Atypical herpes simplex infection masquerading as recalcitrant pemphigus vulgaris

Abstract

A 57-year-old woman presented with refractory genital erosive disease. One year earlier she experienced gingival fragility; direct immunofluorescence resulted in the diagnosis of cicatricial pemphigoid, and prednisone therapy led to initial improvement. Initial skin biopsy of her genital erosions demonstrated full-thickness ulceration with viral cytopathic change and a re-epithelializing subepidermal separation. Indirect immunofluorescence revealed intercellular IgG staining on monkey oesophagus at a titre of 1:320 consistent with pemphigus, leading to the diagnoses of pemphigus vulgaris with herpetic superinfection. Immunosuppressive treatment initially led to improvement; however, disease subsequently recurred as extensive genital erosions. We diagnosed atypical herpes simplex virus infection and oral candidiasis, discontinued all immunosuppressive medications, and initiated antiviral and antifungal therapy. Dramatic resolution was observed and the patient has remained free of disease for 13 months while taking only prophylactic famciclovir.

Refractory pemphigus vulgaris associated with herpes infection: case report and review

“…It is also relevant to observe that the majority of PV patients are treated with immunosuppressive therapy, which favors viral opportunistic infections 4 , often with atypical clinical presentation, as described by HALE & BYSTRYN 10 and KALAJIAN & CALLEN 11 Several cases of HSV as a trigger or as something which worsens PV have been described in literature (Table 1). It is observed that, in such cases, the complete remission of lesions has only occurred after introducing antiviral treatment, in the majority of cases, acyclovir.…”

Pemphigus Foliaceus with Herpes Simplex Virus;A Case Report

“…They found significant prevalence of HSV1 in lesions of pemphigus patients, especially in pemphigus foliaceus [13]. There have been several case reports of pemphigus with associated herpes virus infection mostly seen as a complication of immunosuppressive therapy as in our case [14][15][16][17][18][19][20][21] [ Table 2]. …”

Laboratory Diagnosis of Herpesvirus Infections in Patients with Pemphigus Vulgaris Lesions

“…Conversely, other studies suggested that herpesviruses could trigger PV progression [2,3,7,8,9]. In addition, other studies have suggested that herpesvirus infections represented a complication of pemphigus resulting from immunosuppressive therapies and/or a causative factor associated with treatment-resistant lesions [6,10,12,13,14,15,16,17,22,23,24,25,26]. …”

“…The most common viral infections reported in pemphigus patients are caused by herpesviruses, particularly HSV [6,10,11,12,15,23,24,25,26,28,29,30]. HSV infections are often identified in association with recurrent/persistent PV lesions in patients already undergoing corticosteroid treatment.…”

“…HSV infections are often identified in association with recurrent/persistent PV lesions in patients already undergoing corticosteroid treatment. In most cases, HSV diagnosis was delayed for several months, because viral lesions mimicked PV lesions [12,22,25,26]. However, when the HSV diagnosis was established and the treatment was initiated, the lesions cleared promptly.…”