A case of mistaken identity: unilateral erythema elevatum diutinum associated with IgA paraproteinaemia.

We report the case of a 27-year-old woman presenting with unilateral painless nodules of the left hand following trauma. Initially diagnosed as nontuberculous mycobacterium infection and treated with prolonged courses of antibiotics with little improvement, the condition was subsequently diagnosed histologically as erythema elevatum diutinum (EED). The lesion was treated with surgical excision, and the patient remained lesion-free at the 1-year follow-up. Although the lesion was otherwise asymptomatic, further investigation demonstrated an underlying IgA paraproteinaemia. This case should alert physicians to consider the diagnosis of EED in a unilateral presentation, and highlights the importance of investigation for underlying associated haematological disease.

Aortic stenosis in cardiovascular ochronosis.

Alkaptonuria (endogenous ochronosis) is a rare metabolic disorder caused by a deficiency of homogentisic acid oxidase, an enzyme responsible for the metabolic degradation of tyrosine. Patients with alkaptonuria commonly present with joint pain owing to degenerative arthritis. Other affected patients may present with pigmentation of the ear cartilage and sclera. This article reports a case of aortic stenosis associated with ochronosis in a 48-year-old man who presented with severe cardiac failure. He had no previous diagnosis of alkaptonuria, which was confirmed by mass spectrometry analysis of urine. The pathogenesis of cardiovascular ochronosis is unclear, but is probably related to the extensive extracellular deposits of ochronotic pigment in the cardiac tissue.

Not just another paediatric neck lump: metastatic cribriform adenocarcinoma of the palate in an adolescent.

OBJECTIVE: This paper reports the case of a 13-year-old girl with a 2-year history of left cervical lymph node swelling that was diagnosed as metastatic cribriform adenocarcinoma of the tongue and minor salivary gland. CASE REPORT: A 13-year-old girl with a left, level II cervical lymph node underwent excisional biopsy after an ultrasound suggested suspicious features. The histology indicated polymorphous low-grade adenocarcinoma, and a primary lesion in the left palate was identified. The patient underwent left maxillectomy, neck dissection and reconstruction. Histological analysis of resection specimens led to a revised diagnosis of cribriform adenocarcinoma of the tongue and minor salivary gland. CONCLUSION: Cribriform adenocarcinoma of the tongue and minor salivary gland has recently been described as a separate entity to polymorphous low-grade adenocarcinoma in light of histological and behavioural differences, including higher rates of metastasis at presentation. This is the first report in the world literature of an adolescent with this entity. It is possible that some previous reports of polymorphous low-grade adenocarcinoma in childhood would have been more accurately described as cribriform adenocarcinoma of the tongue and minor salivary gland.