Cutaneous Vasculitis Research Articles

P55 The boggy uveitis

Abstract Introduction Blau syndrome is a rare autosomal dominant autoinflammatory disease that affects young children. Typically, it causes widespread granulomatous inflammation, causing uveitis, arthritis, and dermatitis. The triad may be characteristic of the disease but is not present in all cases and may occasionally be incomplete. A characteristic finding is the presence of boggy swellings in the involved joints. Our patient presented with uveitis and boggy swellings of the wrists without arthritis. The history of dermatitis was remote. A high clinical index of suspicion and exome sequencing are imperative to make this rare diagnosis. Case description A six-year-old female was brought to an ophthalmologist with a complaint of a progressive decrease in vision in both eyes over the last four months. The vision deteriorated rapidly over the last couple of months. A detailed eye examination revealed features of granulomatous posterior uveitis with bilateral inflammatory cataracts. She was referred to the Rheumatology Outpatient Clinic. A detailed examination revealed boggy swellings on the bilateral wrist joints. As per the father, these swellings had been present for the last four years. The child never complained of pain in the wrists, or swelling and/or pain in any other joint. There was no history suggestive of arthritis or uveitis in the parents or immediate family members. The child occasionally developed an itchy red rash on the back. Such a rash has not occurred for the last many months. There was no skin lesion at the time of the examination. Suspecting Blau syndrome, a blood sample was sent for clinical exome sequencing (CES). Surgery (cataract extraction and intraocular lens implantation) was performed in the right eye. Intraoperative findings were noted, and features of granulomatous uveitis were evident. The patient was initiated on oral corticosteroids at 1 mg/kg of prednisolone by the eye surgeon. Four weeks later, the CES result was reported; it was positive for a heterozygous ‘pathogenic’ variant (detected in exon 4 of the NOD2 gene). The patient was reviewed after the report, and methotrexate was added. We started tapering the corticosteroids after six weeks of initial therapy. On the last eye examination (three months after the surgery), the uveitis had subsided. The vision was normal in the operated eye and had considerably improved in the unoperated eye. The surgery for the left eye was now deemed non-obligatory and was thus deferred. Discussion Blau syndrome is a rare autosomal dominant syndrome that causes arthritis, dermatitis, and granulomatous uveitis in children. A characteristic clinical feature of Blau syndrome is the presence of boggy swellings in the involved joints. Skin involvement usually occurs before the onset of arthritis and uveitis, presenting in the form of a rash (macro or micropapular and scaly), and occurs in infancy. Arthritis appears subsequently (between two and four years of age), followed by uveitis (around four years of age). Other uncommon manifestations of this disease are lymphadenopathy, sialadenopathy, erythema nodosum, neuropathies, leukocytoclastic vasculitis, glomerular and interstitial nephritis, interstitial lung disease, and pericarditis. A close mimic of this disease is sarcoidosis. Blau syndrome is an autosomal dominant disorder due to a mutation in the caspase recruitment domain gene CARD15/NOD2. There is no consensus on the optimal treatment for Blau syndrome. Corticosteroids and immunosuppressive agents, including methotrexate and azathioprine, have been used. Biologic agents have also been used with good results. In our case, the patient never had joint pains. He did not seek help until the child’s vision was impaired. Commonly, the ophthalmologist may be the only qualified physician of first contact. Joint pains may often be treated with self-medication or may be labelled as “growing pains” but the loss of vision is an unignorable catastrophic symptom and is invariably reported. The alert eye specialist must also enquire about fever, arthritis, and dermatitis in children with granulomatous uveitis. This correlation must be made sooner rather than later to prevent delay in diagnosis and therapy. The temporal dispersion of symptoms, i.e., the occurrence of different symptoms at different ages in the affected individual, also makes the diagnosis of Blau syndrome very challenging. Key learning points • Blau syndrome is a rare autosomal dominant disorder due to a mutation in the caspase recruitment domain gene CARD15/NOD2. • It causes arthritis, dermatitis, and granulomatous uveitis. • A characteristic clinical feature of Blau syndrome is the presence of boggy swellings in the involved joints. • A strong index of suspicion is required for diagnosis of this rare disorder.

