Retiform purpura (RP) is a morphological pattern of cutaneous ischemia resulting from vascular compromise. Its clinical relevance lies in its orientation toward vasculitis or thrombotic vasculopathy. We report the case of a woman with painful violaceous retiform lesions on the distal lower extremities, Raynaud's phenomenon with a "blue toe" on the hand, and polyarthralgias. Laboratory workup revealed antinuclear antibodies (ANA) 1:2560 (AC-5 pattern), anti-RNP > 240 U/mL, anti-Sm > 320 U/mL, low C4, and negative antineutrophil antibodies (ANCA) and rheumatoid factor. Magnetic resonance imaging showed diffuse muscular edema and bilateral fascial thickening; computed tomography reported reactive adenopathies. Skin biopsy demonstrated ischemic changes consistent with small vessel involvement in a mixed vasculitic/vasculopathic pattern, with epidermal necrosis and intraluminal fibrin. Systemic lupus erythematosus (SLE) with features of small vessel vasculitis was diagnosed. Treatment included methylprednisolone and prednisone pulses, cyclophosphamide, rituximab, and alprostadil for digital ischemia, with clinical improvement. RP may reflect a mixed inflammatory/thrombotic process in the context of overlapping autoimmunity. Deep biopsy, autoimmune profiling, and assessment of prothrombotic state are key axes for decisions regarding immunosuppression and/or antithrombotic strategies. Key Points • Retiform purpura is a high-risk cutaneous pattern that requires early differentiation between vasculitis and thrombotic vasculopathy, as each condition demands divergent therapeutic strategies (immunosuppression versus antithrombotic therapy). • In patients with SLE, cutaneous vasculitis - though uncommon - may present with a dominant ischemic phenotype (retiform purpura, digital ischemia) that initially mimics medium-vessel vasculitis such as polyarteritis nodosa or systemic sclerosis; accurate diagnosis requires integration of clinical, histological, and immunological data. • Management of severe ischemic SLE with a mixed inflammatory/occlusive mechanism may require a parallel approach: intensive immunosuppression to control vascular inflammation, combined with vasoactive and anticoagulant measures to preserve distal perfusion and tissue viability.

Secondary Raynaud's Phenomenon (RP) associated with connective tissue diseases (CTD) frequently presents as critical digital ischemia refractory to standard protocols. This review describes the clinical feasibility of a combined approach involving mechanical (surgical adventitial stripping) and chemical (Botulinum Toxin Type A [BTX-A] and/or prostanoid analogues) sympathectomy. We retrospectively reviewed four patients from tertiary centers in Mexico and Argentina presenting with severe refractory RP. Interventions involved periarterial digital sympathectomy augmented by intradigital BTX-A and/or intravenous (IV) prostanoids. Longitudinal clinical observations were recorded regarding pain reduction using the Visual Analog Scale (VAS), digital ulcer healing, and vascular imaging. Clinical remission was achieved in 3/4 patients. Case one healed a 0.5 cm necrotic lesion following bilateral stripping and 80 IU BTX-A. Case two, achieved complete tissue stabilization at 16-month follow-up; intraoperative angiography confirmed the immediate restoration of digital arch perfusion post-intervention. Case three, presented with a "late" capillaroscopy pattern, maintained a 60-month ulcer-free interval using 80 IU BTX-A and Alprostadil. Case four involved 8/10 ischemic digits; the course was complicated by a radial artery thrombosis requiring a radio-radial venous graft bypass. This patient suffered a relapse with three new distal ulcers immediately following an administrative gap in Bosentan therapy. These hypothesis-generating findings suggest that multimodal sympathectomy is a feasible adjunctive strategy for limb salvage in refractory complicated secondary RP. Current literature enforces these findings supporting the notion for standardized dosing and stepwise approach for future research to support these hypothesis generating views.

