Recurrent Erysipelas in Myotonic Dystrophy Type 1 with IgG Deficiency

Abstract

mal creatine kinase. Clinical cardiological examination and echocardiography were normal but electrocardiography showed arteriovenous block I and left anterior hemiblock. Molecular genetic analysis revealed a CTG expansion of 130 repeats in the DMPK gene. The diagnosis of DM1 was established. After the last episode of gluteal erysipelas in September 2005, she underwent a long-term oral penicillin therapy for 6 months until February 2006. Dear Sir, Dystrophia myotonica type 1 (DM1), the most common muscular dystrophy in adults, is a hereditary, autosomal dominant, multi-system disease, caused by a CTG repeat expansion in a non-coding region of the dystrophia myotonica protein kinase (DMPK) gene on chromosome 19q13.3 [1, 2] . DM1 is frequently associated with IgG deficiency [3] , which is independent of the CTG repeat size [4] . Here we report a DM1 patient with IgG deficiency and recurrent erysipelas. The patient is a 61-year-old HIV-negative female with a history of recurrent erysipelas, occurring 4 times during the last 5 years. The erysipelas developed twice in the face (2001 and 2002) and twice in the gluteal region (2003 and September 2005). Her relatives reported increasing cognitive impairment and social regression during the last 6 years. In February 2005 she was admitted because of gait disturbance from weakness of the distal lower limbs. Clinical neurological examination revealed ptosis, dysarthria, weakness for foot extension (M5–), wasting of the distal upper and lower limb muscles, clinical myotonia and absent Achilles tendon reflexes. Nerve conduction studies were normal but electromyography revealed myotonic discharges and myogenic motor unit architecture. Ophthalmological examination showed incipient cataract bilaterally. Blood chemical investigations revealed slightly elevated glutamate oxalate transaminase but norReceived: June 26, 2006 Accepted: October 14, 2006 Published online: March 26, 2007