S Albert

Guidelines for the management of pemphigus vulgaris

These guidelines for management of pemphigus vulgaris have been prepared for dermatologists on behalf of the British Association of Dermatologists. They present evidence‐based guidance for treatment, with identification of the strength of evidence available at the time of preparation of the guidelines, and a brief overview of epidemiological aspects, diagnosis and investigation.

Bullous pemphigoid antigen II (BP180) and its soluble extracellular domains are major autoantigens in mucous membrane pemphigoid: the pathogenic relevance to HLA class II alleles and disease severity

Background  Mucous membrane pemphigoid (MMP), a chronic autoimmune subepithelial blistering disease, is associated with circulating IgG and/or IgA autoantibodies against several basement membrane zone antigens. The heterogeneity of clinical presentation and diversity of target autoantigens have contributed to difficulties in characterizing this condition immunologically.

An evaluation of the usefulness of mycophenolate mofetil in pemphigus

Summary Background Pemphigus is a group of autoimmune blistering diseases of the skin and/or mucous membranes requiring management with immunosuppressive therapy. The optimal therapeutic regimen would rapidly induce remission and maintain effectiveness with minimal adverse effects in the long term.

Evaluation of a BP180-NC16a enzyme-linked immunosorbent assay in the initial diagnosis of bullous pemphigoid

Background  Bullous pemphigoid (BP) is the most common subepidermal immunobullous disease, characterized by circulating IgG autoantibodies targeting BP180 and BP230 hemidesmosomal proteins. Several immunological studies have demonstrated that the membrane proximal noncollagenous domain NC16a of BP180 is the immunodominant region targeted by BP autoantibodies. Recently, a commercial BP180 NC16a‐specific enzyme‐linked immunosorbent assay (ELISA) has become available for detecting pathogenic anti‐BP180 autoantibodies in BP sera. However, it remains unclear whether the diagnostic potential of the ELISA is equivalent to that of the ‘gold‐standard’ diagnostic technique of immunofluorescence (IF).

Pemphigoid nodularis (non-bullous): a clinicopathological study of five cases

Summary Pemphigoid nodularis is a rare clinical variant of pemphigoid characterized by overlapping clinical features of both prurigo nodularis lesions and pemphigoid‐like blisters. In pemphigoid nodularis, the immunopathological findings are identical to those of bullous pemphigoid (BP). We describe five patients who presented with the typical clinical phenotype of prurigo nodularis, who were found to have circulating and tissue‐bound antibasement membrane zone autoantibodies. By immunoelectron microscopy and Western immunoblotting studies, the circulating antibodies were shown to target the hemidesmosome and specifically the BP antigens 1 and 2 (BP180 and BP230). In contrast to the majority of reported cases, none of these patients has ever developed blisters. The role of antibasement membrane zone antibodies in the development of the eruption, or the role of the eruption in the development and persistence of autoantibodies, is not clear. These cases demonstrate that the presence of these antibodies is not sufficient for the development of blisters.

Paraneoplastic pemphigus secondary to fludarabine evolving into unusual oral pemphigus vegetans

We report a patient with chronic lymphocytic leukaemia who developed paraneoplastic pemphigus (PNP) soon after the initiation of fludarabine therapy. He presented with severe oral and cutaneous erosions. Initially, he had high titres of circulating autoantibodies as detected by indirect immunofluorescence (IF) on multiple epithelial substrates (normal human skin, monkey oesophagus, and rat bladder) and by desmoglein 1 and 3 enzyme‐linked immunosorbent assays (ELISAs). His oral erosions have subsequently progressed into unusual hyperplastic papillomatous lesions affecting the inner aspect of lips and buccal mucosae, histologically consistent with pemphigus vegetans. Desmoglein 1 antibodies and IF on rat bladder substrate have become negative after 18 months of therapy. Several agents had been initiated to bring the disease under control originally, but a partial remission was achieved and maintained with mycophenolate mofetil and low‐dose prednisolone.

Discoid lupus erythematosus with secondary amyloidosis

Background   Secondary localized cutaneous amyloidosis is a clinically unapparent phenomenon associated with various cutaneous pathologies, usually tumours of epidermal origin. The amyloid is thought to be derived from keratinocytes.

Psoriasis associated with vulval scarring

Psoriasis is a chronic inflammatory skin disorder which is generally not associated with scarring. We report two patients with long‐standing severe anogenital psoriasis, that was associated with loss of the labia minora, thus clinically mimicking the scarring associated with lichen sclerosus. Histopathological finding were however, consistent with psoriasis with no evidence of lichen sclerosus. Elastic fibres were present and there was no evidence of abnormal collagen or fibrous tissue. The association of vulval psoriasis with scarring has not been reported previously.

Lichen planus pemphigoides evolving into pemphigoid nodularis

Lichen planus pemphigoides (LPP) and pemphigoid nodularis are rare clinical variants of bullous pemphigoid (BP), which are characterized by histological findings of lichen planus (LP) and nodular prurigo, respectively, and the finding of linear deposits of IgG and/or C3 at the basement membrane zone in perilesional skin. In both cases bullae may arise at the site of pre‐existing LP‐like or nodular prurigo‐like eruptions, and clinically uninvolved skin. The disease spectrum of LPP and pemphigoid nodularis differs from that of classical BP phenotype, and their presentations are often indolent. LPP may predominantly affect a younger age group and is responsive to standard treatments used in acquired autoimmune bullous diseases, while pemphigoid nodularis is more common in elderly women and is relatively resistant to therapy. We describe a patient who had LPP for nearly two decades and subsequently developed a nodular eruption with a concurrently detected antibullous pemphigoid antigen 2 (BP180) autoantibody. His overall clinicopathological features were indicative of LPP evolving into another BP variant, pemphigoid nodularis.