[Bullous amyloïdis revealing a light chains lambda myeloma].Rev Med Interne. 2006 Sep; 27(9):694-8.RM
Although rare, skin lesions are regularly reported in patients with systemic amyloidosis. The existence of bullous skin lesions however is very rare; only thirty cases have been previously reported. We report a new case of bullous amyloidosis revealing a light chains lambda myeloma, and underline the usual characteristics of this type of systemic amyloidosis.
An 85-year-old man was hospitalised for a bullous eruption associated with a general asthenia. Bullous amyloidosis revealing a light chains lambda myeloma was diagnosed and confirmed by histopathological examination of a skin biopsy specimen. The patient died of a severe congestive heart failure, 15 days later, due to cardiac involvement of the amyloidosis.
Bullous amyloidosis lesions can be an early manifestation of occult dysglobulinemia. Early diagnosis would allow rapid treatment, before onset of systemic amyloidosis, which is often lethal.