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Advances in dermatology and venereology Acta Dermato-Venereologica
Eosinophilic Granulomatosis with Polyangiitis ( Churg-Strauss ) Relapsing as Bullous Eruption
Shany SHERMAN 1 # , Noa GAL 1 # , Elena DIDKOVSKY 2 , 4 , Yair MOLAD 3 , 4 , Lev PAVLOVSKY 1 , 4 and Emmilia HODAK 1 , 4
1
Department of Dermatology , 2 Institute of Pathology , and 3 Rheumatology Unit , Rabin Medical Center – Beilinson Hospital , 4941492 Petach Tikva , and 4 Sackler Faculty of Medicine , Tel Aviv University , Tel Aviv , Israel . E-mail : shanyshnush @ gmail . com Accepted Nov 8 , 2016 ; Epub ahead of print Nov 10 , 2016
Eosinophilic granulomatosis with polyangiitis ( EGPA ), also known as Churg-Strauss syndrome , is a systemic vasculitis affecting small-to-medium-size vessels . It is characterized by asthma , followed by hypereosinophilia and , in the final phase , necrotizing vasculitis with extravascular granulomas and tissue infiltration by eosinophils . Since anti-myeloperoxidase anti-neutrophil cytoplasmic antibodies ( ANCA ) are present in 30 – 40 % of patients , EGPA is classified as an ANCA-associated vasculitis ( 1 , 2 ).
Cutaneous lesions are a prominent feature of the vasculitic phase of EGPA , occurring in approximately 60 % of patients ( 3 ). Palpable purpura is the predominant cutaneous manifestation . Others include infiltrated plaques , erythematous papules , cutaneous or subcutaneous nodules , urticaria , livedo reticularis , and digital gangrene ( 4 ).
Reports of EGPA presenting as vesicles or bullae are rare and their histological characteristics and correlation between clinical , serology and histopathology findings are scarcely described ( 3 – 6 ). We describe here a patient with bullous manifestations of relapsed EGPA and the corresponding histopathological findings .
CASE REPORT
A 47-year-old man was referred by his rheumatologist to the dermatology department for evaluation . The patient described a pruritic rash that had appeared one week previously over his inner arms , which had spread rapidly to his wrists and legs . He also complained of mild dyspnoea . Past medical history was remarkable for ANCA-negative EGPA , diagnosed 2 years earlier , which manifested as perimyocarditis , recurrent asthma exacerbations , mononeuritis multiplex , recalcitrant diarrhoea , palpable purpura on the shins , and elevated blood eosinophil count of up to up to 6 × 10 3 / μl . There were histopathological findings of intestinal eosinophilic infiltrates . The patient had initially been treated with systemic steroids and cyclophosphamide , followed by courses of azathioprine and biannual rituximab .
On admission , the patient was afebrile . Physical examination revealed expiratory wheezes over both lung fields and a symmetrical blistering figurate eruption involving the axillae , inner arms , wrists , and lower limbs . The rash consisted of indurated and oedematous erythematous-to-orange plaques overlaid by grouped , clear-fluid , tense bullae and vesicles in a linear and annular arrangement at the periphery of the lesions ( Fig . 1 ). Blood tests revealed an eosinophil count of 4.9 × 10 3 / μl . Repeated cultures and polymerase chain reaction tests from blister fluid were negative for bacterial and herpetic infections . Perilesional direct immunofluorescence showed no deposition of antibodies . Biopsy from a representative bullous lesion revealed marked oedema in the papillary dermis leading to a subepidermal blister ( Fig . 2A ). Throughout the dermis there were foci of eosinophilic collagen fibres with eosinophilic deposits ( i . e . “ flame figures ”), along with
Fig . 1 . Eosinophilic granulomatosis with polyangiitis and a bullous rash . Note the vesicles overlying the oedematous plaques .
many eosinophils , few neutrophils and nuclear dust ( Fig . 2B , 2C ). At the periphery of these foci , there were variable numbers of histiocytes and occasional multinucleated giant cells ( Fig . 2C ). There was also a non-necrotizing granuloma composed of epithelioid histiocytes , multinucleated giant cells and eosinophils ( Fig . 2D ). A diagnosis of cutaneous EGPA was made and prednisone treatment was increased from a maintenance dosage of 20 mg to 40 mg for 10 days , followed by a gradual tapering down , leading to rapid clearance of the lesions .
Two months later , the patient was hospitalized due to perimyocarditis with pericardial tamponade . Pericardial biopsy showed granulomatous infiltrates and flame figures similar to the findings in the skin biopsies . After an additional 2 months of follow-up , the patient is stable and there is no recurrence of the rash .
DISCUSSION
The characteristic histopathology of cutaneous manifestations of EGPA ( 3 – 6 ) includes dermal leukocytoclastic or eosinophilic vasculitis , eosinophilic infiltrate , and granuloma formation . In our case , of ANCA-negative EGPA , histopathological study of a bullous lesion demonstrated severe dermal oedema leading to formation of a subepidermal blister , abundant dermal eosinophils , doi : 10.2340 / 00015555-2571 Acta Derm Venereol 2017 ; 97 : 406 – 407
This is an open access article under the CC BY-NC license . www . medicaljournals . se / acta Journal Compilation © 2017 Acta Dermato-Venereologica .