Sheng-Yiao Lin

Shiang Yu Yang

Stephen Chu-Sung Hu

English

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ACTA DERMATOVENEROLOGICA CROATICABullous Pemphigoid Masquerading as Erythema  Annulare CentrifugumActa Dermatovenerol Croat                                 2017;25(3):255-256                          Letter to the eDitorDear editor,Bullous pemphigoid (BP), a relatively common au-toimmune blistering disease in the elderly, is charac-terized by large, tense bullae on urticarial, erythema-tous, or normal skin. however, atypical BP with poly-morphic clinical presentations is rarely encountered, leading to misdiagnosis and delayed treatments (1). BP with lesions resembling erythema gyratum repens or figurate erythema has been regarded as a paraneo-plastic phenomenon (1). herein we report a case with erythema annulare centrifugum-like presentation of BP without evidence of underlying malignancy.A 64-year-old woman first presented with multiple large, tense bullae on the trunk and four extremities. She was diagnosed with BP according to the typical clinical, histopathological, and direct immunofluores-cence findings. there were no annular lesions at that time. After a treatment course of systemic cortico-steroids and azathioprine, the cutaneous symptoms were controlled. one year after discontinuing her medications, a pruritic bullous eruption reappeared with several annular erythematous plaques (Figure 1, a). the patient reported no mucosal involvement and took no new medications before the onset of skin lesions. on physical examination, multiple cir-cular and arcuate erythematous lesions with slightly raised borders were seen on the trunk and both legs. Some erosions and tiny vesicles were noted on the erythematous edges. there were no other systemic symptoms or abnormalities. Laboratory studies, in-cluding complete blood count, liver and renal func-tion tests, electrolytes, antinuclear antibody, comple-ment levels, anti-ro and anti-La antibodies, urine routine, stool routine, and chest X-ray, were normal. the biopsy specimen obtained from the rim of the Figure 1. (a) Several annular and arcuate erythematous lesions on the trunk. there are some erosions on slightly raised bor-ders. (b) Skin biopsy showed slight vacuolar change at the basement membrane zone with perivascular and interstitial infil-tration of lymphocytes and eosinophils in the upper dermis (hematoxylin and eosin, ×100). (c) Direct immunofluorescence demonstrated linear deposition of C3 along the dermoepidermal junction.255256 ACTA DERMATOVENEROLOGICA CROATICA  Acta Dermatovenerol CroatLetter to the editor   2017;25(3):255-256annular lesions revealed slight vacuolar change at the dermoepidermal junction and perivascular and interstitial lymphocytic infiltration with numerous eosinophils in the upper dermis (Figure 1, b). Direct immunofluorescence showed linear deposits of im-munoglobulin G (igG) and C3 along the basement membrane (Figure 1, c). histopathological features and immunofluorescence examinations were consis-tent with BP. there was no evidence of hematological or solid malignancy from further imaging and labora-tory testing.the patient was started on oral prednisolone 30 mg/day and azathioprine 150 mg/day, with signifi-cant improvement over the following month. Com-plete regression of all skin lesions was achieved two months later, so the prednisolone dose was gradually tapered and then ceased. Under maintenance mono-therapy of azathioprine 100 mg/day, there were no signs of BP recurrence or malignant disease during the one-year follow-up period.the annular erythema variant of BP is extremely rare. therefore, in this case, erythema multiforme, subacute cutaneous lupus erythematosus, erythema annulare centrifugum, and urticarial vasculitis should be considered in the clinical differential diagnoses. Pathological features and immunofluorescence re-sults can clearly rule out these possibilities. Until now, only 13 cases of BP presenting as annular erythema had been documented in the english literature, de-scribed as figurate erythema-like, erythema gyratum repens-like, or erythema annulare centrifugum-like manifestations (1-3). An association with internal ma-lignancy in patients with these types of lesions had been reported (1). Nevertheless, as in most previous case reports (3), malignant diseases were not found in our patient.the precise mechanism of the annular erythema form of BP is unknown. Some authors considered it a variant of pre-bullous phase lesions, usually pre-senting as itchy erythematous patches or urticarial plaques (4). Based on this case, however, this assump-tion is less likely because the annular, erythema an-nulare centrifugum-like skin lesions appeared one year after the initial onset of bullous eruption, and simultaneously with the exacerbation of the bullous phase of BP. the exact pathogenesis of annular BP may be similar to that in erythema annulare centrifu-gum. Further investigations are warranted to clarify this issue.it should be noted that an erythema annulare centrifugum-like or figurate erythema-like manifes-tation in the absence of underlying malignancy can occasionally be a feature of BP. Making the correct di-agnosis may be difficult if there is no concurrent bul-lous presentation. Clinicians should be vigilant for the development of this type of BP. the histological and direct immunofluorescence findings and the detec-tion of circulating autoantibodies by indirect immu-nofluorescence or enzyme-linked immunosorbent assay remain crucial tools for establishing a definitive diagnosis.References:1. Cozzani e, Gasparini G, Burlando M, Drago F, Pa-rodi A. Atypical presentations of bullous pemphi-goid: Clinical and immunopathological aspects. Autoimmun rev 2015;14:438-45.2. Di Zenzo G, Della torre r, Zambruno G, Borra-dori L. Bullous pemphigoid: from the clinic to the bench. Clin Dermatol 2012;30:3-16.3. Dolenc-Voljč M, Žgavec B, Vizjak A, tenyi V, Luzar B. Figurate erythema in a patient with bullous pemphigoid and toxocara infection. Acta Derma-tovenerol Alp Pannonica Adriat 2013;22:87-9.4. Mussani F, Siddha S, rosen CF. Figurate erythe-ma: an unusual presentation of the nonbullous phase of bullous pemphigoid. J Cutan Med Surg 2012;16:61-3.Shiang-Yu Yang1,2, Stephen Chu-Sung Hu1,2, Sheng-Yiao Lin1,3 1Departments of Dermatology, Kaohsiung Medical University Hospital, Kaohsiung, Taiwan2Departments of Dermatology, College of Medicine, Kaohsiung Medical University, Kaohsiung, Taiwan3Department of Dermatology, Kaohsiung Municipal Ta-Tung Hospital, Kaohsiung, TaiwanCorresponding author:Sheng-Yiao LinNo. 100, tzyou 1st road, Kaohsiung 807, taiwanshengyiao@gmail.comreceived: April 2, 2017Accepted: July 21, 2017

Bullous pemphigoid masquerading as erythema annulare centrifugum

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