Paraneoplastic pemphigus. A report of two cases associated with chronic B-cell lymphocytic leukaemia

Abstract

Paraneoplastic pemphigus (PNP) is an autoimmune blistering and erosive mucocutaneous disease associated with neoplasia. Clinical manifestations are polymorphous, and include erythema, bullae, erythema multiforme-like lesions and severe mucous membrane involvement. PNP manifesting as lichenoid dermatitis has recently been observed. We describe two Italian men with fatal PNP featuring typical PNP autoantigens associated with chronic B-cell lymphocytic leukaemia. The first patient presented with an extensive blistering eruption, several erythema multiforme-like lesions and severe mucosal involvement. The second patient presented with a lichenoid dermatitis, then developed bullae, and died with an erythrodermic and exfoliative dermatosis resembling pemphigus foliaceus. Our patients represent two Italian cases of well-documented PNP. In patient 2, the sequence of clinical presentations was unique, and strongly supports the hypothesis of epitope spreading through chronic lichenoid inflammation of the dermo-epidermal junction exposing new self antigens, leading to the humoral response characteristic of PNP.

Paraneoplastic pemphigus with negative direct immunofluorescence in epidermis or mucosa but positive findings in adnexal structures

“…In the first report of this entity, Anhalt et al 2 emphasized that the IgG deposits in the intercellular spaces were often faint and focal by DIF. Later publications by both Anhalt and Kimyai‐Asadi commented on the negative results of the DIF test in PNP, 5,6 findings that have since been corroborated by other authors 7–11 …”

“…One of these was the presence of immunoglobulin G (IgG) and complement in the epidermal intercellular spaces associated with granular‐linear complement deposition along the epidermal basement membrane zone on direct immunofluorescence (DIF) test 2 . Diagnostic criteria have since been expanded, 3,4 however, and some authors have reported negative DIF results in PNP patients 4–11 . We present three PNP patients who had negative epithelial DIF findings in one or more biopsies, while IgG and/or C3 were observed at the intercellular level in cutaneous adnexa in two cases.…”

A novel case of IgA paraneoplastic pemphigus associated with chronic lymphocytic leukemia

“…The reported patient was also found to have underlying CLL, a common associated malignancy in PNP. 5,6 Using the criteria of Camisa and Helm, 4 this patient met the requirements for a diagnosis of PNP.…”

Toxic epidermal necrolysis

“…For example, lichenoid lesions may be present alongside bullous lesions. 77 The lesions of TEN are monomorphous. Acantholysis, a cardinal feature of PNP, is not seen in TEN; and DIF is positive in PNP, but negative in TEN.…”