The inhibition of the checkpoint receptor-ligand system has become a therapeutic target in the treatment of solid malignancies in immuno-oncology. Increased attention has been given to immune-related adverse events (irAEs) associated with the use of checkpoint inhibitors. Multiple irAEs may occur in a single patient. Here, we report a unique case in which Raynaud's syndrome was associated with two other autoimmune complications, hypothyroidism and pneumonitis. The patient was a 48-year-old Caucasian man whose complaints started with a sore throat. Ultrasound and CT revealed left cervical lymph node enlargement. Aspiration cytology showed infiltrating human papillomavirus (HPV)-positive squamous cell cancer. As the outcome of surgery was uncertain, neoadjuvant chemotherapy and irradiation were successfully applied. Later, CT revealed tumor relapse. Nivolumab therapy was initiated, and a total of 21 cycles were administered to the patient. Three months after the initiation of nivolumab, new-onset Raynaud's phenomenon (RP) developed. No systemic connective tissue disease could be identified. RP improved following oral pentoxifylline treatment. Later, hypothyroidism and pneumonitis developed, both of which were easily controlled. Eventually, significant tumor progression occurred despite nivolumab treatment, and Cetuximab was administered; however, the patient died. It has been established that if two or more irAEs develop in the same patient, one of these irAEs is more likely to be autoimmune rheumatic in nature. It is very important to detect these side effects in time; physicians must be familiar with their appropriate treatment because all side effects may be essential to the patient's survival.

Mixed connective tissue disease (MCTD) is an autoimmune disorder characterized by overlapping features of multiple connective tissue diseases in addition to anti-U1-ribonucleoprotein (RNP) antibodies and Raynaud's phenomenon. A 53-year-old female patient with MCTD, interstitial pneumonia, and idiopathic thrombocytopenic purpura underwent partial tongue resection under general anesthesia. Perioperative management included steroid coverage and careful titration of the oxygen concentration. Although venous access was difficult, the anesthetic course was uneventful. MCTD can present perioperative challenges including pulmonary and cardiac complications, effects of long-term steroid use, and airway and cardiovascular disturbances. Safe anesthesia for patients with MCTD requires assessment of disease activity and severity across multiple conditions and individualized management accordingly.

ObjectiveTo describe the prevalence, clinical manifestations, histopathological features, and antibody profile associated to skin involvement in patients with catastrophic antiphospholipid syndrome (CAPS).MethodsWe performed a cross-sectional study of the patients included in the "CAPS Registry," a registry developed by the European Forum on Antiphospholipid Antibodies (aPL)1,2 This database contains data from patients with CAPS collected from April 1992 to December 2024. Demographic clinical manifestations, laboratory features (including aPL antibodies), pathological findings in involved skin, and outcome were retrieved.ResultsCutaneous involvement was described in 377 episodes (47%) from 361 patients. A female sex prevalence of 49.4% was observed. The average age was 39.4 (SD ± 17.9) years. The more frequent skin manifestations observed were livedo reticularis (17.6%), skin necrosis (13%), ischemic ulcers (10.4%), skin ischemic (9.7%), skin purpura (7.1%), gangrene (5%), splinter haemorrhages (2.6%), and Raynaud's phenomenon (1.5%). The most frequently affected organs in CAPS episodes with skin involvement were kidneys (72%), lungs (61%), central nervous system (54.5%), and heart (54.4%). Thrombocytopenia and hemolysis features were more common in patients with skin involvement (p < 0.05 for all). Thrombotic microangiopathy was the predominant pathological finding, present in 82 (68.3%) episodes. No difference regarding mortality were found in episodes with or without skin involvement (29.3% vs 32.1%, p = 0.42).ConclusionThe presence of skin involvement in patients with CAPS is frequent. The most frequent manifestations were livedo reticularis and skin necrosis. Skin involvement does not seem to be associated to mortality.

