Mucocutaneous Manifestations of Primary Systemic Amyloidosis: A Descriptive Study from Eastern India

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INTRODUCTION: 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. AIM AND OBJECTIVE: We aimed to study the clinical characteristics of mucocutaneous manifestations in patients with PSA at a tertiary care center in Kolkata. PATIENTS AND METHODS: Consecutive patients with PSA, who presented to our facility with cutaneous features were included in this cross-sectional observational study done over 4 years. RESULTS: 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. LIMITATIONS: The study was limited by its hospital-based design, small sample size, and lack of lesional biopsies. CONCLUSIONS: 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.

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