COVID-19 and COVID-19 Vaccine-Related Skin Ulcerations in the Lower Extremities: A Case Report and Literature Review

Several associations have been made between COVID-19 and vasculitis. Recent data also shows the prevalence and association of de novo vasculitis with either COVID-19 infection or COVID-19 post vaccination. In this article, we present the case of new-onset leukocytoclastic vasculitis, secondary to COVID-19 vaccination, that was complicated by severe infected and nonhealing ulcers in the lower extremities. Case A 53-year-old male patient presented to the dermatology clinics with a three-week history of painful necrotic patches coalescent of the lateral malleolus of the right and left ankles. History goes back to when the patient reported developing pruritic papules two weeks after receiving his second shot of the Pfizer BioNTech COVID-19 vaccine (BNT162b2). Punch biopsy was consistent with leukocytoclastic vasculitis. He was prescribed a four-week course of systemic corticosteroids and antibiotics as per cultures. Vascular assessment confirmed normal peripheral arterial and venous system. Two months later, the patient re-presented with fever and worsening of his lower extremity ulcers. He underwent debridement of his wounds. Intra-operative cultures revealed multidrug resistant bacteria. He required an additional debridement session a few days later and a 14-day course of Piperacillin-Tazobactam. The patient was subsequently discharged on corticosteroids and Azathioprine and followed up in the vascular surgery and rheumatology clinics. At four months follow-up, the patient's wounds were almost completely healed. Conclusion This article highlights a case of severe new-onset COVID-19 vaccine-associated leukocytoclastic vasculitis complicated with infected ulcers that required debridement twice in addition to a prolonged course of antibiotics and immunosuppression therapy. To our knowledge, none of the cases reported in the literature were this severe in nature. In this post-pandemic era, it must remain high on the differential list, and healthcare specialists should maintain a high index of suspicion when evaluating sudden new-onset skin lesions that do not have an immediately apparent etiology.

Gastrointestinal manifestations and pathogenesis in childhood immunoglobulin A vasculitis.

Immunoglobulin A vasculitis (IgAV), previously known as Henoch-Schönlein purpura, is the most common form of systemic vasculitis in childhood. The primary organs involved are the skin, gastrointestinal (GI) tract, joints, and kidneys. The spectrum of GI involvement in IgAV ranges from being mild and self-limited to severe manifestations often requiring surgical intervention. Galactose-deficient IgA1 on the immunoglobulin hinge region and its immune complexes are thought to play a central pathogenetic role in IgAV, however, an association between such molecules and specific GI mucosal damage remains unclear. GI endoscopy (both upper and lower) shows a variety of mucosal findings, many of which are not specific for IgAV. In upper GI endoscopy, however, the mucosal features can be diagnostic when found localized in the more distal part of upper GI tract (second and/or third parts of the duodenum). Abdominal computed tomography and capsule endoscopy have demonstrated that the small intestine is most commonly involved in IgAV. The GI mucosal involvement when evaluated microscopically shows IgA deposition which is histologically diagnostic. Conversely, leukocytoclastic vasculitis is less useful. Since the 1960s, cases of duodenojejunitis, in which IgAV was suspected but evident purpura was not dermatologically present, have often been labeled as "idiopathic". In a pediatric case series, IgA enteropathy, without dermatological manifestations (i.e., purpura), was reported to have similar symptoms, as well as endoscopic characteristics and immunohistological findings as in IgAV. Subsequently, several case reports provide additional supportive evidence that IgA enteropathy must be a variant of IgAV. Thus, the immunologically driven auto-immune vasculitis results in the symptom complex dependent on the organ system involved, and the subsequent clinical features which are manifested. Present classification criteria are useful and universally available for diagnosing IgAV. However, based upon current knowledge including IgA enteropathy, minor modification of the IgAV criteria is proposed in the review.

An Australian Shepherd with possible Behçet-like disease characterized by cutaneous and intestinal vasculitis leading to septic abdomen.

An Australian Shepherd with possible Behçet-like disease characterized by cutaneous and intestinal vasculitis leading to septic abdomen.

Concurrent Post-Zoster Cutaneous Vasculitis and Acute Calcium Pyrophosphate Deposition Disease (CPPD) in an Elderly Patient: A Case Report

Concurrent Post-Zoster Cutaneous Vasculitis and Acute Calcium Pyrophosphate Deposition Disease (CPPD) in an Elderly Patient: A Case Report

Vasculitis associated with VEXAS syndrome.