Raynaud's phenomenon (RP) is a common vasospastic disorder characterized by transient digital ischemia triggered by cold exposure or emotional stress. It is classified as primary or secondary, the latter often associated with autoimmune connective tissue diseases such as systemic sclerosis, mixed connective tissue disease, and Sjögren's disease /autoimmune epithelitis (SjD/AE). Although primary RP is typically benign, some patients progress to secondary forms, highlighting the need for careful follow-up. RP pathogenesis reflects interactions between vascular, neural, and immune mechanisms. Increased sensitivity of α2-adrenergic receptors, heightened sympathetic activity, and endothelial dysfunction drive exaggerated vasoconstriction. Emerging evidence also supports an immune-mediated component, with functional autoantibodies targeting G protein-coupled receptors that may alter vascular tone. Genetic studies implicate adrenergic and immune pathways in susceptibility. Nailfold capillaroscopy aids early diagnosis and risk stratification. Management includes lifestyle measures and vasodilators such as calcium channel blockers and phosphodiesterase-5 inhibitors, while botulinum toxin A shows promise.

Autoimmune/inflammatory syndrome induced by adjuvants (ASIA) encompasses a spectrum of immune-mediated clinical conditions triggered by exposure to adjuvant substances such as aluminum, vaccine components, and silicone breast implants (SBIs). We present the case of a 68-year-old woman with a history of silicone breast augmentation, followed by implant rupture and reimplantation. Years later, she developed Raynaud's phenomenon, cutaneous calcinosis, and sicca symptoms. The patient also exhibited cognitive impairment and depressive symptoms. Laboratory investigations confirmed the presence of anti-centromere antibodies and elevated acute-phase reactants. Nailfold capillaroscopy demonstrated an early scleroderma pattern. Imaging revealed silicone migration into axillary lymph nodes, histopathologically consistent with siliconomas. A diagnosis of limited cutaneous systemic sclerosis was made. Following implant removal and the excision of silicone-laden lymph nodes, the patient experienced significant improvement in systemic, cutaneous, and neuropsychiatric symptoms, with subsequent normalization of her autoimmune profile, supporting the diagnosis of ASIA. While the relationship between SBIs and systemic autoimmune manifestations remains controversial, this case highlights the broad clinical spectrum of ASIA and underscores the importance of clinical suspicion, multidisciplinary evaluation, and individualized therapeutic strategies in patients with unexplained autoimmune manifestations and a history of adjuvant exposure.

We report a 64-year-old male with rheumatoid arthritis and Raynaud's phenomenon who presented with diffuse arthralgias and progressive ulcerating lesions involving the palms and elbows. Differential diagnoses included recurrent Rickettsial infection, vasculitis, pyoderma gangrenosum, and cutaneous tuberculosis. Laboratory studies demonstrated marked leukocytosis (135.4 ×10⁹/L), while computed tomography (CT) imaging revealed hepatomegaly, lymphadenopathy, and pulmonary micronodules concerning for infiltrative disease. Additional history revealed a 40-pound unintentional weight loss over three months. Peripheral flow cytometry established a new diagnosis of chronic lymphocytic leukemia (CLL), and skin punch biopsy demonstrated epidermal necrosis with dense dermal lymphoid infiltrates consistent with leukemia cutis. This case highlights leukemia cutis as a rare initial manifestation of CLL that may mimic infectious or autoimmune conditions, emphasizing the importance of thorough evaluation of unexplained cutaneous lesions and significant leukocytosis to facilitate earlier diagnosis and treatment consideration.

The Scleroderma Clinical Trials Consortium (SCTC) is committed to advancing the understanding and treatment of systemic sclerosis (SSc). This review focuses on the development and validation of various outcome instruments by the SCTC, which are crucial for evaluating the effectiveness of interventions in clinical trials and observational studies. Key instruments discussed include the Assessment of Systemic Sclerosis-Associated RAynaud's Phenomenon Questionnaire, the SCTC Radiologic Scoring System for calcinosis, the Mawdsley Questionnaire for calcinosis, the University of California Los Angeles SCTC Gastrointestinal Tract Instrument (GIT) 2.0, the SCTC Activity Index and the SCTC Damage Index. Additionally, the SCTC and World Scleroderma Foundation (WSF) Skin Scoring Certification is highlighted as an important resource for researchers. Each instrument's purpose, description and validation process are examined, underscoring their pivotal role in advancing SSc research and improving patient outcomes.