS score., Results: A total of 565 patients with ostomy were analysed; 40.2% (n = 227) presented with peristomal skin lesions. Moisture-associated skin damage (27.9%) was the most common, while ulcerations (21.9%) and eczematous skin alterations (19.1%) were seen with comparable frequency. Peristomal pyoderma gangrenosum was diagnosed in 7.9% of all observed peristomal lesions. Among patients with inflammatory bowel disease (n = 98), peristomal ulcerations were the leading finding (35.7%), and pyoderma gangrenosum was more frequent in these patients (16.3%)., Conclusions: It is necessary to assess and classify peristomal skin lesions. Further studies for the validation of different scores or the development of diagnostic tools are needed., Competing Interests: The authors declare that they have no conflict of interest., (Thieme. All rights reserved.)

A 44-year-old male presented with a 2-month history of erythematous ulcerative papules and plaques on the scalp, face, and bilateral lower legs. He had a 5-year history of well-controlled HIV on antiretroviral therapy and recurrent syphilis infections. His face had violaceous plaques, while bilateral ankles and calves had ulcerative lesions with necrotic centers and purple borders. The morphologies clinically mimicked pyoderma gangrenosum on the lower extremities and cutaneous lymphoma on the face. Biopsy and reactive rapid plasma reagin confirmed a diagnosis of lues maligna, and the patient was successfully treated with penicillin G benzathine., (© 2024 The Author(s). International Journal of Dermatology published by Wiley Periodicals LLC on behalf of the International Society of Dermatology.)

Scleroderma or systemic sclerosis (SSc)-associated digital ischaemic complications, such as digital ulcers (SSc-DUs), appear relatively early during the disease course and are a major burden with substantial deterioration of quality of life. Expert rheumatologist and wound specialists have defined a DU; however, international application of the definition is still disorganised. Appearance of SSc-DUs is secondary to the onset of Raynaud's phenomenon and as a consequence, recommended first-line of treatment mainly includes vasodilators; however, many DUs are refractory to this treatment. Despite important practical issues, such as a lack of well-characterised SSc-wound healing animal model, significant efforts are needed to mechanistically understand the pathogenesis of SSc-DUs for developing clinically targetable disease modifying therapies., (© 2024 The Wound Healing Society.)

Introduction: Trigeminal trophic syndrome causes self-injurious lesions secondary to cutaneous dysesthesia following damage of the trigeminal nerve. A similar syndrome, greater auricular trophic syndrome, can result following sacrifice of the greater auricular nerve during a parotidectomy., Case Presentation: A 59-year-old woman presented with ulceration and crusting of her right ear 5 months after parotidectomy. She was determined to have greater auricular trophic syndrome with prurigo nodularis-like histopathologic changes and was successfully treated with topical clobetasol, occlusive dressing, and behavioral modification., Discussion: Six similar cases have been reported. Mental health disorders were noted in 4 of these cases, and treatment focused on managing psychiatric comorbidities. This case considers treatment of a patient without a prior mental health disorder., Conclusions: Greater auricular trophic syndrome is a rare complication following a parotidectomy. Occlusive dressing and behavioral modification led to subsequent improvement of the ulceration for this patient., (Copyright© Board of Regents of the University of Wisconsin System and The Medical College of Wisconsin, Inc.)

BACKGROUND Giant cell tumors of bone typically occur in early adulthood, when the growth plate has closed. The distal radius is the second most common location affected, accounting for 10% to 12% of cases. Complications of poor soft tissue healing are rare in the distal radius, owing to its rich blood supply. However, the curettage procedure and use of bone cement and external fixation can affect the local blood supply. CASE REPORT We present a rare case of a 24-year-old woman with no significant medical history who underwent surgery at a local hospital to treat a giant cell tumor of the radius. During postoperative wound dressing changes, a 4×3-cm area of flushed skin color with a small blister and reduced local sensation was found on the dorsal side of the wrist. The skin condition worsened despite treatment at the surgical outpatient clinic, leading to referral to scar specialist outpatient treatment. Examination revealed a well-healed surgical scar on the palmar side of the wrist, but a skin defect with necrotic tissue and tendon exposure on the dorsal side. The diagnosis was postoperative soft tissue necrosis of the skin with a giant cell tumor of the bone. CONCLUSIONS This case report discusses the management of chronic non-healing postoperative wounds in giant cell tumors of the distal radius. It emphasizes the importance of appropriate dressing changes, selecting suitable dressings, nutritional support, and effective nurse-patient communication. The case serves as an example of best practices for managing these types of wounds.

