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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.