To define the prevalence, distribution, and characteristics of patients with VEXAS who have confirmed vasculitis. Patients with VEXAS syndrome, verified by positive UBA1 mutation, were included. Chart review was performed to identify. characteristics and outcomes. Vasculitis diagnosis was based on either histopathology showing vascular inflammation or non-invasive angiography findings. Summary statistics were calculated. Eighty-nine patients met inclusion criteria. All were male with a median age of onset of 66.9 years (IQR 60.1, 72.7). Median (IQR) follow up was 3.8(2.2-5.5) years during which 21 patients (23.6%) had evidence of vasculitis. Vasculitis subtypes included small vessel vasculitis (19.1%), cutaneous medium vessel vasculitis (2.2%), and large vessel vasculitis (2.2%). No patient had more than one vessel size involved. Histopathology in small vessel vasculitis patients was consistent with cutaneous leukocytoclastic vasculitis in the majority, though one patient had leukocytoclastic peritubular capillaritis on renal biopsy. Cranial symptoms (headache, vision changes, or jaw pain) were noted in 18.0%. Two additional patients not experiencing cranial symptoms exhibited large vessel involvement with confirmed carotid thickening on non-invasive angiography; one of these had a positive temporal artery biopsy. VEXAS syndrome manifests as a variable vessel vasculitis in a quarter of patients, with cutaneous small and medium vessel involvement being particularly common. Some patients may have positive ANCA serologies or even renal vasculitis leading to misdiagnosis. Cranial symptoms are common and may mimic giant cell arteritis, though documented large vessel inflammation is rare.

Pharmaceutical Care in Patients with Side Effects to Oral Antineoplastic Agents: A Case Report

Background Chronic myeloid leukaemia (CML) is a haematopoietic stem cell disease characterised by the proliferation of granulocytes and their immature myeloid precursors. The treatments recommended are oral treatments dispensed in the outpatient unit (OU) of the hospital’s pharmacy service (PS), where patient-centred pharmaceutical care (PC) is essential to enhance therapeutic adherence, detect drug interactions and create a pharmacist-patient relationship to educate the patient on the main potential toxicities derived from the treatment and warning signs or symptoms requiring immediate attention. Methods We present the case of a 55-year-old woman, followed up in consultation by the Haematology Service (HS) since October 2021 for CML in the chronic phase. Results She started treatment on nilotinib 300 mg every 12 hours. The patient attended OU and was offered PC. After 12 months, nilotinib was well tolerated, but our patient experienced a loss of effectiveness, so HS decided to switch to dasatinib 100 mg daily. After one year of treatment, our patient was presented with symptoms of swelling and dyspnoea on moderate exertion. In January 2024, following a joint Haematology-Pharmacy session, a switch to bosutinib 400 mg daily was decided. After 15 days of treatment, the patient presented without an appointment due to a pruritic generalised rash on the torso, face, and extremities, being diagnosed with leukocytoclastic vasculitis. Conclusion In conclusion, we describe case reports of a patient who has a lack of effectiveness and ARs in several TKIs and the importance of identifying side effects, through early and close PC, in order for the patient’s evolution to be favourable.

Refractory Cutaneous Vasculitis in a Young Child with Granulomatous Polyangiitis Successfully Treated with Rituximab: A Case Report

Refractory Cutaneous Vasculitis in a Young Child with Granulomatous Polyangiitis Successfully Treated with Rituximab: A Case Report

Letrozole induced Necrotizing Cutaneous Vasculitis: A Rare Entity

Cutaneous small vessel vasculitis (CSVV) could be due to varied etiologies of which drug induced CSVV is a prime cause. It commonly presents as purpura but necrotizing lesions can be an uncommon presentation of the disease. Aromatase inhibitors like letrozole are newer drugs used in the management of breast carcinoma. There have been few reports of drug induced vasculitis secondary to letrozole. Herein, we report a case presenting with multiple purpura with ulcers and hemopurulent discharge over bilateral legs after letrozole intake for breast carcinoma. Patient was diagnosed with necrotizing CSVV due to letrozole based on history and clinico-histopathological correlation. To the best of our knowledge, this is the first report of letrozole induced necrotizing CSVV from India.

A Classical Manifestation of Eosinophilic Granulomatosis with Polyangiitis with an Unusual Catastrophic Outcome

A 57-year-old male patient, with a known case of bronchial asthma presented with acute dyspnea, cough with expectoration, fever, severe myalgia, and pedal edema. On physical examination, he had tachypnea, high temperature, bilateral polyphonic rhonchi, audible wheeze, and asymmetric sensory and motor neuropathy. Cutaneous examination revealed multiple palpable purpuric lesions with few hemorrhagic bullae and crusted erosions distributed symmetrically over bilateral limbs suggestive of cutaneous small vessel vasculitis. Investigations showed elevated total counts, eosinophilia, deranged electrolytes, and abnormal liver and renal function tests. Histopathology revealed eosinophilic predominant vasculitis. Testing for perinuclear anti-cytoplasmic antibody was positive. A diagnosis of eosinophilic granulomatosis with polyangiitis (Churg–Strauss disease) was thus confirmed. The patient succumbed in 2 weeks due to multi-organ failure. All the characteristic findings were found in our patient, but a fatal outcome in a very short period after diagnosis was an unusual presentation.