Patients with granulomatosis with polyangiitis occasionally present with cutaneous manifestations, which are important clues for the early diagnosis. Although pyoderma gangrenosum-like ulcers are rarely observed, a unique case with unusual clinical features is presented herein. A 75-year-old woman with positive proteinase 3-antineutrophil cytoplasmic antibody (PR3-ANCA) repeatedly developed aseptic abscesses on the abdomen, buttock, lower legs, and forearms. Histopathological features of biopsy taken from ulcers showed necrotizing granulomatous inflammation with multinucleated giant cells without leukocytoclastic vasculitis. The present case was initially diagnosed as limited granulomatosis with polyangiitis without renal and lung involvement. However, two years later, she died of cerebral hemorrhage.

Radiation-induced skin ulcer following cancer and/or tumour is well-documented in the literature. However, radiation-induced skin ulcer following the excision of keloid is yet to be reported. Here, we report the case of a 33-year-old female patient with a suprapubic skin ulcer of five months' duration following keloid treatment with electron beam therapy at recommended dosage. Various examinations, including a skin biopsy, metagenomic sequencing, magnetic resonance imaging and immunochemistry, indicated that the skin ulcer was induced by radiotherapy. While postoperative radiotherapy has been recommended immediately following keloid excision to reduce the risk of recurrence, the present case highlights the risk of skin refractory ulcer following keloid radiotherapy.

Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare hematologic malignancy that can manifest with skin nodules and erythematous plaques. In most cases BPDCN progresses rapidly, causing multiple skin lesions and also affecting internal organs and bone marrow, warranting initiation of systemic therapies or hematopoietic stem cell transplantation (HCT). Although not curative, radiotherapy for isolated lesions might be indicated in case of (imminent) ulceration and large or symptomatic lesions. To this end, doses of 27.0-51.0 Gy have been reported. Here, we present the case of an 80-year-old male with BPDCN with multiple large, nodular, and ulcerating lesions of the thorax, abdomen, and face. Low-dose radiotherapy of 2 × 4.0 Gy was administered to several lesions, which resolved completely within 1 week with only light residual hyperpigmentation of the skin in affected areas and reliably prevented further ulceration. Radiotoxicity was not reported. Therefore, low-dose radiotherapy can be an effective and low-key treatment in selected cases of BPDCN, especially in a palliative setting, with a favorable toxicity profile., (© 2024. The Author(s).)

Background: Tumors indicating the advanced stage of mycosis fungoides (MF) have a rich clinical spectrum. Although it is known that the prognosis of MF generally worsens following the development of tumors, some cases may have a relatively indolent course, and the role of clinical characteristics regarding prognosis has still not been well understood., Methods: MF patients were retrospectively evaluated regarding the development of tumors. Besides demographic characteristics, data of the subtype and stage of the disease were recorded. The clinical features of tumors, including number (<5, 5-10, 11-20, or >20), location, dimension (diameter of ≥5 cm), presence of ulceration, and surrounding inflammation, were noted. Univariate and multivariate analyses evaluated the relationship between overall survival (OS) with demographic and clinical features., Results: Among 730 consecutive MF patients, tumors developed in 8.2% (n = 60), of whom 46.7% were diagnosed with advanced-stage MF from the beginning. The most common subtype was folliculotropic MF (53.3%). Most patients (55%) had multiple tumors, and the most frequent localization was the trunk (71.7%). Most tumors presented as smooth-surfaced, indurated papules and/or nodules (70%), while others were reddish-purple, occasionally accompanied by ulceration (50%), perilesional inflammation (23.3%), and attaining large dimensions (25%). Mortality was recorded in 51.7% of patients, and the 5-year OS rate from the diagnosis of tumors was 49%. Independent poor prognostic factors for OS in multivariate analysis included older age at the time of diagnosis, presence of tumors at the initial MF diagnosis, presence of over 20 tumors, and the existence of large tumors., Conclusions: Tumoral MF seen in older patients, the first diagnosis of MF in this stage, presenting with generalized and large tumors, seems to be a predictive factor for OS., (© 2024 the International Society of Dermatology.)

