A previously healthy 76-year-old woman was referred to our dermatology clinic for painless intraoral bullae that had appeared over the preceding 3 weeks. The lesions would appear after slight friction (e.g., toothbrushing), with associated bleeding, and heal with no scarring. She also reported 4 months of unintentional weight loss and gradual onset of weakness. On examination, she had intraoral hemorrhagic bullae (Figure 1A) and waxy papules and plaques with central hemorrhagic components at both inguinal folds (Figure 1B). No other mucocutaneous involvement was present. Laboratory investigations showed a normal platelet count with no alteration of prothrombin time or activated partial thromboplastin time. Histopathology of intraoral lesions revealed hematic extravasation and λ light chain deposition on the capillary endothelium. A hematologist performed a bone marrow biopsy, which was diagnostic for immunoglobulin light chain amyloidosis. No alterations were found on electrocardiography or echocardiography, and renal and hepatic laboratory tests were normal. Hematologists started therapy with daratumumab along with cyclophosphamide, bortezomib, and dexamethasone, and she will be followed-up in our dermatology clinic and the hematology clinic every 3 months.
Immunoglobulin light chain amyloidosis is a rare condition, with an incidence of nearly 10 per million person-years.1 It is characterized by deposition of misfolded monoclonal immunoglobulin light chains, which leads to organ dysfunction.
Mucocutaneous manifestations occur in one-third of cases, frequently constituting the first sign of disease. The most common findings are purpuric lesions on waxy plaques located on the periorbital region (raccoon eyes) and the axillae, and axillae inguinal folds, but can also appear on oral or genital mucosae, where hemorrhagic bullae may also develop. Differential diagnosis includes sclerodema, myxedema, immune thrombocytopenia, traumatic purpura, scurvy, autoimmune bullous diseases, and vasculitis.2 Treatment depends on whether a patient is eligible to receive autologous stem cell transplantation based on demographic, clinical, and laboratory criteria, including age younger than 70 years.1 If a person is ineligible, bortezomib-based regimens are considered.1 When diagnosed at an advanced stage, median survival can be as short as 5 months. The most frequent causes of death are infection and cardiac or hepatic failure.3
Footnotes
Competing interests: None declared.
This article has been peer reviewed.
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