Diffuse Alveolar Hemorrhage Linked to Hydralazine

Hydralazine, a commonly prescribed vasodilator for hypertension, is associated with adverse effects such as vasculitis, glomerulonephritis, and drug-induced lupus. We present a rare case of hydralazine-induced diffuse alveolar hemorrhage (DAH) in a 74-year-old male with a history of hypertension. The patient was admitted with symptoms including hemoptysis, dyspnea, and dark urine. Initial findings included a biopsy-confirmed leukocytoclastic vasculitis, elevated MPO antibodies, and evidence of DAH on bronchoscopy. Despite the absence of glomerulonephritis, the patient exhibited signs of pulmonary-renal syndrome, including worsening renal function and anemia. The patient’s condition improved significantly after cessation of hydralazine and initiation of intravenous methylprednisolone. This case underscores the importance of considering hydralazine-induced ANCA vasculitis in patients presenting with unexplained pulmonary hemorrhage, particularly when other common causes are ruled out. Early recognition and management are crucial to prevent potentially life-threatening complications associated with this rare condition.

Unveiling the causal link between herpes virus infection and cutaneous leukocytoclastic angiitis: Insights from Mendelian randomization analysis

AbstractBackgroundCutaneous leukocytoclastic angiitis (CLA) is a clinically relevant condition, with previous studies suggesting an association with herpes virus infections. However, the causality of this association remains unclear. This study aimed to investigate the causal relationship between herpes viruses and CLA.MethodsGenetic variants linked to the herpes virus were retrieved from the Integrative Epidemiology Unit at the University of Bristol open genome‐wide association studies project and FinnGen database. Data on CLA, involving 262 CLA cases and 207,482 healthy controls, were obtained from the FinnGen consortium R7. Mendelian randomization (MR) analysis, including the inverse variance weighted (IVW), MR‐Egger, and weighted median methods, was performed. Sensitivity analyzes were conducted to ensure the accuracy of the results.ResultsOf the 15 herpes viruses investigated, only human herpesvirus 6 (HHV‐6) demonstrated a causal association with CLA (odds ratio: 1.886, 95% confidence interval: 1.053–3.378, p = 0.033), indicating that HHV‐6 infection significantly increases the risk of CLA. Furthermore, IVW and MR‐Egger tests for heterogeneity confirmed homogeneous MR analysis results without evidence of horizontal pleiotropy (p > 0.05). No significant causal relationship was observed for other herpes viruses, such as herpes simplex virus, varicella‐zoster virus, cytomegalovirus, and Epstein‐Barr virus.ConclusionOur MR analyzes strongly support a causal relationship between HHV‐6 and CLA, elucidating the etiology of this condition and highlighting the potential of HHV‐6‐targeted therapeutic interventions in CLA treatment. However, further research is necessary to expound the underlying mechanisms and explore potential therapeutic interventions targeting HHV‐6‐associated CLA.

A unique and rare presentation of Henoch-Schonlein purpura: a case report

Henoch-Schönlein purpura (HSP), or IgA vasculitis, is a common small vessel vasculitis in children, typically presenting with palpable purpura, abdominal pain, arthritis, and renal involvement. However, atypical presentations can delay diagnosis and management. A 12-year-old girl presented with multiple episodes of loose stools and abdominal pain. The initial diagnosis focused on acute gastroenteritis, and she was treated symptomatically. Subsequently, she developed urticarial rashes with breathing difficulty, complicating the diagnosis. Despite symptomatic treatment, she experienced severe abdominal pain, hematemesis and melena. Further investigations revealed antral gastritis, duodenitis, and cystitis. On the 14th day of admission, the patient developed characteristic palpable purpura on the lower limbs. Skin biopsy confirmed leukocytoclastic vasculitis with IgA deposits, consistent with HSP. The patient recovered with supportive care and remained symptom-free on follow-up. This case underscores the importance of considering HSP in pediatric patients with atypical presentations, such as gastrointestinal symptoms and urticaria without initial purpura. Early recognition and appropriate management are essential to prevent complications and ensure favourable outcomes. The variability in HSP presentations necessitates a high index of suspicion for timely diagnosis and treatment.

