Purpuric Lesions Research Articles

A Rare Case of Acute Infectious Purpura Fulminans Caused by Klebsiella Pneumoniae and Human Herpesvirus Type 5

BackgroundPurpura fulminans (PF), a rare, life-threatening disorder, is a hematological emergency in which there is skin necrosis, disseminated intravascular coagulation (DIC), and protein C deficiency. In PF, the skin necrosis and DIC are secondary to protein C deficiency. This may progress rapidly to multiorgan failure caused by the thrombotic occlusion of small- and medium-sized blood vessels.Case ReportThis article presents the case of a 22-year-old male with fever as well as necrotic and purpuric skin lesions. The ultrasound and computed tomography scans revealed infections in the skin wounds as well as venous microthrombosis and thrombosis in multiple intracranial and pulmonary vessels. The laboratory tests showed signs of sepsis, thrombocytopenia, an abnormal decrease in protein C and antithrombin III, DIC, multiple organ and system failures, gastric varices, and gastrointestinal hemorrhage. The blood, sputum, and secretions under the skin lesions were cultured and were positive for Klebsiella pneumoniae. The results of the high-throughput genetic testing of the pathogenic microorganism DNA were consistent. In addition, human herpesvirus type 5 was detected. The histopathological examination of the skin lesions revealed pathological features consistent with PF. After successful treatment by the departments of Dermatology, Emergency Critical Care Medicine, and the Intensive Care Unit, the patient was discharged after 67 days of hospitalization.ConclusionAdults with acquired protein C and/or S deficiency states, including certain bacterial and viral infections, who drink alcohol and take varieties of non-steroidal anti-inflammatory analgesics at the same time, may develop acute infectious PF. Clinicians should be aware of this for early diagnosis and treatment.

Popeye's sign and transthyretin amyloidosis.

A 76-year-old man with a history of cervical open-door laminoplasty for ossification of the posterior longitudinal ligament presented to our hospital with a symptom of exertional dyspnoea. A 12-lead electrocardiogram revealed I-degree atrioventricular block (PQ interval 262 ms), left axis deviation, and complete right bundle branch block (Panel A). A transthoracic echocardiogram indicated left ventricular hypertrophy with a maximum wall thickness of 17 mm and a left ventricular ejection fraction of 61% (Panel B1). Strain echocardiogram using speckled tracking showed marked diminution of global longitudinal strain with apical sparing (Panel B2). Physical examination found subconjunctival hemorrhage in right eye, several purpuric lesions in upper extremities, and a large bulge on his left upper arm when he flexed his arm, so-called Popeye’s sign, indicating a hallmark of biceps tendon rupture (see Supplementary material online, Video S1; Panel C; arrow). A monoclonal component was not detected by serum and urine immune-electrophoresis and free light chain assays. 99mTc-labelled bone scintigraphy showed grade 3 myocardial uptake (Panel D). Eventually, endomyocardial biopsy and genetic sequencing analysis of the transthyretin gene confirmed the patient as a wild-type transthyretin amyloidosis (Panel E). Popeye’s sign is uncommon, but pivotal to diagnose transthyretin amyloidosis.

Sunitinib-Induced Cutaneous Small-Vessel Vasculitis: A Rare Case Report

Introduction: Sunitinib is an inhibitor of tumor angiogenesis and has been used as the first-line drug in metastatic renal cell carcinoma (RCC). Sunitinib attracts attention due to its cutaneous toxicities, which are unusual but can range from benign self-resolving skin rash to severe systemic involvement. Herein, we report a 51-year-old male case with metastatic RCC, clear cell type. Case Presentation: Due to multifocal metastatic disease, the patient was started on tablet sunitinib 50 mg once daily (2 weeks on and 1 week off protocol) with palliative intent. On the 4th day of the second cycle, the case developed multiple reticulate palpable purpuric lesions over bilateral shins without any other constitutional or systemic symptoms, such as fever, joint pain, or abdominal pain. Skin histopathology was consistent with clinical suspicion of cutaneous small-vessel vasculitis. The potential involvement of other organs was ruled out. The lesions resolved completely within 2 weeks through symptomatic treatment with topical fluocinolone acetonide 0.01% cream and emollients. Conclusions: Sunitinib was continued uninterrupted without other organ involvement or the reappearance of lesions. Therefore, oncology care providers should always be vigilant and thoroughly evaluate any cutaneous toxicities associated with sunitinib, although it is a rare manifestation.

