The temporal bone may be the first involved site in cases of systemic disease, and may even present with acute, mastoiditis-like symptomatology. This study aimed to evaluate the incidence of such non-infectious ‘acute mastoiditis’ in children.

Retrospective chart review of 73 children admitted to a tertiary referral centre for acute mastoiditis.

In 71 cases (97.3 per cent), an infectious basis was identified. In the majority of cases (33 of 73; 45 per cent), the responsible bacteria was Streptococcus pneumoniae. However, histopathological studies revealed a non-infectious underlying disease (myelocytic leukaemia or Langerhans' cell histiocytosis) in two atypical cases (2.7 per cent).

‘Acute mastoiditis’ of non-infectious aetiology is a rare but real threat for children, and a challenging diagnosis for otologists. A non-infectious basis should be suspected in every atypical, persistent or recurrent case of acute mastoiditis.

We report an extremely rare case of Langerhans' cell histiocytosis involving isolated cervical lymph nodes, and we discuss the diagnosis and treatment of this infrequent disease.

We present a case report and literature review concerning this disease entity.

A 54-year-old man presented with persistent, multiple, left neck masses. Histopathological study of lymph node specimens showed proliferation of Langerhans' cells coupled with eosinophilic and neutrophilic microabscesses. Positive immunohistochemical staining for Cluster of Differentiation 1a (CD1a) in Langerhans' cells confirmed the diagnosis. No other abnormalities were noted on a series of image studies. The patient was managed with ‘watchful waiting’ without subsequent therapy. The neck lesions regressed gradually over time, and the patient did well over a two-year follow-up period.

The clinical presentation of Langerhans' cell histiocytosis is highly variable, and the choice of treatment depends on the involved organs. Definitive diagnosis depends on identification of characteristic immunohistochemical or ultrastructural features of the biopsy specimen. Watchful waiting may be an effective management strategy in cases of adult isolated lymph node Langerhans' cell histiocytosis, due to its possible spontaneous regression.