Leukocytoclastic Vasculitis as an Initial Indicator of Prostate Cancer: A Case Report

Leukocytoclastic Vasculitis as an Initial Indicator of Prostate Cancer: A Case Report

Diagnostic utility of direct immunofluorescence test panels for cutaneous vasculitis: A scoping review.

Due to the immune-mediated nature of non-infectious cutaneous vasculitis, skin biopsy specimens are often submitted for direct immunofluorescence (DIF) testing when vasculitis is considered clinically. However, evidence regarding the clinical value of DIF has not been rigorously appraised. In this scoping review, we aimed to systematically evaluate the peer-reviewed literature on the utility of DIF in vasculitis to assist with the development of appropriate use criteria by the American Society of Dermatopathology. Two electronic databases were searched for articles on DIF and vasculitis (January 1975-October 2023). Relevant case series involving more than or equal to three patients, published in English, and with full-text availability were included. Additional articles were identified manually via reference review. Due to study heterogeneity, findings were analyzed descriptively. Of 255 articles identified, 61 met the inclusion criteria. Cumulatively representing over 1000 DIF specimens, several studies estimated DIF sensitivity to be 75%. While vascular immunoglobulin A (IgA) deposits on DIF were associated with renal disease, other systemic associations were inconsistent. Vascular IgG deposition may be overrepresented in ANCA-associated vasculitis. Granular vascular and epidermal basement membrane zone Ig deposition differentiated hypocomplementemic from normocomplementemic urticarial vasculitis. Few studies have assessed the added value of DIF over routine microscopy alone in vasculitis. This scoping review discovered that DIF testing for vasculitis has been performed not only for diagnostic confirmation of vasculitis but also for disease subtype classification and prediction of systemic associations. Future studies on test sensitivity ofDIF compared to that of histopathology are needed.

Possible Terbinafine and/or Itraconazole Induced Vasculitis - A Case Study

Introduction: It is essential to exclude other causes, such as autoimmune diseases and bacterial infections, before attributing cutaneous/systemic vasculitis to drug use. Case Study: This report discusses the case of a young man who developed multi-organ failure and cutaneous vasculitis following the use of antifungal medications (terbinafine and itraconazole) for dermatophyte infections. Tests for autoimmune diseases and infections were negative. Given his drug history and a skin biopsy indicating leukocytoclastic vasculitis, it was inferred that the vasculitis was likely drug-induced. Despite treatment with steroids, intravenous immunoglobulins, and plasmapheresis, the patient did not survive, possibly due to delayed diagnosis and treatment. Conclusion: In community practice, Drug-induced Vasculitis (DIV) is frequently overlooked. When patients present with skin rash, fever, and multi-organ dysfunction, DIV should be considered, particularly in the context of recent drug use. Over-the-counter antifungals, like terbinafine or itraconazole, can cause DIV and may be fatal if not promptly diagnosed and treated.

Diagnostics and treatment of urticarial vasculitis associated with COVID-19

Currently, the problem of COVID-19 continues. The possible consequences of acute infection are not entirely clear. Various dermatological manifestations of COVID-19, including urticarial vasculitis (UV), are increasingly being reported. Urticarial vasculitis (UV) is a rare chronic inflammatory disease presenting with long-lasting wheals with or without angioedema [1]. Histopathological, UV is a leukocytoclastic vasculitis, a common form of small vessel vasculitis, including arterioles, capillaries, and postcapillary venules, in which the inflammatory infiltrate consists of neutrophils with fibrinoid necrosis and nuclear disintegration into fragments (“leukocytoclasia”) [1]. We report a case of urticular vasculitis in a 54-year-old woman who does not suffer from systemic diseases, with a burdened allergic anamnesis (drug allergy to levomycetin with a clinic of widespread toxicoderma). For 2 months after discharge, the patient took colchicine at a dosage of 0.5 mg, 1 tablet 2 times a day, which reduced the manifestation of clinical symptoms and achieved remission. According to the results of a comprehensive examination, a previously transferred COVID-19 infection is considered as a likely etiological factor.

Clinical Characteristics of Distinct Subgroups of Patients with Primary Sjögren's Syndrome Classified by Serological Profiles: A Comparison Study.