Murine typhus complicated by sHLH mimicking adult-onset Still's disease

IntroductionAdult-onset Still's disease (AOSD) is a rare multisystemic disorder and a diagnostic challenge for physicians because of the wide range of differential diagnoses. Common features of AOSD and secondary hemophagocytic lymphohistiocytosis (sHLH) could favour diagnostic uncertainty, in particular in case of infection-related sHLH. ObservationA 61-year-old man was admitted to our internal medicine department for suspected AOSD. He reported a 2-week history of sudden onset fever, headaches, myalgia, sore throat, diarrhoea, and an erythematous macular rash of the trunk as well as petechial purpuric lesions on both legs on return from Reunion Island. Laboratory tests found cytopenia, hepatic cytolysis, hypertriglyceridaemia, and hyperferritinaemia. Hemophagocytosis was diagnosed on bone marrow aspiration in favour of the diagnosis of secondary hemophagocytic lymphohistiocytosis (sHLH). Subcutaneous anakinra (100mg) was initiated to treat sHLH with favourable course. Oral doxycycline was added 3days later because of atypical features for AOSD diagnosis such as diarrhoea, hypergammaglobulinaemia, and doubtful serologies for Rickettsia and Coxiella. Three weeks later, Rickettsia typhi serology was checked again and revealed an increase in IgG titer>4 times that confirmed the diagnosis of murine typhus. A diagnosis of murine typhus complicated by sHLH was retained, successfully treated by anakinra and doxycycline. ConclusionOur observation shows that AOSD diagnosis has to be stringent due to the many differential diagnoses, particularly infection complicated by sHLH, which may be rare. It is important to consider murine typhus in patients returning from endemic areas, such as La Reunion or other tropical areas, when they present fever of unknown origin with non-specific clinical features. Moreover, this case illustrates the effectiveness of IL-1 blockers as a treatment for symptomatic sHLH without severity criteria, regardless of the aetiology.

P035 Subtle skin rash was the only presenting symptom of a premalignant condition; monoclonal B-cell lymphocytosis driven cryoglobulinaemic vasculitis, a case report

Abstract Background/Aims Intermittent episodes of subtle skin rash at the hands are commonly associated with dermatitis or allergy. We are reporting a case of cryoglobulinaemic vasculitis with a delayed diagnosis of more than two years because of subtle symptoms and normal investigations; highlighting important points to consider during the assessment of similar cases. Methods Case description: In June 2018, a 59-year-old man was initially assessed for intermittent episodes of pruritic skin rash usually on cold exposure, for the preceding 2 years. The patient described and showed pictures of non-blanching deep red-bluish plaques with raised irregular margins involving only the hands; usually self-resolving over 2-3 days without specific treatment and there were no defined predisposing factors. There was history of Raynaud’s, loss of appetite and weight loss of half a stone, but otherwise no other CTD/vasculitis symptoms. Clinical examination showed no skin, joints or systemic abnormalities. Investigations revealed positive RF at 30 U/mL, low C3 and C4 at 0.5 and < 0.1 g/L respectively; whereas full blood count, renal function, liver function, urine protein, ESR, CRP, immunoglobulins, s. protein electrophoresis, ANA, ANCA, HIV and hepatitis screen were all negative. Type 1 cryoglobulinaemia was detected at room temperature and cryoprecipitating IgG kappa was 3g/L on polyclonal background. Peripheral blood immune-phenotyping confirms kappa light chain and monoclonal B-cell lymphocytosis of CLL phenotype. Nailfold capillaroscopy revealed coiled capillaries and corkscrew ramification. CT-Chest abdomen pelvis showed widespread generalised lymphadenopathy. Over 3 years post diagnosis; there was no transformation of MBL to CLL clinically or haematologically except for episodes of rash, thus the plan was to continue monitoring for ‘B symptoms’, and to consider rituximab if significant symptoms appear. Results Cryoglobulins (immunoglobulins that precipitate in cold) are classified into three types: type I (monoclonal), type II (mixed) and type III (polyclonal). Polyclonal cryoglobulins are associated with autoimmune rheumatic diseases, hepatitis B and C. Mixed essential cryoglobulinaemia presents with purpuric skin lesions showing a leucocytoclastic vasculitis on biopsy. Type I monoclonal IgG, IgM, IgA, or their κ or λ light chains represent 10-15 % of the cases of cryoglobulinaemia. It is characterised by overwhelming association with hematologic neoplasia including MGUS, smouldering multiple myeloma, multiple myeloma, waldenström's macroglobulinemia, and chronic lymphocytic leukemia. Treatment is generally directed towards treating the underlying pre-malignant or malignant disorder. Conclusion Cryoglobulinaemia has a wide variety of clinical presentation scenarios, but physicians can play a key role by early recognition. In addition to the routine investigations, patients with suspected vasculitic rash especially to cold exposure need to be assessed for rheumatoid factor, complements and cryoglobulins, and cases confirmed with type I cryoglobulinaemia should be investigated for haematological malignancies. Nailfold Capillaroscopy is crucial in distinguishing secondary Raynaud’s, another feature of cryoglobulinaemia. Disclosure Z. Alkutobi: None. D. Laila: None. G. Ansari: None. A. Nandagudi: None.

