cutaneous necrotizing vasculitis

Multiple Myeloma (MM) is characterized by monoclonal immunoglobulin production leading to widespread skeletal destruction and renal dysfunction. Light chain multiple myeloma (LCMM) affects 15% of individuals with MM and has an overall poor prognosis. Cutaneous manifestations are uncommon and it is rarely complicated by Type I Cryoglobulinemia (CG). Here we present an atypical case of κ-predominant LCMM complicated by Type I CG in an 80-year-old man who presented with a progressive non-blanching necrotic rash and ulcers involving his face, distal extremities, and oropharynx of two months duration prior to his admission at our facility...

A 63-year-old man presented with two days of palpable purpura over the right anterior shin and calf with notable point tenderness on the distal mid-calf without any palpable deep abnormality. Localized right calf pain worsened with walking and was associated with headache, chills, fatigue, and low-grade fevers. A punch biopsy of the anterior right lower leg showed necrotizing neutrophilic vasculitis of superficial and deep vessels. Direct immunofluorescence showed non-specific focal granular deposition of C3 within vessel walls...

In December 2019, a new infectious pathogen named severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) was identified in Wuhan, China. Transmitted through respiratory droplets, SARS-CoV-2 is the causative pathogen of coronavirus disease 2019 (COVID-19). Although this new COVID-19 infection is known to cause primarily interstitial pneumonia and respiratory failure, it is often associated with cutaneous manifestations as well. These manifestations with COVID-19 can be classified into seven categories: (i) chilblain-like skin eruption (e...

Eosinophilic granulomatosis with polyangiitis is a systemic vasculitis characterized by the presence of asthma, hyper-eosinophilia, and necrotizing vasculitis with extravascular eosinophilic granulomas. We report the case of a 25-year-old male who presented to the outpatient department complaining of joint aches and numbness in the hands and legs. Physical examination revealed erythematous blanchable macular rashes on palms and soles. Raynaud's phenomenon was also observed. Lab workup revealed elevated WBC count and peripheral blood eosinophilia...

Cocaine and some of its main adulterants, such as levamisole, can cause multiple cutaneous and mucosal manifestations, including ischemic complications, neutrophilic dermatoses, midline destructive lesions, and vasculitis associated with antineutrophil cytoplasmic antibodies (ANCAs). Striking systemic symptoms are generally not seen. In all these conditions, positive test results may be observed for antinuclear antibodies, antiphospholipid antibodies, and various ANCAs, sometimes with characteristic staining patterns...

INTRODUCTION: Ocular involvement due to varicella-zoster virus (VZV) infection includes conjunctivitis, scleritis, keratitis, uveitis, and necrotizing retinitis. Non-necrotizing chorioretinopathy as a late manifestation has been described. CASE REPORT: A 50-year-old immunocompetent man developed herpes zoster ophthalmicus (HZO) in the right V1 dermatome with acute anterior uveitis (AAU) treated with oral valaciclovir and topical steroid and a chalazion in the upper eyelid with associated madarosis...

Torasemide is a loop diuretic with a molecule that is chemically similar to the sulphonamides described as eosinophilic granulomatosis with polyangiitis (EGPA) triggering drugs. The presented case is probably the first description of torasemide-induced vascular purpura in the course of EGPA. Any diagnosis of vasculitis should be followed by an identification of drugs that may aggravate the disease. A 74-year-old patient was admitted to the Department of Dermatology with purpura-like skin lesions on the upper, and lower extremities, including the buttocks...

Bullous dermatoses is a heterogeneous group of blistering skin disorders that can either be inherited or acquired. Subepidermal blisters may result in ulceration and scarring following their rupture. Wegener's granulomatosis (WG) is a granulomatous necrotizing vasculitis affecting small- to medium-sized blood vessels. It is associated with anti-neutrophil cytoplasmic antibodies (ANCA) and can be manifested cutaneously as multiple bullous and ulcers. A case of WG was reported in an 18-year-old man presented with multiple skin bullous and ulcers...

Bullosis diabeticorum (BD) is an uncommon cutaneous manifestation of diabetes that can affect the upper limbs. It is characterized by spontaneous and painless non-inflammatory bloody blisters, which can progress to necrosis, requiring differential diagnosis to rule out other dermatological diseases, such as porphyria cutanea tarda, pseudoporphyria, epidermolysis bullosa acquisita, and pemphigoid, and vascular diseases, such as vasculitis, peripheral arterial disease, and Buerger's disease, among others. In this report, we describe a 77-year-old male patient with poorly controlled diabetes and hypertension who presented with spontaneous onset of lesions on the upper limbs, initially with bullous characteristics, progressing to necrotic ulcers after spontaneous rupture...

Cocaine and some of its main adulterants, such as levamisole, can cause multiple cutaneous and mucosal manifestations, including ischemic complications, neutrophilic dermatoses, midline destructive lesions, and vasculitis associated with antineutrophil cytoplasmic antibodies (ANCAs). Striking systemic symptoms are generally not seen. In all these conditions, positive test results may be observed for antinuclear antibodies, antiphospholipid antibodies, and various ANCAs, sometimes with characteristic staining patterns...