As the clinical course of systemic sclerosis (SSc) varies widely, prognostic indicators have been sought to predict the outcomes of individual patients. Racial differences in SSc render it necessary to validate prognostic indicators in different patient cohorts. In this study, we aimed to assess clinical and laboratory parameters in Japanese patients with early-stage SSc with diffuse cutaneous involvement and/or interstitial lung disease, and identify predictive factors for disease progression. We performed multivariate analyses of baseline clinical information to estimate symptoms 4 years later in Japanese patients with diffuse cutaneous SSc and/or SSc with interstitial lung disease. Patients were enrolled in the study within 5 years of disease onset at 10 Japanese SSc centers. Over 12 years, 115 patients followed up for 4 years were included in this study. The modified Rodnan skin score (mRSS) at 4 years correlated with the baseline mRSS and finger-to-palm distance, defined as the average length from the distal tip of the fourth finger to the distal palmar crease. The percentage predicted vital capacity (%VC) in year 4 positively and negatively correlated with initial %VC and the presence of anti-topoisomerase I antibodies, respectively. The Health Assessment Questionnaire Disability Index (HAQ-DI) at 4 years was positively and negatively associated with baseline HAQ-DI and %VC, respectively. The occurrence of digital ulcers within 4 years was associated with the initial presence of digital ulcers, finger-to-palm distance, and the presence of digital pitting scars and anti-topoisomerase I antibodies. This study identified several factors that may predict the progression of early-stage SSc in Japanese patients. Finger-to-palm distance may be a useful tool for predicting the progression of skin thickening and the development of digital ulcers in the early stages of severe SSc, but larger, long-term prospective studies are needed to confirm our findings., (© 2024 The Author(s). The Journal of Dermatology published by John Wiley & Sons Australia, Ltd on behalf of Japanese Dermatological Association.)

Competing Interests: Competing interests: None declared.

While ulceration is one of the most common infantile hemangioma (IH) complications, severe bleeding is a rare consequence, with a paucity of patients reported. We report a 5-month-old girl with a very large, mixed, partial segmental IH of the upper chest wall who, despite medical intervention, developed severe ulceration and multiple episodes of life-threatening bleeding that ultimately led to hemorrhagic shock. Experience in our patient and a review of six previous reports shows that severe bleeding is a risk when ulceration extends directly into an arterial feeding vessel that is often visible clinically. Other potential predictors for severe bleeding include large to very large IH size with extension of the tumor into underlying structures, segmental or partial segmental patterning, mixed and bulky morphology, and white discoloration as a sign of impending or worsening ulceration., (© 2024 Wiley Periodicals LLC.)

Aims: To describe the demographic characteristics of heroin and cocaine injectors with chronic injection-related trophic disorders, as well as the clinical and progressive characteristics of these disorders., Methods: A descriptive, observational, multicenter and retrospective study over the last 15 years. Patients were recruited via a call for cases and by consulting the health data warehouse of the university hospital center., Results: The population comprised 39 injection drug users, of whom 79.5% were male, with a median age of 41 years. Subjects had numerous co-addictions and 70.5% were infected with hepatitis C virus. Trophic disorders were multiple in some cases: 43.5% of patients had lymphoedema, 87% had ulcers, and 56.5% had injection-related scars. Ulcers were multiple, large, and present for a median of 3 years. They were located on the upper limbs in 32.5% of cases. Ulcers constituted a source of complications in 64.5% of cases and these were infectious in 91% of cases (local, osteoarticular or systemic). During follow-up, 8 patients died and 21.5% of patients requiring ulcer care were lost to follow-up., Conclusions: This study showed a high rate of complications, particularly infections, of ulcers in injection drug users. Localization of these ulcers to the upper limbs, although rare in the general population, is relatively frequent in this population. Follow-up is difficult and cooperation between dermatologist and addictologist is essential to improve patient care., Competing Interests: Declaration of competing interest The authors declare the following financial interests/personal relationships which may be considered as potential competing interests: [Olivier Cottencin has acted as a chairman or speaker of symposia organized by Janssen, Indivior, and Recordati.]., (Copyright © 2024 Elsevier Masson SAS. All rights reserved.)