P152 A rare case of necrotising vasculitis presenting with calf swelling in a 15-year-old female

Abstract Background/Aims We present the case of MF, a 15-year-old female who presented to the paediatric clinic with swelling and pain affecting her right calf. She was previously fit and well. Initial imaging was suggestive of localised myositis but a subsequent muscle biopsy was in keeping with necrotising vasculitis. Methods In May 2021, MF presented to a general paediatric clinic with a 6-week history of right lateral calf pain and swelling to the lateral border of the right proximal fibula. She was systemically well. Blood tests and an x-ray were normal. An ultrasound scan of the right calf showed fusiform thickening of the peroneus longus muscle proximally. There was no discrete mass. The appearance was thought to be an inflammatory process such as myositis. An MRI scan was advised. Over the course of the next few weeks, the swelling and pain to the right calf progressed and she was unable to fully weight bear. Repeat blood tests were normal except CRP 11. CK was normal. At the end of June, an MRI scan of the right lower leg showed significant swelling and oedema along the length of the peroneus longus musculature as demonstrated on ultrasound. Florid oedema extended to involve the soleus and to a lesser extent the lateral head of gastrocnemius. There was thickening and oedema through the subcutaneous fat circumferentially compared to the left side (see images). The appearances were thought to be consistent with myositis but an inflammatory malignancy could not be fully excluded and an opinion from the Birmingham team was recommended. The Birmingham MDT advised that malignancy was unlikely but a CT scan was recommended. The CT scan confirmed the MRI findings (see image). Results MF's symptoms progressed rapidly over a short period of time with diffuse swelling of the right lower limb with associated pain and numbness. She also developed purpuric lesions around the ankle (see photos). The CRP increased to 59 and then 123. CK remained normal. Local rheumatology opinion was sought once malignancy had been excluded. The initial impression was of a focal myositis but a muscle biopsy was requested urgently. IV steroids were administered followed by high dose oral prednisolone as a reducing course. The symptoms improved rapidly. The subsequent biopsy showed a florid necrotising vasculitis affecting the small-medium arterial vessels. There was no evidence of myositis (see image). ANCA was negative and CT renal and abdominal angiogram was normal. Nerve conduction studies showed a very severe sensory lesion of the right sural nerve, which explained the persistent numbness. Mycophenolate mofetil was added and clinical improvement is ongoing. Conclusion We present a rare case of localised vasculitis in the right calf. A literature review indicates this is the first case presented in an adolescent. Disclosure S.C. Earl: None. J. Smith: None. A. Ramanan: Grants/research support; Abbvie.

Characteristic Linear and Zigzag Purpuric Lesions in a Patient with Long-term and Repeated Exposure to Bedbug Bites.