The use of propylthiouracil (PTU) is associated with the development of autoantibodies, namely, antineutrophil cytoplasmic antibodies (ANCAs), which are associated with the pathogenesis of ANCA-associated systemic vasculitis, most often related to the myeloperoxidase subtype (ANCA-MPO). The authors report the case of a 61-year-old woman on PTU for one year who was referred to Internal Medicine for a three-month evolution of painless non-blanching purple patches, non-pruriginous, involving the chest and legs...

Amiodarone can be used in a variety of arrhythmias. Given its widespread use, the probability of clinicians encountering its cutaneous adverse effects is high. A few cases of amiodarone-induced cutaneous vasculitis were reported in the literature, probably because it is underdiagnosed in clinical practice. Indeed, amiodarone-related cutaneous reactions may present a wide range of manifestations and are sometimes difficult to diagnose. Herein, we report a case with a sizeable necrotic ulcer on the left lower leg shortly after amiodarone exposure...

INTRODUCTION: Allopurinol, widely used in the treatment of hyperuricemia and gout, has been shown to cause severe cutaneous reactions, including Stevens-Johnson syndrome and toxic epidermal necrolysis, as well as systemic reactions such as DRESS (Drug Reaction with Eosinophilia and Systemic Symptoms). The HLA-B*5801 allele is known to be a risk factor for severe cutaneous manifestations of hypersensitivity to allopurinol, mostly in Asian populations. OBSERVATION: We report the observation of a 47-year-old Chinese patient, with no previous medical history, carrying the HLA-B*5801 allele, who developed an isolated allopurinol hypersensitivity necrotizing renal vasculitis without cutaneous manifestations...

We describe the case of a 58-year-old female with an intensely painful and rapidly enlarging necrotic cutaneous ulcer to the right shin on a background of partial immunoglobulin A deficiency (IgAD). She was seen by various healthcare professionals and managed with upscaling antibiotics for cellulitis requiring an inpatient hospital stay. The dermatology team made a clinical diagnosis of ulcerative Pyoderma Gangrenosum (PG) on assessing the patient 13 days post-onset of symptoms. The patient responded dramatically to steroids and oral tetracycline...

Cutaneous leukocytoclastic vasculitis (CLV) is a vasculitis that involves mainly small blood vessels in the skin. CLV has different causes (drugs, infections, or neoplastic or systemic inflammatory diseases). Sporotrichosis has rarely been associated with CLV. We report a case of disseminated cutaneous sporotrichosis caused by microorganisms in the Sporothrix clade in a Chinese woman with a tuberculous peritonitis history. Her lesions included many ulcers with crusts on the limbs. A skin biopsy yielded a histologic diagnosis of leukocytoclastic vasculitis...

BACKGROUND AND AIM: Granulomatosis with polyangiitis (GPA) is a systemic disease that consists of vasculitis and granulomatous inflammation, and that usually affects the respiratory tract, the ear, nose, and throat sphere, and the kidneys. GPA may also cause skin manifestations that include ulcerations, nodules, or papules. An enterocutaneous fistula (ECF) is an abnormal tract that connects the skin surface to the gastrointestinal system. METHODS: We report the first case of an ECF as a concurrent clinical manifestation during a new-onset GPA in a 68-year-old male patient...

BACKGROUND: Mevalonate 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...

Eosinophilic granulomatosis with polyangiitis (EGPA) is a rare systemic vasculitis. This report describes the case of a 68-year-old female that showed up at the emergency department with extensive haemorrhagic bullous lesions, affecting elbows, the dorsal side of hands, feet and knees, with loss of tissue and necrotic areas. The evaluation led to the diagnosis of antineutrophil cytoplasmic antibody-positive EGPA with multisystem involvement: cutaneous, pulmonary, renal, intestinal and peripheral and central nervous system...

Cutaneous involvement is one of the most common extraintestinal manifestations of inflammatory bowel disease (IBD). More commonly, pyoderma gangrenosum and erythema nodosum are noted, but psoriasis, aphthous stomatitis, Sweet's syndrome, and vasculitis may also occur. Leukocytoclastic vasculitis (LCV) is a rare cutaneous manifestation, characterized by the appearance of palpable purpura, urticaria, and ulcer-necrotic lesions predominantly in the lower extremities that improve with immunosuppressive therapy...

PURPOSE OF REVIEW: The main purpose of this review is to present newly reported cutaneous manifestations of systemic vasculitis, updates in investigations to verify systemic involvement in cases with cutaneous vasculitis and new therapeutic guidelines. The spectrum of COVID-19-related vasculitis is also covered. RECENT FINDINGS: Only a few reports highlighted new cutaneous presentations or associations with some systemic vasculitic entities. For example, the association of inflammatory disorders with Takayasu arteritis, the importance of considering Kawasaki disease in febrile children with erythema nodosum, the development of necrotic ulcers on fingers and toes in Behçet's disease and the possible presence of polyarteritis nodosa-like pathological features in vulvar ulcers of Behçet's disease...