Paraneoplastic Pemphigus: An Indication for Treatment in Chronic Lymphocytic Leukemia

Abstract

Paraneoplastic disorders are rare multiorgan diseases associated with hematological malignancies such as chronic lymphocytic leukemia (CLL). Some of these paraneoplasms manifest as cutaneous lesions, appearing as a simple rash, ulcers or skin thickening. The pathogenesis for this process has been described as development of certain autoimmune reactions against cell wall antigens and proteins. An example is paraneoplastic pemphigus (PNP) which manifests as cutaneous bullae. Bullae may occur anytime during the course of the malignancy i.e. acute phase or remission. Diagnosis involves evaluation of clinical findings, serology and presence of characteristic histological findings. Its pathogenesis is described as development of auto-antibodies against cell junctional and basement membrane proteins. Presence of paraneoplasms has been associated with poorer prognosis and increased mortality in hematological malignancies including CLL. Currently, there are established indications for the treatment of CLL; however, presence of paraneoplasms as an indication for treatment is unclear. Patients with paraneoplasms who underwent expeditious treatment have exhibited better clinical outcomes. Herein we describe a case of a CLL patient in remission presenting with PNP and its response to treatment.

Keywords: chemotherapy; chronic lymphocytic leukemia; paraneoplasm; paraneoplastic syndrome; pemphigoid like lesion; skin lesions.

Figure 1. The pemphigus lesions of the neck and forearm before (1 & 2) and after (3 & 4) treatment. Arrows point to lesions of interest.