Necrotising Cutaneous Lesions in Anti Neutrophil Cytoplasmic Antibodies- Associated Vasculitis Mimicking Necrotising Fasciitis

Necrotising vasculitis is a very rare occurrence and commonly caused by an underlying autoimmune pathology. Anti Neutrophil Cytoplasmic Antibodies (ANCA) associated vasculitis is an established cause for cutaneous manifestations of necrotising vasculitis, and is associated with multisystemic involvement, in which presentations of one system may feature predominantly and lead to misdiagnosis. A 33-year-old male presented with acute history of shortness of breath and necrotising blisters over both his ankles and arms. He was initially provisionally diagnosed with necrotising fasciitis, which was eventually ruled out as the serological biomarkers (including low level of C3 and presence of cytoplasmic type of ANCA on indirect immunofluorescence) drew suspicion of an autoimmune pathology. A multidisciplinary approach led to the commencement of steroid, which improved the patient’s condition rapidly. The histological examination of sample from the skin biopsy later confirmed the diagnosis of ANCA-Associated Vasculitis (AAV). The patient eventually recovered with excellent outcomes. Despite its rarity, any abnormal skin lesion may be a subtle presentation of necrotising cutaneous vasculitis. A high index of suspicion through a multidisciplinary approach, coupled with meticulous investigative process including serological biomarkers and skin biopsy, helps to avoid misdiagnosis which may be detrimental to the patient.

Necrotising Vasculitis in Covid-19: watch out for bowel perforation

Introduction: SARS-Cov-2 infection can be asymptomatic, greatly impair respiratory function and, sometimes, affect other organs. Gastro-enteric involvement seems to be not so rare and many patients suffered for abdominal pain, anorexia, nausea and vomiting, diarrhoea or jaundice. Case presentation: Here we report a case of a Covid-19 patient who developed a right colon perforation due to a pseudomembranous colitis without Clostridium Difficile toxins finding associated to a necrotising vasculitis. In this patient a PCR test performed on gastric fluid showed SARS-CoV-2 enteric replication. Discussion: Necrotizing vasculitis is an anatomopathological typical findings in Covid-19. It has been documented in several organs and tissues. In our case, evident foci of necrotizing vasculitis with intravascular obliteration by fibrin thrombi with macrophagic cells infiltration are anatomopathological findings of a CD toxins free pseudomembranous colitis leading to bowel perforation. Conclusions: Bowel perforation due to necrotizing vasculitis leading to pseudomembranous colitis could be a SARS-Cov-2 related clinical finding.

Eosinophilic granulomatosis with polyangiitis in comorbidpatient: is the immunological diagnostics always unambiguous

Eosinophilic granulomatosis with polyangiitis (EGPA), previously called Churg—Strauss syndrome, represents a rare form of ANCA-associated necrotising vasculitis which affects small vessels. This disease is characterized by typical combination of immunological disturbances, hypereosinophilia, severe bronchial asthma, transient pulmonary infiltrates, and kidney injury which is less frequent than in other forms of necrotising vasculitis.Verification of the diagnosis is often hampered by comorbidities, such as chronic obstructive pulmonary disease (COPD) in the patients with long-term smoking history and occupational hazards.In this article, we report a clinical case of EGPA in elderly patient with preceding COPD which caused diagnostic difficulties for this eosinophilic syndrome. Clinical pattern at the beginning of disease was presented by moderate inspiratory dyspnea and cough with small amounts of mucus sputum, which appeared after longterm exposure to chlorine-containing substances. COPD diagnosis in this patient was based on clinical pattern, long smoking experience, and occupational hazards. However, persistence of the symptoms during the ongoing therapy, as well as multidirectional dynamics of transient pulmonary infiltrates found on repeated CT-scans, prompted us to intensify diagnostic search for a systemic disease. Clinical, laboratory and instrumental signs of bronchial asthma were revealed, as well as hypereosinophilia and sensory polyneuropathy, which, if combined with CT-scan data, allowed us to prove the EGPA diagnosis.This case shows that, despite great value of immune diagnostics, with negative blood tests for ANCA, it is necessary to detect mutually complicating comorbid pathology. EGPA was considered the basic diagnosis, and COPD as accompanying disorder, taking into account such reasons as an unfavorable prognosis for EGPA and the need for long-term chemotherapy with systemic corticosteroids and monoclonal antibodies. ANCA-negative testing in the patient, absence of severe kidney and skin lesions allows to suggest better clinical prognosis in this patient.

Toxic epidermal necrolysis and concurrent granulomatosis with polyangiitis (Wegener’s granulomatosis). Management of a rare case and review of the literature

[Formula: see text] Toxic epidermal necrolysis (TEN) is a rare, acute life-threatening mucocutaneous disorder that is characterised by epidermal loss/exfoliation exceeding 30% total body surface area (TBSA) and is on a spectrum that includes erythema multiforme and Stevens–Johnson syndrome (SJS). It is estimated that 80% of TEN cases are related to medication reactions; the association based on the recognition that TEN usually develops 1–3 weeks following administration of the suspect drug. It is agreed that primary treatment consists of prompt withdrawal of causative drugs and transfer to a regional burn unit. Transfer to a burn unit, no more than 7 days after onset of symptoms, has been acknowledged as reducing the risk of infections, hospital length of stay and infection-related mortality. Due to the uncertainty surrounding TEN pathogenesis, several different modalities have been proposed for the treatment of TEN, including high-dose intravenous immunoglobulins, plasmapheresis, cyclophosphamide, cyclosporine and systemic steroids; however, these therapies are relatively ineffective. The use of systemic corticosteroids for treatment of TEN has in particular been deemed controversial due to associations with increased infections leading to greater length of hospital stay and increased mortality. Granulomatosis with polyangiitis (GPA), formerly known as Wegener’s granulomatosis, is a rare relapsing-remitting disorder of unknown aetiology, characterised by granulomatosis inflammation and necrotising vasculitis predominantly affecting small- to medium-sized vessels. While a 5-year survival rate of 75–83% is now realised, relapse and associated morbidity is of concern. The established treatment for GPA follows the recommendations of the French National Authority for Health (HAS) for systematic necrotising vasculitis. With induction treatment, it is recommended that GPA be treated with a combination of systemic corticosteroids and immunosuppressants. A review of the literature failed to identify any previous case where both of these conditions coincide. Our search was conducted through databases which included MEDLINE, PubMed, Scopus, AMED, CINAHL and EMBASE, using keywords: toxic epidermal necrolysis, Wegener’s granulomatosis, granulomatosis with polyangiitis. We submit the rare case of a 22-year-old woman who presented to our regional burn unit with both GPA and TEN, and we discuss the presentation, investigation and multidisciplinary management of the patient, as well as reviewing the literature regarding these two conditions.