{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,13]],"date-time":"2026-08-13T19:41:55Z","timestamp":1786650115791,"version":"build-2736575974"},"reference-count":10,"publisher":"Ovid Technologies (Wolters Kluwer Health)","issue":"3","license":[{"start":{"date-parts":[[2026,4,28]],"date-time":"2026-04-28T00:00:00Z","timestamp":1777334400000},"content-version":"unspecified","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc-nd\/4.0"}],"content-domain":{"domain":["lww.com","ovid.com"],"crossmark-restriction":true},"short-container-title":[],"published-print":{"date-parts":[[2026,5]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:sec>\n                    <jats:title>Introduction:<\/jats:title>\n                    <jats:p>Primary systemic amyloidosis (PSA), also referred to as amyloid light-chain (AL) amyloidosis, is characterized by the deposition of insoluble monoclonal immunoglobulin light chains or L-chain fragments in a variety of tissues and organs of the body. There is a dearth of literature regarding the mucocutaneous manifestations of PSA in the Indian population, which prompted us to undertake the present study.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Aim and Objective:<\/jats:title>\n                    <jats:p>We aimed to study the clinical characteristics of mucocutaneous manifestations in patients with PSA at a tertiary care center in Kolkata.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Patients and Methods:<\/jats:title>\n                    <jats:p>Consecutive patients with PSA, who presented to our facility with cutaneous features were included in this cross-sectional observational study done over 4 years.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results:<\/jats:title>\n                    <jats:p>The study involved 14 patients diagnosed with systemic amyloidosis (PSA) presenting with mucocutaneous symptoms. The mean age at diagnosis was 62.8 years, with a female-to-male ratio of 2:5. Myeloma-associated primary amyloidosis was identified in 35.7%. All patients showed pinch purpura, macroglossia, and tongue ridging. Periorbital purpura was seen in 92.8%, facial purpura in 85.7%, waxy nodules in 78.5%, xerosis in 57%, and diffuse alopecia in 35.7%. Relatively rare features included thickened skin, nail dystrophy, bullous lesions, lingual and subcutaneous nodules, atrophie blanche, and cutis laxa. Macular\/lichen amyloidosis was also observed in one patient each. The head and neck were universally involved (100%). Eight patients had systemic features.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Limitations:<\/jats:title>\n                    <jats:p>The study was limited by its hospital-based design, small sample size, and lack of lesional biopsies.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusions:<\/jats:title>\n                    <jats:p>PSA has a wide gamut of cutaneous features, and recognizing these skin lesions in the setting of PSA may be a significant clinical indicator of the underlying systemic illness.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.4103\/idoj.idoj_704_25","type":"journal-article","created":{"date-parts":[[2026,4,29]],"date-time":"2026-04-29T12:06:43Z","timestamp":1777464403000},"page":"386-391","update-policy":"https:\/\/doi.org\/10.1097\/lww.0000000000001000","source":"Crossref","is-referenced-by-count":0,"title":["Mucocutaneous Manifestations of Primary Systemic Amyloidosis: A Descriptive Study from Eastern India"],"prefix":"10.4103","volume":"17","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-3977-0462","authenticated-orcid":false,"given":"Sudip K.","family":"Ghosh","sequence":"first","affiliation":[{"name":"Department of Dermatology, Venereology, and Leprosy, R. G. Kar Medical College, Kolkata, West Bengal, India"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sharmistha","family":"Panja","sequence":"additional","affiliation":[{"name":"Department of Dermatology, Venereology, and Leprosy, R. G. Kar Medical College, Kolkata, West Bengal, India"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Biswajit","family":"Majumder","sequence":"additional","affiliation":[{"name":"Department