Abstract is missing (Short communication)

Dilated Cardiomyopathy Revealing a Henoch-Schönlein Purpura in a Young Patient: About a Case and Review of the Literature…

Schönlein-Henoch purpura is a non-thrombocytopenic systemic vasculitis of small-caliber vessels due to immune complexes. We report the case of an adult 30 year old man admitted for a generalized edematous syndrome with purpuric lesions in the 2 lower limbs revealing a dilated cardiomyopathy whose etiological assessment was in favor of a Schönlein-Henoch purpura with cardiac, renal, dermatological involvement. The diagnosis was difficult to establish given the clinical polymorphism. Also, management was complicated given the multifocal involvement. The treatment was based on corticosteroid therapy and rituximab but the evolution was, unfortunately, not satisfactory.

Acute hemorrhagic edema of infancy

Acute hemorrhagic edema of childhood, also called acute hemorrhagic edema of infancy (AHEI) or Finkelstein-Seidlmayer disease is a vasculitis of small blood vessels. The etiology of the disease is unknown, but various reports in the literature indicate precursor triggers including a preceding infectious disease. This article presents a clinical observation of an 8-month-old boy with ecchymotic skin lesions on legs and ears as well as genital edema, with a history of mild upper respiratory infection two weeks before referral. Laboratory findings were normal. After a few days, he was discharged without particular treatment. Despite the low prevalence of this disease, it is important to consider it during examinations due to the clinical nature of the diagnosis. Based on clinical and laboratory findings, a diagnosis of AHEI was made. We decided not to perform any therapy, and after about two weeks lesions had a self-limited resolution. The child was monitored clinically for about six months, and he did not present any relapse of the disease during the follow-up period. This patient was admitted to the hospital, underwent important laboratory tests, and was evaluated by multiple subspecialty services prior to diagnosis. While keeping in mind more worrisome diagnoses, physicians should consider the diagnosis of AHEI in well-appearing young children with purpuric lesions on the face and ears and non-pitting edema of the extremities. Awareness and early recognition of AHEI may prevent hospital admission, invasive workup, and parental and physician concern. Key words: acute hemorrhagic edema of infancy, Finkelstein-Seidlmayer disease, vasculitis

Skin manifestations in patients with COVID-19: a prospective observational study during the first wave of the pandemic in the UK and review of the recent literature.

BackgroundCOVID-19 (SARS-CoV-2) is a viral infection that presents in heterogeneous forms with effects on multiple organ systems including the skin.ObjectivesThe objectives of this prospective observational study were to identify cutaneous lesions in confirmed COVID-19-positive patients admitted to a district general hospital in the eastern region of England, to determine the prevalence of these lesions and compare the findings with the current literature.Materials & MethodsThe study was conducted at the Luton and Dunstable University Hospital during the first peak of the pandemic in the United Kingdom to identify skin manifestations in patients infected with COVID-19. Several variables were taken into consideration and photographs of skin lesions were taken. Unlike previous similar studies, all patients included in this study had a positive nasopharyngeal PCR swab for SARS-CoV-2. All photographs were analysed by a dermatology consultant.ResultsA total of 93 patients were included in the study; 40% (n = 37) had cutaneous lesions but only 5.5% of the total patients (n = 6) presented with likely coronavirus-related skin changes. Lesions identified were pseudochilblain and purpuric/livedoid type. We also noted several coagulation abnormalities in these patients.ConclusionCOVID-19 can present with a variety of skin manifestations. Pseudochilblain lesions and purpuric livedoid lesions have been described in the literature and although the underlying mechanism is not fully understood, it is possible that these lesions represent thromboinflammatory processes as a result of the hypercoagulability state associated with COVID-19. More research is required to better understand the pathophysiology and epidemiology of these manifestations.Supplementary dataSupplementary data associated with this article can be found, in the online version, at doi: 10.1684/ejd.2022.4202.

