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Mucocutaneous manifestations in systemic amyloidosis A retrospective analytical study in a tertiary care center
Author(s) -
FloresBozo LuisRodrigo,
EchevarríaKeel Josune,
DomínguezCherit Judith,
EsquivelPedraza Lilly,
MéndezFlores Silvia
Publication year - 2019
Publication title -
international journal of dermatology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.677
H-Index - 93
eISSN - 1365-4632
pISSN - 0011-9059
DOI - 10.1111/ijd.14443
Subject(s) - mucocutaneous zone , medicine , amyloidosis , al amyloidosis , dermatology , single center , transthyretin , aa amyloidosis , pathology , gastroenterology , immunology , disease , immunoglobulin light chain , antibody , familial mediterranean fever
Background The mucocutaneous clinical profile of patients with amyloidosis in Latin America has been scarcely reported. Objective To describe clinical characteristics of skin and mucosal manifestations in systemic amyloidosis in a tertiary care center in Mexico City. Methods A cross sectional, retrospective analytical study was performed in patients with systemic amyloidosis over a 15‐year period. Statistical analysis was done. Results A total of 98 patients were included (53 [54%] men; overall median age = 49 years old). Acquired systemic immunoglobulin light chain amyloidosis ( AL ) was the most common (49%) type, followed by (24.5%) wild‐type transthyretin amyloidosis (H‐ TTR ) (24.5%), undetermined cases (21%), and reactive systemic amyloidosis (AA) (6.1%). There were mucocutaneous manifestations in 34.7% of cases, mostly multiple myeloma‐related AL ( ALMM ). Head and neck was the most often affected site (38.2%), and purpuric macules were the most common morphology (44.1%). Mucocutaneous affectation was predominantly observed in AL (50.0%) compared to other types (20.0%) of amyloidosis ( P = 0.01). Likewise, involvement of organs was also significantly different among the diverse amyloidosis types ( P < 0.05). The most frequent comorbidities were hypertension (18.3%) and hypothyroidism (18.3%). Conclusion The clinical spectrum of manifestations in amyloidosis is wide. Involvement of skin and mucosa in amyloidosis is common; significant differences were observed concerning distribution of mucocutaneous amyloid manifestations among the different types of amyloidosis.