Ecthyma-Gangrenosum-Like Bullous Pemphigoid

Abstract

Bullous pemphigoid (BP) is a chronic, autoimmune, subepidermal blistering skin disease with varied clinical presentations. Diagnosis is based on the clinical picture, histopathological findings, and direct and indirect immunofluorescence studies. In unclear cases, ELISA or Western blot analysis helps to establish a definite diagnosis by the detection of immunoglobulin G autoantibodies specific for the hemidesmosomal BP antigens BP230 and BP180. We report 3 cases of BP with an as yet not characterized, distinctive ecthyma-gangrenosum-like presentation. Patients were female, above 80 years of age, physically immobile, and skin lesions showed truncal localization and bacterial colonization. Factors contributing to physical immobility were a high body mass index, psychiatric disease, sedative medication and rheumatic disease. The clinical picture resembled ecthyma gangrenosum but lacked systemic infection with Pseudomonas aeruginosa. Lesional bacteriological studies revealed Staphylococcusaureus and/or P. aeruginosa. Diagnosis proved challenging in all cases. Suspicion has to be high, and repeated diagnostic procedures and additional laboratory studies may be necessary to establish a definitive diagnosis of BP. In summary, we propose this combination of truncal ecthyma-gangrenosum-like lesions with bacterial colonization in the context of older age and immobility as a clinically distinct presentation or variant of BP.

The Value of BP230 Enzyme-Linked Immunosorbent Assay in the Diagnosis and Immunological Follow-Up of Bullous Pemphigoid

“…Following French national recommendations for the diagnosis of BP [2], immunological tests are recommended but often not necessary for the diagnosis of typical situations. In contrast, BP180 and BP230 ELISA were reported as useful for the diagnosis of atypical BP, especially BP with atypical cutaneous presentation [14] or BP with mucosal lesions that are difficult to distinguish from mucous membrane pemphigoid [11]. However, it was shown that BP230 ELISA may be positive in some cases of mucous membrane pemphigoid and may not replace electronic immunomicroscopy as a definitive discriminant test [15,16].…”

‘Bullous’ Pemphigoid: What Are You? Urgent Need of Definitions and Diagnostic Criteria

“…IIF studies, ELISAs or Western blot) [1,2,[6][7][8][9] , the presence of a seroreactivity should always be interpreted according to the clinical context and the results of DIF studies (the only exception is gestational pemphigoid, in which strong positive BP180 ELISA values may replace DIF studies [10] ). In patients with atypical clinical findings, such as those reported by Geiss Steiner et al [3] , in our view the presence of circulating autoantibodies by either ELISA or IIF studies does not allow to make a diagnosis of BP if DIF microscopy studies are negative. Nevertheless, these cases have at least the merit to remind us of the importance to consider a non-bullous, 'atypical' variant of an AIBD in elderly patients with chronic itchy skin disorders, even in those in whom almost nothing else suggests this diagnosis a priori.…”

“…In this issue of Dermatology, Geiss Steiner et al [3] report 3 elderly female patients with a long history of widespread fibrinonecrotic ulcerations on the trunk and the limbs. Unexpectedly, some immunopathological findings suggested a diagnosis of BP.…”

Evaluation of clinical and histopathologic/direct immunofluorescence diagnosis in autoimmune vesiculobullous dermatitis: utility of direct immunofluorescence

“…According to our results concerning BP, DIF microscopy seems to be a useful diagnostic method for definitive diagnosis. In a study of Libsker and Borradori (8), authors evaluated atypical variants of BP which were described by Geiss Steiner et al (9). Author's view about these atypical variants was that the presence of circulating autoantibodies by either ELISA or indirect IF studies did not allow to make a diagnosis of BP if DIF microscopy is negative (8).…”