The Red Puffy Ear Sign - A Clinical Sign to Diagnose a Rare Cause of Meningoencephalitis

Fortschritte der Neurologie-Psychiatrie (Jul 2012)

Prinz S, Dafotakis M, Schneider RK, Mühlenbruch G, Stopschinski BE 

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• azathioprine

We report on the case of a 63-year-old patient with a meningoencephalitis, presenting itself with headache and a general neuropsychological retardation. Additionally, a reddening and swelling of both auricles could be seen. Magnetic resonance imaging showed confluent, contrast-uptaking lesions. In the cerebrospinal fluid an aseptic lymphocytic pleocytosis was found. A biopsy of the ear revealed a chronic lymphoplasmacellular inflammatory reaction. We diagnosed a relapsing polychrondritis, an inflammatory disease of the cartilage, which can in rare cases affect the central nervous system. Typically, the patients complain about red swollen ears, the"red puffy ear sign". After initiation of steroid and azathioprin therapy the patient recovered fully.

Mucocutaneous leishmaniasis masquerading as Wegener granulomatosis

JCR: Journal of Clinical Rheumatology 16 (3), 125-8 (Apr 2010)

 

Brahn E, Pegues DA, Yao Q, Craft N;

 

A 43-year-old Brazilian female presented in 2001 with nasal stuffiness and sinusitis. A biopsy was consistent with limited Wegener's granulomatosis although antineutrophil cytoplasmic antibodies were negative. Her nasal inflammation progressed despite trials of prednisone, methotrexate, and azathioprine. A septal perforation developed and a repeat biopsy showed granulomatous inflammation. In 2006 the patient was referred to Division of Rheumatology, University of California, Los Angeles. 

The nose was grossly erythematous and a magnetic resonance imaging revealed nasal destruction and sinusitis. Palatine biopsies showed chronic inflammation. Cyclophosphamide at 150 mg/d resulted in markedly improved mucocutaneous lesions. The patient developed a leg and arm rash in 2007. A skin biopsy was positive for Leishmania braziliensis. The cyclophosphamide was discontinued and amphotericin B was initiated with transient benefit. Remission was achieved with pentavalent antimony. 

Despite multiple nasopharyngeal biopsies, for a 6-year span, mucocutaneous leishmaniasis masqueraded as Wegener's granulomatosis. Cyclophosphamide not only resulted in clinical improvement, due to reduced inflammatory response, but also allowed widespread cutaneous dissemination.

Autoimmune sialadenitis

Hals-Nasen-Ohrenheilkunde (HNO) (Mar 2010)

 Guntinas-Lichius O, Vissink A, Ihrler S

Using the European-American classification criteria the diagnosis of autoimmune sialadenitis in Sjögren's syndrome can generally be easily established or excluded. In addition, sonography performed by the ENT physician is helpful in diagnosing and especially in follow-up screening for MALT lymphomas, which 5%-10% of patients develop. Therapy of sicca symptoms is primarily symptomatic using substitution with fluids and stimulation with oral cholinergic drugs. Corticosteroids and/or antibiotics may play a role in patients with severe inflammatory episodes of autoimmune sialadenitis. Systemic therapy with immunomodulatory drugs such as azathioprine or cyclophosphamide is reserved for patients with extraglandular manifestations. However, the efficacy of this therapy is not proven by clinical studies. Rituximab, a new monoclonal CD20 antibody, seems to offer the first possibility of a causal therapy, under which the lymphoepithelial lesions in the salivary glands can disappear and saliva production improves. However, larger clinical studies are needed to evaluate the efficacy of this new therapy.

Optimal treatment of autoimmune sialadenitis requires interdisciplinary collaboration between ENT physician, oral and maxillofacial surgeon, rheumatologist, ophthalmologist, dentist, and pathologist.