Extensive haemorrhagic-bullous skin manifestation of systemic AA-amyloidosis associated with IgGlambda-myeloma.

Abstract

In an 86-year-old woman with a multiple myeloma of the IgG lambda subtype a coinciding systemic amyloidosis manifested as a macroglossia, diffuse alopecia and generalized cutaneous involvement. The skin was affected by milium-like papules, petechial haemorrhages and an increased tissue fragility with subsequent blister formation. The typical histology and immunohistology pattern revealed large intradermal amyloid masses, reacting positively with anti-amyloid A antibodies, which surrounded cuff-like dilatated blood capillaries. The abundance of these amyloid deposits led to significant deflexibilization and fragility of the capillaries and the dermal matrix eventually resulting in the haemorrhagic-bullous eruptions. The peculiar feature of the present case is the intensity of bullous-haemorrhagic skin damage due to amyloid A deposition without any detection of cutaneous IgGl as the myeloma-derived paraprotein assumed to be causative for the development of systemic AA amyloidosis.

• Authors: J U Grundmann, B Bonnekoh, H Gollnick
• Journal: European journal of dermatology : EJD (Eur J Dermatol) Vol. 10 Issue 2 Pg. 139-42 (Mar 2000) ISSN: 1167-1122 [Print] France
• PMID: 10694315 (Publication Type: Case Reports, Journal Article)

Chemical References

• Immunoglobulin G
• Immunoglobulin lambda-Chains
• Serum Amyloid A Protein

Topics

• Aged
• Aged, 80 and over
• Alopecia (etiology)
• Amyloidosis (complications, pathology)
• Capillary Fragility
• Ecchymosis (etiology)
• Female
• Follow-Up Studies
• Humans
• Immunoglobulin G (analysis)
• Immunoglobulin lambda-Chains (analysis)
• Macroglossia (etiology)
• Multiple Myeloma (complications)
• Purpura (etiology)
• Serum Amyloid A Protein (analysis)
• Skin (blood supply)
• Skin Diseases (complications, pathology)
• Skin Diseases, Vesiculobullous (etiology)