Purpura fulminans, presenting feature of multisystem inflammatory syndrome in children (MIS-C)

Introduction. Coronavirus disease 2019 (COVID-19) has been a worldwide health issue since early 2020, caused by the severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2). Even though children do not usually present with se­vere COVID-19 unless the patient has an underlying di­sease, post­in­fectious complications, such as the multisystem in­flam­ma­to­ry syndrome in children (MIS-C), have been a sig­ni­fi­cant cause of child morbidity and mortality during the pan­de­mic. Even more challenging is that MIS-C has a very broad differential diagnosis due to its clinical heterogeneity. Case report. We report the case of a 6-month-old infant with­out another medical history who was brought to the ge­ne­ral practitioner’s office for persistent fever and cough. The symp­toms did not ameliorate under antipyretic and mucolytic treat­ment, so the patient later presented in the emergency room with associated dyspnea, diarrhea, vomiting and rapidly ge­ne­ra­li­zing purpuric skin lesions suggestive of purpura ful­mi­nans. He was admitted into our hospital and received em­pi­ri­cal antibiotic treatment. He also developed neck stif­fness. Meningococcemia was thus suspected; however, all cultures came out negative. Laboratory studies showed an important inflammatory syndrome, thrombocytopenia, se­vere anemia, coagulopathy, low protein C, protein S and an­ti­throm­bin levels, low fibrinogen, high D-dimer levels and hypo­al­bu­mi­ne­mia. Cerebral magnetic resonance imaging (MRI) showed no evidence of brain lesions. SARS-CoV-2 IgG-type antibodies were positive, therefore MIS-C was sus­pec­ted. The laboratory studies were extended and revealed high NT-proBNP, IL-6 and IL-2 soluble antigen levels. Other pos­si­ble diagnoses were excluded, which permitted our team to establish the diagnosis of MIS-C. The specific treatment was initiated with intravenous immunoglobulins (IVIG) and high-dose methylprednisolone associated with enoxaparin, broad-spectrum antibiotics and with symptomatic treatment. The patient rapidly evolved well, with the remission of fe­ver, inflammatory syndrome, coagulopathy and all other clinical and laboratory alterations. The skin lesions ame­lio­ra­ted as well, however, at a slower pace. Conclusions. MIS-C is a potentially life-threatening complication of COVID-19 in children. Therefore, clinicians must maintain a high degree of suspicion in the setting of the ongoing SARS-CoV-2 pandemic. However, the diagnostic criteria should be strictly followed due to the clinical similarities this pathology might have with other known diseases.

Daily follow-up of a scary onset of ecchymotic purpuric lesions in an infant

ENWEndNote BIBJabRef, Mendeley RISPapers, Reference Manager, RefWorks, Zotero AMA Al-Muriesh M, Liu J, Du H, et al. Daily follow-up of a scary onset of ecchymotic purpuric lesions in an infant. Advances in Dermatology and Allergology/Postępy Dermatologii i Alergologii. 2022;39(4):806-808. doi:10.5114/ada.2022.118925. APA Al-Muriesh, M., Liu, J., Du, H., Wu, S., Wu, T., & Sun, Y. et al. (2022). Daily follow-up of a scary onset of ecchymotic purpuric lesions in an infant. Advances in Dermatology and Allergology/Postępy Dermatologii i Alergologii, 39(4), 806-808. https://doi.org/10.5114/ada.2022.118925 Chicago Al-Muriesh, Maher, Juan Liu, Hongxia Du, Shidi Wu, Ting Wu, Yanhong Sun, and Changzheng Huang. 2022. "Daily follow-up of a scary onset of ecchymotic purpuric lesions in an infant". Advances in Dermatology and Allergology/Postępy Dermatologii i Alergologii 39 (4): 806-808. doi:10.5114/ada.2022.118925. Harvard Al-Muriesh, M., Liu, J., Du, H., Wu, S., Wu, T., Sun, Y., and Huang, C. (2022). Daily follow-up of a scary onset of ecchymotic purpuric lesions in an infant. Advances in Dermatology and Allergology/Postępy Dermatologii i Alergologii, 39(4), pp.806-808. https://doi.org/10.5114/ada.2022.118925 MLA Al-Muriesh, Maher et al. "Daily follow-up of a scary onset of ecchymotic purpuric lesions in an infant." Advances in Dermatology and Allergology/Postępy Dermatologii i Alergologii, vol. 39, no. 4, 2022, pp. 806-808. doi:10.5114/ada.2022.118925. Vancouver Al-Muriesh M, Liu J, Du H, Wu S, Wu T, Sun Y et al. Daily follow-up of a scary onset of ecchymotic purpuric lesions in an infant. Advances in Dermatology and Allergology/Postępy Dermatologii i Alergologii. 2022;39(4):806-808. doi:10.5114/ada.2022.118925.

