wegener s granulomatosis ( wg ) is an uncommon necrotizing vasculitis that most commonly affects the upper airways , lungs and kidneys , but can involve any other organ .
the disease presents with varying symptoms and signs and early recognition and initiation of adequate immunomodulatory therapy are essential in limiting the potentially life - threatening aspects of the disorder .
we report a case illustrating that wg may present with localized , cutaneous manifestations preceding systemic disease .
an 18-year old caucasian man presented with a painful rash on the truncus that had developed over the course of a few weeks .
the patient was known to have type-1 diabetes mellitus since the age of 8 , but was otherwise healthy and reported no recent travel activity or antecedent trauma to the affected area .
objective examination of the skin revealed inflamed cystic and nodular lesions confined to the chest and left shoulder , but there was no significant suppuration or other apparent signs of infection .
the condition resembled a case of severe acne , and initial histopathological examination of a skin biopsy supported this preliminary clinical diagnosis .
a few weeks later the patient developed a sore throat and signs of upper respiratory tract infection including cough , dyspnoea and mild chest pain .
the skin changes remained limited to the chest and shoulder but had clearly progressed and now appeared as deep vasculitic ulcerations , including multiple elements more than 0.5 cm deep ( figure 1 ) .
these findings and symptoms were accompanied by several weeks of fever ( up to 39.6c ) , malaise and substantial unilateral facial pain , and the patient was admitted to a department of nephrology for further investigation and treatment . 
 figure 1cystic and nodular lesions on the chest , notice necrotic wounds with element size ranging from a few millimeters up to 2 cm . in consistence with vasculitis the elements are inflamed and do not blanch on pressure . 
 
cystic and nodular lesions on the chest , notice necrotic wounds with element size ranging from a few millimeters up to 2 cm .
the blood leukocyte and thrombocyte counts were normal as were liver and renal function tests , but c - reactive protein ( crp ) was 300 mg / l ( normal range , less than 10
mg / l ) and anti - neutrophil cytoplasmic antibodies including specific identification of proteinase 3 ( c - anca / pr3-anca ) was 223 ku / l ( normal range , less than 7
the patient s kidney function was continually assessed and monitored , but levels of creatinine and urea remained within normal ranges and examination of urine including microbiological analysis revealed no abnormalities .
chest x - ray examination was performed and several lung infiltrates with caverns were noted .
a subsequent ct guided lung biopsy demonstrated no sign of infection , but significant inflammation of the tissue was observed , and a diagnosis of wg with concomitant mononeuritis multiplex involving the trigeminal nerve was established .
the patient received systemic methylprednisolone and cyclofosfamide pulse therapy , which resulted in prompt improvement of the clinical condition and skin lesions as well as a decrease in the crp and anca titers .
shortly after , however , the patient developed fever and renewed elevation of the c - anca / pr3-anca , and treatment was supplemented with rituximab leading to immediate resolution of symptoms and no residual pulmonary or cutaneous sequelae on recent follow - up .
our patient initially presented with a localized rash on the truncus , but his condition was insidiously complicated by malaise , fever and progression of the cutaneous lesions , and ultimately a diagnosis of systemic wg was established .
the etiology of the disease remains unknown , however , several studies suggest that infectious antigens and especially s. aureus may contribute to the pathogenesis of vasculitis in susceptible hosts . the clinical presentation of wg is complex and found to be dependent on the number of organs affected and the duration of the disease . as in our case ,
the clinical course of wg is characterized by an initial or localized phase which may affect any organ followed by a generalized or systemic phase in approximately 80% of cases . however , in most patients specific cutaneous findings develop concurrently or after the onset of systemic involvement and affect the head or extremities , but in our case they were confined to the truncus and preceded systemic symptoms and signs , which has only seldomly been described . in wg
specific cutaneous lesions are seen in approximately 15% of cases , and are usually associated with renal disease and musculoskeletal affection , but this was not noted in our patient .
patients may develop more than one type of cutaneous lesion and these may change over time or with treatment , but several studies have identified palpable purpura as the most characteristic cutaneous lesion , and nodules , papules , ulcerations and deep erythema nodusum - like subcutaneous nodules complete the clinical spectrum . oral involvement is not uncommon in wg , but this was not observed in our patient during the course of the disease . in this present case
the level of c - a nca / pr3-anca was markedly elevated , and anca positivity has been found to occur in 9095% of cases with active wg . however , as levels correlate with disease activity and relapses in the absence of anca are rare , repeat testing and evaluation of blood biochemistry in order to follow the course of the disease is highly recommended .
as illustrated by our case the diagnosis of wg represents a challenge and is based on anamnestic information closely correlated with clinical features , pathologic findings and anca testing .
relevant differential diagnoses are numerous and include leukocytoclastic vasculitis , henoch - schnlein purpura , pyoderma gangrenosum , lymphoma , erythema nodosum , rheumatoid arthritis , drug reactions , and a variety of infectious conditions . however , like wg , these conditions often present with varying and uncharacteristic symptoms and signs and , as in our case , dermatological evaluation may provide valuable diagnostic information .
if untreated , systemic wg leads to more than 90% mortality in the first two years , hence early recognition and initiation of adequate immunomodulatory treatment are essential and associated with significantly decreased morbidity and mortality .
high dose corticosteoroids and pulsed intravenous cyclofosfamide remain the mainstay of initial therapy , but relapse and refractory wg still represent therapeutic challenges .
however , data from recent studies evaluating biological therapies , including tnf- blockers ( infliximab ) and monoclonal antibodies ( rituximab ) are promising and future treatment regimes will hopefully further improve the outcome of this patient group .
we report a case illustrating that patients with wg may initially present with localized cutaneous symptoms and signs preceding serious systemic disease .
prompt recognition of the condition and initiation of early and adequate immunomodulatory therapy is crucial in order to reduce mortality and morbidity .
dermatologists therefore need to be aware of wg as a possible differential diagnosis , especially in patients presenting with characteristic skin manifestations and accompanying systemic symptoms and signs .
 wegener s granulomatosis ( wg ) is a rare , systemic vasculitis involving multiple organs . 
 the clinical presentation is highly diverse , and there is considerable risk of mortality if diagnosis and treatment are delayed . 
 we present a case illustrating that patients with wg may initially present with localized cutaneous symptoms and signs . 
Introduction
Case Report
Discussion