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Extensive haemorrhagic-bullous skin manifestation of systemic AA-amyloidosis associated with IgGlambda-myeloma.
Eur J Dermatol. 2000 Mar; 10(2):139-42.EJ

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+Show Affiliations

Grundmann JU
Department of Dermatology and Venereology, Otto-von-Guericke University, Leipziger Stasse 44, D-39120 Magdeburg, Germany. Jens-Uwe.Grundmann@medizin.uni-magdeburg.de
Bonnekoh B
No affiliation info available
Gollnick H
No affiliation info available

MeSH

AgedAged, 80 and overAlopeciaAmyloidosisCapillary FragilityEcchymosisFemaleFollow-Up StudiesHumansImmunoglobulin GImmunoglobulin lambda-ChainsMacroglossiaMultiple MyelomaPurpuraSerum Amyloid A ProteinSkinSkin DiseasesSkin Diseases, Vesiculobullous

Pub Type(s)

Case Reports
Journal Article

Language

eng

PubMed ID

10694315

Citation

Grundmann, J U., et al. "Extensive Haemorrhagic-bullous Skin Manifestation of Systemic AA-amyloidosis Associated With IgGlambda-myeloma." European Journal of Dermatology : EJD, vol. 10, no. 2, 2000, pp. 139-42.
Grundmann JU, Bonnekoh B, Gollnick H. Extensive haemorrhagic-bullous skin manifestation of systemic AA-amyloidosis associated with IgGlambda-myeloma. Eur J Dermatol. 2000;10(2):139-42.
Grundmann, J. U., Bonnekoh, B., & Gollnick, H. (2000). Extensive haemorrhagic-bullous skin manifestation of systemic AA-amyloidosis associated with IgGlambda-myeloma. European Journal of Dermatology : EJD, 10(2), 139-42.
Grundmann JU, Bonnekoh B, Gollnick H. Extensive Haemorrhagic-bullous Skin Manifestation of Systemic AA-amyloidosis Associated With IgGlambda-myeloma. Eur J Dermatol. 2000;10(2):139-42. PubMed PMID: 10694315.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Extensive haemorrhagic-bullous skin manifestation of systemic AA-amyloidosis associated with IgGlambda-myeloma. AU - Grundmann,J U, AU - Bonnekoh,B, AU - Gollnick,H, PY - 2000/2/29/pubmed PY - 2000/3/25/medline PY - 2000/2/29/entrez SP - 139 EP - 42 JF - European journal of dermatology : EJD JO - Eur J Dermatol VL - 10 IS - 2 N2 - 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. SN - 1167-1122 UR - https://www.unboundmedicine.com/medline/citation/10694315/Extensive_haemorrhagic_bullous_skin_manifestation_of_systemic_AA_amyloidosis_associated_with_IgGlambda_myeloma_ L2 - http://www.diseaseinfosearch.org/result/381 DB - PRIME DP - Unbound Medicine ER -