Dermatological manifestations of Coronavirus Disease 2019 (COVID-19)

The prevalence of dermatological manifestations among patients with COVID-19 has a wide variation from 0.2 - 20.4% in different countries. The commonly described manifestations are; maculopapular eruptions, urticarial lesions, pseudo-chilblains, vesicular eruptions and livedo or necrosis. There are several other manifestations described such as; enanthems, purpuric flexural lesions, Dengue-like rash, erythema multiforme-like lesions and vasculitis-like lesions. The study done in the National Hospital of Sri Lanka has revealed a prevalence of 1.7%. The survey among dermatologists island wide shows few cases with different cutaneous manifestations of COVID-19. It is important to be vigilant as the COVID-19 virus as well as the knowledge about it, is still evolving.

Vasculitis in a patient with mevalonate kinase deficiency (MKD): a case report

BackgroundMevalonate kinase deficiency (MKD) is a rare autoinflammatory condition caused by biallelic loss-of-function (LOF) mutations in mevalonate kinase (MVK) gene encoding the enzyme mevalonate kinase. Patients with MKD display a variety of non-specific clinical manifestations, which can lead to diagnostic delay. We report the case of a child presenting with vasculitis that was found by genetic testing to be caused by MKD, and now add this autoinflammatory disease to the ever-expanding list of causes of monogenic vasculitides.Case presentationA 2-year-old male presented with an acute 7-day history of high-grade fever, abdominal pain, diarrhoea, rectal bleeding and extensive purpuric and necrotic lesions, predominantly affecting the lower limbs. He had been suffering from recurrent episodes of fever from early in infancy, associated with maculopapular/petechial rashes triggered by intercurrent infection, and after vaccines. Extensive infection screen was negative. Skin biopsy revealed small vessel vasculitis. Visceral digital subtraction arteriography was normal. With a diagnosis of severe idiopathic cutaneous vasculitis, he was treated with corticosteroids and mycophenolate mofetil. Despite that his acute phase reactants remained elevated, fever persisted and the vasculitic lesions progressed. Next-generation sequencing revealed compound heterozygous mutation in MVK c.928G > A (p.V310M) and c.1129G > A (p.V377I) while reduced mevalonate enzyme activity was confirmed suggesting a diagnosis of MKD as a cause of the severe vasculitis. Prompt targeted treatment with IL-1 blockade was initiated preventing escalation to more toxic vasculitis therapies and reducing unnecessary exposure to cytotoxic treatment.ConclusionsOur report highlights the broad clinical phenotype of MKD that includes severe cutaneous vasculitis and emphasizes the need to consider early genetic screening for young children presenting with vasculitis to exclude a monogenic vasculitis which may be amenable to targeted treatment.

Atypical Targetoid Lesions as the Presenting Sign of Myeloid Sarcoma

Myeloid sarcoma (MS), also known as chloroma or granulocytic sarcoma, is a rare extramedullary tumor mass that commonly occurs in acute myeloid leukemia (AML). MS may occur before other clinical signs of AML, or alongside other known AML symptoms. MS can also appear in isolation, which makes diagnosis challenging. Clinical knowledge about a typical presentation of myeloid sarcoma is limited, given the heterogeneity in the location and size of MS lesions. We describe a patient with a history of AML who developed an eruption of atypical targetoid purpuric lesions. Diagnostic workup, including laboratory and histologic specimen from the skin were consistent with MS. MS should be suspected in a patient with a history of myeloproliferative disorder who develops atypical targetoid purpuric papules.

