Primary systemic amyloidosis, acquired cutis laxa and cutaneous mucinosis\nin a patient with multiple myeloma
A fifty-seven year old, female informed the presence of bilateral eyelid hyperchromia\nfor the last fifteen years, followed by an increase in palpebral volume that started\nthree years after the first symptoms. Six years ago, she observed cutaneous laxity in\nskin-fold areas and bilateral palpebral ptosis. In the last four years, the patient\nreports pain and paresthesia in a stocking-like pattern affecting the lower limbs. The\npatient denied any familial history of cutaneous lesions.\nShe had a previous history of bilateral carpal tunnel syndrome and blepharoplasties\nperformed in two different occasions, 2008 and 2009.\nDermatologic examination of eyelids showed hyperchromia, increase of local volume,\ncutaneous laxity and bilateral ptosis with periorbital ecchymoses (). We observed cutaneous laxity, hyper-chromic papules and\ncomedones over the skinfolds, such as the armpits, cervical and inframammary regions\n(). Biopsies of both axillary and\npalpebral lesions were performed.\nHistopathological examination of the axillary lesion did not show any significant\nalteration with hematoxiline-eosine stain, detecting only one epidermic cyst,\ncorresponding to the observed papule. Orcein staining showed the fragmentation of\nelastic fibers and von Kossa staining was negative, i.e., without evidence of calcium,\nthus establishing the diagnosis of acquired cutis laxa ().\nHistological exam of the palpebral material showed an amorphous eosinophilic substance,\nlocated on the superior and vascular dermises, positive for red Congo staining and with\ngreen birefringence to polarized light, which confirmed the diagnosis of amyloidosis\n().\nLaboratory exams demonstrated normocytic and normochromic anemia, an inversion of the\nalbumin-globulin ratio, IgG-Kappa monoclonal gammopathy, Bence-Jones proteinuria and 40%\nof plasma cells in the bone marrow specimen, prompting a diagnosis of multiple myeloma.\nThe patient evolved with citrine-content blisters on the inframammary region. A plaque,\nwith severe edema, located on the left arm was biopsied, showing dermic edema with\nmoderate fibroblast proliferation, some of which were stellate ( and ). Colloidal\niron staining determined the presence of mucin, and the diagnosis of papular mucinosis,\nwhich was not in agreement with the clinical presentation, thus making it difficult to\nclassify the disease in this case ( and\n). A negative result for red Congo stain\ndiscarded bullous amyloidosis.\nThe final diagnoses were, accordingly, primary systemic amyloidosis, acquired cutis laxa\nand cutaneous mucinosis, all dermatologic manifestations associated to MM.