Background: Systemic sclerosis (SSc) is a complex autoimmune connective-tissue disease, characterised by vasculopathy and fibrosis of the skin and internal organs. Activation of microvascular endothelial cells (ECs) causes the intimal hyperplasia that characterises the vascular remodelling in SSc. The most frequent complication of SSc is the development of digital ulcers (DUs). Thymic stromal lymphopoietin (TSLP) may trigger fibrosis and sustain vascular damage. Aim of this study was to evaluate the correlation between serum level of TSLP and DUs., Methods: 75 consecutive SSc patients were enrolled and serum TSLP levels were measured. The presence of history of DUs (HDU) was evaluated. Recurrent new DUs were defined as the presence of at least 3 episodes of DUs in a 12-months follow up period. The risk of developing new DUs was calculated by applying the capillaroscopic skin ulcer risk index (CSURI)., Results: The median value of TSLP was higher in patients with HDU than patients without HDU [181.67 pg/ml (IQR 144.67; 265.66) vs 154.67 pg/ml (IQR 110.67; 171.33), p < 0.01]. The median value of TSLP was higher in patients with an increased CSURI index than patients without an increased CSURI [188 pg/ml (IQR 171.33; 246.33) vs 159.33 pg/ml (IQR 128.67; 218), p < 0.01]. Kaplan-Meier curves demonstrated that free survival from new DUs was significantly (p < 0.01) lower in SSc patients with increased TSLP serum levels., Conclusion: TSLP might have a key role in digital microvascular damage of SSc patients., Competing Interests: Declaration of competing interest All the authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest., (Copyright © 2024 The Authors. Published by Elsevier Inc. All rights reserved.)

Objective: Raynaud phenomenon (RP) and digital ulcers (DUs) are the main signs of digital vasculopathy in systemic sclerosis (SSc). Selexipag is an oral prostacyclin agonist approved for SSc-related pulmonary arterial hypertension. Following our previous preliminary short-course report, we herein present long-term data on selexipag safety and efficacy in the treatment of SSc digital vasculopathy., Methods: Selexipag was administered to patients with SSc with severe digital vasculopathy refractory or with contraindication to all other vasoactive therapies. Each subject was assessed at baseline and after 3, 6, and 12 months. Clinical outcomes related to RP and DUs were evaluated along with modified Rodnan skin score of the fingers. Digital perfusion was assessed by laser speckle contrast analysis (LASCA). Nailfold videocapillaroscopy (NVC) was also performed., Results: Eight patients with SSc (63% female, mean age 50.1 years) received selexipag. After 12 months of treatment, RP was reported to significantly decrease in the number of daily episodes and mean duration ( P < 0.001 and P = 0.01, respectively). All patients achieved a complete healing of their DUs ( P = 0.03) within 6 months. A progressive reduction of fingers skin score was observed ( P = 0.03). No structural changes of capillaries were noted on NVC. Conversely, LASCA revealed an important increase in total digital perfusion ( P = 0.004) despite seasonal variability. The safety profile was consistent with that reported in the literature., Conclusion: We observed a sustained efficacy of selexipag on SSc digital vasculopathy during 1 year of administration. Our promising results encourage the design of a new randomized controlled trial to evaluate the effect of selexipag on SSc digital vasculopathy., (Copyright © 2024 by the Journal of Rheumatology.)

