Kimura's disease of the parotid gland with cutaneous features in a Caucasian female patient

Abstract

Kimura's disease is a rare disease of unknown aetiology, commonly presenting with slow-growing head and neck subcutaneous nodules, lymphadenopathy, eosinophilia and elevated immunoglobulin E. This report describes a very rare case of a 41-year-old female, of White-British ethnicity, with a new diagnosis of Kimura's disease of the parotid gland and associated cutaneous features. The patient was investigated for 3 years before a diagnosis of Kimura's disease was reached. A superficial parotidectomy was undertaken and no recurrence was observed in the 20 months following surgery. Kimura's disease is easily misdiagnosed, owing to lack of clinical awareness. This case report highlights the troubling symptomatology as well as complexities of diagnosis and management of Kimura's disease. A high level of clinical suspicion is required, for patients of any ethnicity and sex presenting with features consistent with the disease, in order for prompt diagnosis, investigation and management to be achieved.

Figure 1:

Axial PET scans (A and B) showing increased uptake of the left parotid gland and enlarged left-sided lymph nodes in the neck.

Figure 2:

Coronal PET scan showing increased uptake of the left parotid gland and enlarged left-sided lymph nodes in the neck.

Figure 3:

Axial CT sinuses (with contrast) showing left parotid multifocal soft tissue abnormality and enlarged left-sided cervical lymph nodes.

Figure 4:

Coronal CT sinuses (with contrast) showing left parotid multifocal soft tissue abnormality (A) with enlarged left-sided cervical and superficial supraclavicular fossa lymph nodes (B).

Figure 5:

Histological examination of the parotid gland demonstrating diffuse lymphoid infiltrate with prominent follicular hyperplasia and well developed mantle zones (haematoxylin and eosin stain, ×2.5).

Figure 6:

Histological examination of the parotid gland demonstrating eosinophil-rich mixed inflammatory cell infiltrate. Prominent hyalinization of the sinusoidal vessels with thick collagen bundles within the surrounding stroma can be observed (haematoxylin and eosin stain, ×20).