Cytogenetically cryptic TNIP1-PDGFRB and PCM1-FGFR1 fusion leading to myeloid/lymphoid neoplasms with eosinophilia (MLN-eo) in children

▪Introduction: MLN-eo associated with gene rearrangements of PDGFRA, PDGFRB, FGFR1, or PCM1-JAK2 are rare haematological neoplasms primarily affecting adults. Eosinophilia commonly occurs but may also be absent. The heterogeneous clinical picture and the rarity of the disease, especially in children, may delay an early diagnosis. MLN-eo are characterized by constitutive tyrosine kinase activity due to gene fusions. It is thus of prognostic importance to obtain a prompt genetic diagnosis to start a specific therapy.Here we report two female paediatric cases of MLN-eo (6 months and 13 years old at initial diagnosis).Methods: In both cases, bone marrow morphology, karyotyping, fluorescence in-situ hybridization analysis (FISH) via break apart probes (PDGFRB (5q32), FGFR1 (8p12), JAK2 (9p24), FIP1L1/CHIC2/PDGFRA (4q12)), targeted RNA sequencing and in one case array CGH were performed.Results: The 6 months old girl was admitted to hospital with a 3-month history of rash and leukocytosis with eosinophilia. The skin showed multiple purpuric lesions (Fig 1 A/B). Mild splenomegaly was noted. White blood count (WBC) was 48000/µl with 38% eosinophils. Bone marrow trephine showed hypercellular marrow with mild fibrosis and eosinophilia without increase in blasts. Biopsy of a skin nodule displayed a histological pattern of interface dermatitis with eosinophilic infiltrate. (Fig 1 C/D). Fluorescence R-banding showed a normal karyotype (46,XX) (Fig. 2 A). However, FISH and array CGH detected an interstitial deletion of 5' PDGFRB (5q32) in 61 % of interphase nuclei (Fig. 2 B-D). Targeted RNA sequencing (RNA-seq) confirmed, as the array CGH suggested, the suspected TNIP1/PDGFRB fusion. According to the WHO criteria, diagnosis of a myeloid neoplasia with PDGFRB rearrangement due to an interstitial deletion in 5q was made. Because of the PDGFRB rearrangement, imatinib (250 mg/m²/d) therapy was started. Leukocyte and eosinophil counts normalized within 4 days without signs of tumour lysis. Skin lesions disappeared within 2 weeks. After 4 weeks, the dose was reduced to 100 mg/m² 3 x/week. Now at 14 months of age, peripheral counts continue to be normal and the fusion transcript is not detectable in the peripheral blood.The 13 years old girl was admitted with severe tachypnoea due to pleural effusions, hepatosplenomegaly and lymphadenopathy. Echocardiography showed endocarditis, left ventricular fibrosis and mitral insufficiency. WBC was 112170 /µL with 39% eosinophils. Bone marrow aspirate and trephine showed a feature of myeloproliferative neoplasia (MPN) with eosinophilia. The karyotype was normal. A rearrangement involving the FGFR1 locus was detected by FISH (Fig. 3 B/C). Splitting of the probe signals indicated an inversion on chromosome 8. Targeted RNA sequencing revealed a PCM1-FGFR1 fusion transcript. Diagnosis of a MLN-eo with FGFR1 rearrangement and evidence of a PCM1-FGFR1 fusion, most likely caused by an inversion on chromosome 8, was made. The girl stabilized after therapy with prednisone, vincristine, hydroxycarbamide and anti-IL-5 antibody. Peripheral blood counts normalized within 2 weeks. Eight weeks after initial diagnosis she presented with signs of a transient ischemic attack, respiratory distress and arterial hypotension. At that time WBC was 139000/µl with 53% myeloid blasts and 5% eosinophils. Trisomy 8 was detected in all metaphases and 88% of cells in FISH (Fig.3 A-C). Diagnosis of a progression to a myeloid blast phase was made. Induction chemotherapy (cytarabine, idarubicin, etoposidphosphate) was administered. On day +22 bone marrow aspirates showed the persisting picture of MPN. Preparations for hematopoietic stem cell transplantation (HSCT) and ponatinib therapy were begun, but cardiac and respiratory insufficiency that developed during chemotherapy were fatal.Conclusion: As these two cases have shown, standard cytogenetic and molecular methods may not be sufficient to diagnose MLN-eo due to cytogenetically cryptic aberrations. Thus, genetic diagnosis must be precise and quick (e.g. break apart FISH, targeted RNA-seq) in order to initiate adequate therapies with tyrosine kinase inhibitors or HSCT. Patients with rearrangements of PDGFRA or PDGFRB usually respond well to imatinib, whereas patients with FGFR1 and JAK2 gene fusions exhibit more aggressive diseases with variable sensitivity to tyrosine kinase inhibitors and have an early indication for HSCT. [Display omitted] DisclosuresReiter: Novartis: Honoraria, Membership on an entity's Board of Directors or advisory committees, Other: Travel expenses, Research Funding; Blueprint Medicines: Honoraria, Membership on an entity's Board of Directors or advisory committees, Other: Travel expenses; Incyte: Membership on an entity's Board of Directors or advisory committees, Other: Travel expenses; AOP Orphan Pharmaceuticals: Honoraria, Membership on an entity's Board of Directors or advisory committees, Other: travel support; Deciphera: Membership on an entity's Board of Directors or advisory committees, Other: Travel expenses; Abbvie: Membership on an entity's Board of Directors or advisory committees; Celgene/BMS: Honoraria, Membership on an entity's Board of Directors or advisory committees, Other: travel support.