Sjögren's syndrome (SS) is an autoimmune disease characterized by heterogeneous clinical presentation and the presence of various autoantibodies. This study aimed to determine the differences in clinical findings according to antibody positivity in patients with primary Sjögren syndrome (pSS) in the Turkish population. A retrospective study was conducted and 402 patients (378 women and 24 men) with pSS were analyzed. The patients were categorized into three subgroups based on serological tests. These were (1) quadruple seropositivity (positive for anti-Sjögren's syndrome-related antigen A antibodies (anti-SSA; anti-Ro) and anti-Sjögren's syndrome-related antigen B antibodies (anti-SSB; anti-La), rheumatoid factor (RF), and antinuclear antibody (ANA); (2) double seropositivity (positive for ANA and anti-SSA/Ro antibodies); and (3) quadruple seronegativity (negative for ANA, RF, anti-SSA/Ro and anti-SSB/La antibodies). The number of quadruple-seropositive patients was 72 (18.6%), double-seropositive 174 (43.2%), and quadruple-seronegative was 85 (21.1%). The age at diagnosis of quadruple-seropositive pSS was 42.4 ± 10.8, which was significantly younger than that of patients with double-seropositive and quadruple-seronegative pSS (p = 0.021, p = 0.112). In terms of organ involvement, salivary gland enlargement, arthralgia, arthritis, Raynaud's phenomenon, lymphadenopathy, cutaneous vasculitis, interstitial lung disease, neurological involvement, autoimmune thyroiditis, renal interstitial disease, anemia, leukopenia, hypergammaglobulinemia, and hypocomplementemia were more common in quadruple-seropositive patients with pSS than in quadruple-seronegative patients (p < 0.0001). The results of this study confirmed the strong impact of immunological markers on the pSS phenotype at the time of diagnosis. Immunological patterns play a central role in the phenotypic expression of the disease, even during the initial diagnostic phase, and can guide physicians in designing personalized treatment plans for patients with pSS.

Vasculitis associated with psoriatic arthritis: a case report

BackgroundPsoriatic arthritis is a chronic inflammatory condition associated with psoriasis, which affects joints. The coexistence of vasculitis, an inflammation of blood vessels, with psoriatic arthritis is rare, highlighting a complex interplay between autoimmune diseases that affect both joints and vascular structures.Case presentationWe report on a 56-year-old male with a long-standing history of psoriasis and psoriatic arthritis, who presented with new-onset skin lesions and neurological symptoms. Clinical evaluations confirmed the presence of leukocytoclastic vasculitis and asymmetric sensory motor axonal neuropathy. The absence of positive results from various standard tests led to a challenging diagnostic process, ultimately pointing towards vasculitis associated with psoriatic arthritis. The patient was treated with corticosteroids and immunosuppressive therapy, leading to partial symptom improvement.ConclusionsThis case underlines the complex and often overlapping nature of autoimmune conditions, particularly the occurrence of vasculitis in patients with psoriatic arthritis, which is rare. It stresses the importance of considering vasculitis in patients with psoriatic arthritis who present with unexplained skin lesions and neurological symptoms. This case adds to the spectrum of psoriatic disease manifestations and suggests a need for further research to explore the underlying mechanisms and improve management strategies.

Acrocyanosis as a rare presentation of drug-induced cutaneous vasculitis: a case report

BackgroundAcrocyanosis is characterised by persistent bluish discolouration of the extremities, resulting from reduced peripheral blood flow leading to increased oxygen extraction. The aetiology can be divided into primary and secondary causes. While primary acrocyanosis is generally painless and has a benign course, secondary causes may lead to complications. This case reported acrocyanosis secondary to cutaneous vasculitis which progressed to digital gangrene, which is a rare complication of cutaneous vasculitis.Case presentationA 68-year-old man presented with a four-day history of bluish discolouration involving bilateral toes associated with pain and started to become gangrenous. Investigations for critical limb ischemia did not show evidence of critical arterial stenosis. Further history revealed history of recent administration of intramuscular injections with diclofenac, a non-steroidal anti-inflammatory agent for renal colic pain a few days prior to the onset of the. Thorough skin search showed multiple purpuric rash of his thighs, buttocks and abdomen. Skin biopsy confirmed the diagnosis of cutaneous (lymphocytic) vasculitis, which was likely to be drug-induced. The acrocyanosis initially responded to methylprednisolone, however unfortunately it progressed further to digital gangrene which required bilateral transmetatarsal amputations.ConclusionKnowledge on clinical features, aetiology and investigations of secondary acrocyanosis is crucial for early recognition and treatment of the underlying cause to prevent irreversible complications.