Successful intravenous immunoglobulin treatment of atypical lichen myxedematosus associated with hypothyroidism and central nervous system involvement: case report and discussion of the literature.
BACKGROUND
Lichen myxedematosus (LM) is a rare idiopathic disorder characterized by papules, plaques, and/or nodules in the skin secondary to mucin deposition and variable dermal fibrosis in the absence of thyroid disease. Case reports are the predominant literature on LM due to its rarity, and the data are limited regarding the disease. No standard treatment regimen exists, and the response to treatment varies.


OBJECTIVE
This report adds to the limited literature on atypical LM associated with thyroid dysfunction and contributes to the growing body of evidence supporting intravenous immunoglobulin (IVIg) as effective therapy for extensive cases.


RESULTS
We report an unusual case of atypical LM associated with hypothyroidism, central nervous system (CNS) disturbances, and atrial fibrillation in a 64-year-old male. The patient experienced remarkable improvement within 3 months of beginning IVIg treatment; however, he required repeat therapy due to the recurrence of CNS symptoms 3 months after treatment.


CONCLUSIONS
We agree with previous authors that thyroid dysfunction alone should not preclude a diagnosis of LM. Additionally, we contribute to the increasing evidence of IVIg being an effective treatment, particularly in the setting of systemic complications or acute worsening of LM.
Department of Dermatology, University of British Columbia, Vancouver, BC, Canada.
Magnus
M
Macnab
Patrick
P
Kenny
D001281:Atrial Fibrillation / Q000150:complications; D003072:Cognition Disorders / Q000150:complications; D020405:Dermis / Q000473:pathology; D006362:Heavy Chain Disease / Q000150:complications; D006801:Humans; D007037:Hypothyroidism / Q000150:complications; D016756:Immunoglobulins, Intravenous / Q000627:therapeutic use*; D007155:Immunologic Factors / Q000627:therapeutic use*; D008297:Male; D008875:Middle Aged; D053718:Scleromyxedema / Q000150:complications / Q000175:diagnosis / Q000503:physiopathology / Q000628:therapy*
D016756:Immunoglobulins, Intravenous; D007155:Immunologic Factors