Oral Findings in Patients with COVID-19 Infection: Report of Two Cases and Review of Literature

Few reports have been published highlighting oral findings in COVID-19 patients. Since the outbreak of the recent pandemic caused by SARS-CoV-2, it is important to illustrate in the very near future how recurrent these oral findings are in the symptomatic COVID-19 patients in contrast to those asymptomatic ones, as there are multiple findings reported. This case study reports a new combination of oral findings in two COVID-19 patients from India, one symptomatic, and other asymptomatic. Cases presenting oral findings in asymptomatic COVID-19 patients are the rarest. Moreover, till now, petechial or purpuric lesions, solely as theoral findings without any associated skin lesions and oral mucosal hyperpigmentation of our patients are the rare oral findings to be reported in COVID-19 patients. These cases provide evidence for a deeper understanding of the oral alterations seen in COVID-19 patients that need to be addressed at present.

ANCA Associated vasculitis- A case of microscopic polyangiitis with proliferative glomerulonephritis

Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) are a collection of diseases, characterised by destruction and inflammation of small and medium vessels. Microscopic Polyangiitis (MPA) is part of an ANCA-associated vasculitis (AAV).The clinical signs diverge and disturba number of organs such as the kidneys, lungs, stomach and intestine. Skin manifestations such as purpuric, urticarial, nodular, ulcerative, livedoid and necrotic skin lesions were common as in other vaso-occlusive disorder. Morphology and added features aid the diagnostic approach. Here, we report a diagnostically challenging case of microscopic polyangiitis with progressive glomerulonephritis.

Lucio Phenomenon: A Rare Manifestation of Leprosy in Selangor, Malaysia

Leprosy is a chronic infectious disease caused by Mycobacterium leprae, predominantly affecting the skin and peripheral nerves. Lucio phenomenon is a rare reactional state presenting in patient with lepromatous leprosy and described as acute cutaneous necrotising vasculitis. We discussed the case of a 38-year-old male patient presenting with oedematous and dusky discolouration of bilateral hands and feet associated with diffuse facial involvement. His skin condition gradually worsened to form multiple ulcers with bizarre shaped purpuric lesion over bilateral upper and lower limbs, trunk, and face. Histopathological examination of the skin biopsy showed multiple acid-fast bacilli and diagnosis of Lucio’s phenomenon was made in the background of lepromatous leprosy. He was treated with multi drug therapy (MDT) as recommended by the WHO guidelines. A better understanding of rarer manifestation of this illness is important for early diagnosis and to prevent significant morbidity associated with it.International Journal of Human and Health Sciences Supplementary Issue-2: 2021 Page: S20

Painful Plantar Rash with Systemic Symptoms

Painful Plantar Rash with Systemic Symptoms