Sarcoidosis is a disease characterized by immunological granuloma formation in various organs. Cutaneous manifestations occur in about 25% of patients with rare cases showing ulcerative sarcoidosis, that can be debilitating if not treated promptly. This article presents a patient with isolated ulcerative sarcoidosis and reviews existing literature. A 44-year-old woman presented with a non-healing ulcer on her right gluteal area. A skin biopsy confirmed sarcoidosis with non-necrotizing granulomas. Systemic involvement of sarcoidosis was ruled out. Treatment involved topical corticosteroids and intralesional corticosteroid injections, resulting in complete healing. This case emphasizes the importance of considering ulcerative sarcoidosis in non-healing wounds and the efficacy of corticosteroid treatment.

Objectives: Systemic sclerosis (SSc) is a chronic multisystem disease characterised by microcirculatory vascular dysfunction and progressive fibrosis of the skin and internal organs. Interleukin-6 (IL-6) is a proinflammatory cytokine that has been implicated in the pathogenesis of several autoimmune diseases and in the initiation and progression of the cardiovascular disease. In the present work we aimed to study the relationship of IL-6 with clinical manifestations and the cardiovascular risk in patients with SSc., Methods: We carried out a cross-sectional study that included 53 individuals with SSc. A multivariate analysis was performed to study the relationship between IL-6 and disease characteristics and cardiovascular risk assessed by Systematic Coronary Risk Estimation (SCORE2) in SSc., Results: The presence of digital ulcers, calcinosis, and anti-Scl70 antibody was associated with higher levels of IL-6. This was also the case for functional respiratory parameters where this association was found to be significant and negative after correction for covariates. In addition, the SCORE2 cardiovascular risk algorithm showed a positive and significant association with circulating IL-6., Conclusions: IL-6 levels are associated with disease manifestations and cardiovascular risk in patients with SSc.

Systemic sclerosis (SSc) is an autoimmune disease characterized by vasculopathy, aberrant immune activation, and extensive tissue fibrosis of the skin and internal organs. Because of the complicated nature of its pathogenesis, the underlying mechanisms of SSc remain incompletely understood. Angiogenic factor with a G-patch domain and a Forkhead-associated domain 1 (AGGF1) is a critical factor in angiogenesis expressed on vascular endothelial cells, associated with inflammatory and fibrotic responses. To elucidate the possible implication of AGGF1 in SSc pathogenesis, we investigated the association between serum AGGF1 levels and clinical manifestations in SSc patients. We conducted a cross-sectional analysis of AGGF1 levels in sera from 60 SSc patients and 19 healthy controls with enzyme-linked immunosorbent assay. Serum AGGF1 levels in SSc patients were significantly higher than those in healthy individuals. In particular, diffuse cutaneous SSc patients with shorter disease duration had higher levels compared to those with longer disease duration and limited cutaneous SSc patients. Patients with higher serum AGGF1 levels had a higher incidence of digital ulcers, higher modified Rodnan Skin Scores (mRSS), elevated serum Krebs von den Lungen-6 (KL-6) levels, C-reactive protein levels, and right ventricular systolic pressures (RVSP) on the echocardiogram, whereas they had reduced percentage of vital capacity (%VC) and percentage of diffusing capacity of the lungs for carbon monoxide (%DLCO) in pulmonary functional tests. In line, serum AGGF1 levels were significantly correlated with mRSS, serum KL-6 and surfactant protein D levels, RVSP, and %DLCO. These results uncovered notable correlations between serum AGGF1 levels and key cutaneous and vascular involvements in SSc, suggesting potential roles of AGGF1 in SSc pathogenesis., (© 2024 Japanese Dermatological Association.)

Abstract: Skin complications in individuals with an ostomy are widely reported and can cause physical and emotional challenges in everyday life. Chronic parastomal skin complications can be difficult to heal and cause significant pain.Two patients presented to the stomal therapy clinic for treatment and were diagnosed with chronic parastomal skin ulceration. Following standard treatment of wound management, topical corticosteroid ointment, and appliance review, the ulcers either were not improving or had reoccurred. Treatment with a combination cream consisting of 0.2% hyaluronic acid and 1% silver sulfadiazine was initiated, and both patients demonstrated complete healing.Treatment of parastomal skin ulceration with dual-action cream 0.2% hyaluronic acid and 1% silver sulfadiazine was successful for these two patients, with a reduction in pain and purulent fluid noted throughout treatment, in addition to a reduced cost of treatment when compared with standard protocols., (Copyright © 2024 Wolters Kluwer Health, Inc. All rights reserved.)

