Hashimoto's encephalopathy in a patient with septal panniculitis: a case report.

Hashimoto's encephalopathy (HE) is an autoimmune form of encephalopathy, associated with autoimmune thyroiditis. Its prevalence is estimated to be 2:100,000. HE is characterized by behavioral changes, mental confusion, dysarthria, ataxia, psychosis, paranoia, convulsions, hallucinations, headache and hyperthermia. Elevated thyroid antibodies are necessary for diagnosis and the disease responds dramatically to glucocorticoid therapy. We describe a patient with HE and panniculitis, an association reported twice in the literature.

Seronegative spondyloarthritis and Darier's disease: more than a casual association?

A 46-year old man, affected by Darier's disease (DD), was seen because of right hand pain, later extended to shoulders and ankles. Physical examination showed swelling and tenderness of the wrist, metacarpophalangeal and metatarsophalangeal joints, of the right Achilles tendon's enthesis and of the left knee, with psoriatic-like lesions of the scalp. A diagnosis of seronegative spondyloarthritis, supported by HLA-B27 positivity and by the magnetic resonance imaging finding of hand synovitis and unilateral sacro-iliitis, was made. The correlation between DD, spondyloarthritis and psoriasis has been already anecdotally reported. Further observations may clarify if this association is more than casual.

Secondary hemophagocytic lymphohistiocytosis possibly induced by interferon beta-1a therapy.

A 57-year old woman with a history of multiple sclerosis, treated with interferon beta-1a in the last 5 months, was referred for hyperpyrexia (>40°C) that persisted for 15 days. At admission, there was elevation of transaminases, anemia (hemoglobin 8.9 g/dL), thrombocytopenia (platelet 135,000/mm3), and hypofibrinogenemia (fibrinogen 1.26 g/L). C-reactive protein was 10.7 mg/dL, lactate dehydrogenase 1270 U/L and ferritin 2380 ng/ mL, with hepatosplenomegaly and linfoadenomegaly. Hemophagocytic lymphohistiocytosis induced by direct stimulation of macrophages by interferon (IFN) was suspected. IFN was withdrawn as only measure and onemonth later signs and symptoms disappeared, with complete normalization of laboratory examinations.