of Cardiology, R. G. Kar Medical College, Kolkata, West Bengal, India"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Maloy","family":"Pramanik","sequence":"additional","affiliation":[{"name":"Department of Dermatology, Venereology, and Leprosy, R. G. Kar Medical College, Kolkata, West Bengal, India"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"276","published-online":{"date-parts":[[2026,4,28]]},"reference":[{"key":"R1-20260430","doi-asserted-by":"crossref","first-page":"27","DOI":"10.14797\/mdcvj.1070","article-title":"Light chain amyloidosis:Epidemiology, staging, and prognostication","volume":"18","author":"Baker","year":"2022","journal-title":"Methodist Debakey Cardiovasc J"},{"key":"R4-20260430","doi-asserted-by":"crossref","first-page":"7","DOI":"10.1016\/0738-081X(90)90081-B","article-title":"Mucocutaneous manifestations in systemic amyloidosis","volume":"8","author":"Wong","year":"1990","journal-title":"Clin Dermatol"},{"key":"R5-20260430","first-page":"47","article-title":"Systemic amyloidosis. A report of 19 cases","volume":"189","author":"Wong","year":"1994","journal-title":"Dermatology"},{"key":"R6-20260430","doi-asserted-by":"crossref","first-page":"89","DOI":"10.11604\/pamj.2024.49.89.20032","article-title":"Oral manifestations of undiscovered systemic amyloidosis:A series of seven cases","volume":"49","author":"Chebil","year":"2024","journal-title":"Pan Afr Med J"},{"key":"R7-20260430","doi-asserted-by":"crossref","first-page":"1062","DOI":"10.1111\/ijd.14443","article-title":"Mucocutaneous manifestations in systemic amyloidosis A retrospective analytical study in a tertiary care center","volume":"58","author":"Flores-Bozo","year":"2019","journal-title":"Int J Dermatol"},{"issue":"(5, Suppl 1)","key":"R8-20260430","first-page":"AB49","article-title":"Mucocutaneous manifestations of AL type systemic amyloidosis","volume":"70","author":"Lera","year":"2014","journal-title":"J Am Acad Dermatol"},{"key":"R9-20260430","doi-asserted-by":"crossref","first-page":"AB172","DOI":"10.1016\/j.jaad.2016.02.675","article-title":"Mucocutaneous manifestation in systemic amyloidosis at national institute of health sciences and nutrition, Mexico City. Experience in 13 years","volume":"74","author":"Flores-Bozo","year":"2016","journal-title":"J Am Acad Dermatol"},{"key":"R10-20260430","doi-asserted-by":"crossref","first-page":"394","DOI":"10.4103\/0378-6323.53138","article-title":"Primary systemic amyloidosis:Three different presentations","volume":"75","author":"Saoji","year":"2009","journal-title":"Indian J Dermatol Venereol Leprol"},{"key":"R11-20260430","first-page":"1133","article-title":"Characteristics of orofacial amyloidosis:A case series","volume":"1","author":"Liu","year":"2016","journal-title":"Ann Clin Case Rep"},{"key":"R12-20260430","doi-asserted-by":"crossref","first-page":"356","DOI":"10.1016\/j.revmed.2014.02.002","article-title":"Anomalies ungu\u00e9ales r\u00e9v\u00e9lant une amylose syst\u00e9mique AL [Nail abnormalities revealing AL systemic amyloidosis]","volume":"36","author":"\u00c9tienne","year":"2015","journal-title":"Rev Med Interne"}],"container-title":["Indian Dermatology Online Journal"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/www.ovid.com\/10.4103\/idoj.idoj_704_25","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2026,8,13]],"date-time":"2026-08-13T19:03:11Z","timestamp":1786647791000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.ovid.com\/10.4103\/idoj.idoj_704_25"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2026,4,28]]},"references-count":10,"journal-issue":{"issue":"3","published-print":{"date-parts":[[2026]]}},"URL":"https:\/\/doi.org\/10.4103\/idoj.idoj_704_25","relation":{},"ISSN":["2229-5178","2249-5673"],"issn-type":[{"value":"2229-5178","type":"print"},{"value":"2249-5673","type":"electronic"}],"subject":[],"published":{"date-parts":[[2026,4,28]]},"assertion":[{"value":"2025-06-20","name":"received","label":"Received","group":{"name":"publication_history","label":"Publication History"}},{"value":"2025-11-21","name":"accepted","label":"Accepted","group":{"name":"publication_history","label":"Publication History"}}]}}