Background: Trigeminal trophic syndrome is a rare cranial and facial condition caused by damage to the central or peripheral branches of the trigeminal nerve. This syndrome consists of a triad of anesthesia, paresthesia, and crescent-shaped facial ulcer involving the ala nasi and sometimes extending to the upper lip. Although previous screening for human immunodeficiency virus in some patients with trigeminal trophic syndrome was negative, we present a unique case of trigeminal trophic syndrome who tested positive for human immunodeficiency virus with eye complications., Case Presentation: We present a rare case of trigeminal trophic syndrome in a 44-year-old Black African woman who tested positive for human immunodeficiency virus. She presented with a 6-week history of progressive, persistent, and painless left sided facial and scalp ulcerations that started as small skin erosion. Diagnosis of trigeminal trophic syndrome was made on clinical grounds based on the triad of anesthesia, paresthesia, and unilateral crescent-shaped ulcer in the trigeminal dermatome and her past medical history. The ulcer healed completely after counseling and pharmacological therapy, but she later developed left periorbital cellulitis and left upper eyelid full-thickness defect., Conclusion: This is by far the first documented case of trigeminal trophic syndrome with a positive human immunodeficiency virus test. Testing for human immunodeficiency virus in patients with trigeminal trophic syndrome is necessary as this can help improve clinical management and treatment outcomes. Seeking the services of specialists remotely in resource constraint settings is beneficial for managing complications associated with trigeminal trophic syndrome., (© 2024. The Author(s).)

Background: Systemic sclerosis (SSc) is a rare chronic autoimmune disease with heterogeneous manifestations. In the last decade, several clinical trials have been conducted to evaluate new treatment options for SSc. The purpose of this work is to update the recommendations of the Brazilian Society of Rheumatology in light of the new evidence available for the pharmacological management of SSc., Methods: A systematic review including randomized clinical trials (RCTs) for predefined questions that were elaborated according to the Patient/Population, Intervention, Comparison, and Outcomes (PICO) strategy was conducted. The rating of the available evidence was performed according to the Grading of Recommendations Assessment, Development and Evaluation (GRADE) methodology. To become a recommendation, at least 75% agreement of the voting panel was needed., Results: Six recommendations were elaborated regarding the pharmacological treatment of Raynaud's phenomenon, the treatment (healing) and prevention of digital ulcers, skin involvement, interstitial lung disease (ILD) and gastrointestinal involvement in SSc patients based on results available from RCTs. New drugs, such as rituximab, were included as therapeutic options for skin involvement, and rituximab, tocilizumab and nintedanib were included as therapeutic options for ILD. Recommendations for the pharmacological treatment of scleroderma renal crisis and musculoskeletal involvement were elaborated based on the expert opinion of the voting panel, as no placebo-controlled RCTs were found., Conclusion: These guidelines updated and incorporated new treatment options for the management of SSc based on evidence from the literature and expert opinion regarding SSc, providing support for decision-making in clinical practice., (© 2024. The Author(s).)

Abstract: Clinically amyopathic dermatomyositis (CADM) is a rare subtype of dermatomyositis that presents with cutaneous features and no muscle involvement. This case report describes a 26-year-old woman with recurrent and multiple digital ulcerations coinciding with the start of winter each year. There was no evidence of myopathy, and antibody testing yielded negative results. A diagnosis of CADM was ultimately made based on clinicopathologic correlation. The patient's ulcers demonstrated excellent response to a combination therapy of hydroxychloroquine and potent topical and systemic steroids. Herein, the authors discuss the pathologic and immunologic characteristics of CADM., (Copyright © 2024 Wolters Kluwer Health, Inc. All rights reserved.)