Primary systemic amyloidosis, acquired cutis laxa and cutaneous mucinosis in a patient with multiple myeloma

Abstract

A 57-year-old woman presented with periorbital ecchymoses, laxity in skin folds, polyneuropathy and bilateral carpal tunnel syndrome. A skin biopsy of the axillary lesion demonstrated fragmentation of elastic fibers, but with a negative von Kossa stain, consistent with cutis laxa. The diagnosis of primary systemic amyloidosis was made by the presence of amyloid material in the eyelid using histopathological techniques, besides this, the patient was also diagnosed with purpura, polyneuropathy, bilateral carpal tunnel syndrome and monoclonal gammopathy. She was diagnosed as suffering from multiple myeloma based on the finding of 40% plasma cells in the bone marrow, component M in the urine and anemia. The patient developed blisters with a clear content, confirmed as mucinosis by the histopathological exam. The final diagnoses were: primary systemic amyloidosis, acquired cutis laxa and mucinosis, all related to multiple myeloma.

Acral localized acquired cutis laxa as presenting sign of underlying systemic amyloidosis

“…Interestingly, there are reports of non-acral and generalized cutis laxa arising in the setting of plasma cell dyscrasias with and without evidence of amyloid deposition in affected skin. 13,14 Observation of IgG bound to dermal elastic fibers was reported by Cho et al, 15 In summary, we report a case of ALACL which was the presenting sign of systemic primary amyloidosis in an otherwise asymptomatic patient. Previously reported cases are exceedingly rare, and only one prior report described ALACL as the sole clinical finding leading to a diagnosis of plasma cell dyscrasia, 9 as in our case.…”

Frequency of cutis laxa‐like clinical features and elastolysis in scleromyxedema: a retrospective clinicopathologic study of 19 patients with scleromyxedema

“…In these patients, clinical findings are preceded by generalized urticaria or by annular erythematous–papular lesions. This phenomenon has also been described as resulting from a prior inflammatory insult, such as in patients with mid‐dermal elastolysis, multiple myeloma or primary systemic amyloidosis . The mechanism for the decreased density and fragmentation of elastic fibers in these syndromes is yet to be elucidated.…”

Relapsing bullous amyloidosis of the oral mucosa and acquired cutis laxa in a patient with multiple myeloma: a rare triple association

“…In our case, multiple haemorrhagic oral bullae were the first sign of an undiagnosed PSA related to MM and previously diagnosed CLA. There is only one report in the literature with this rare triple association, but in that case the patient did not have oral mucosa involvement or BA . Early diagnosis of PSA is essential to curb progression and increase survival rates for these patients.…”

“…The mechanism is believed to be immune‐mediated. There is no synchronism between the courses of MM and CLA …”

“…It occurs in 13–26% of patients with MM . Mucocutaneous lesions are found in 20–40% of the cases, often as the first sign . Bullous amyloidosis (BA) of the oral cavity has rarely been reported in the literature …”