First Case of Nodular Localized Primary Cutaneous Amyloidosis Treated With Bortezomib and Dexamethasone

Abstract

Nodular localized cutaneous amyloidosis is a rare form of cutaneous amyloidosis and is characterized by an extracellular deposition of insoluble amyloid fibrils which are either primarily cutaneous or a manifestation of an underlying systemic amyloidosis. Biopsy of the lesion is mandatory for the diagnosis, and histopathology shows diffuse amyloid deposits with plasmacytic infiltration. Apple-green birefringence characteristic of amyloidosis is observed when stained with Congo red and viewed under polarized light. Amyloid subtyping is done with laser microdissection followed by mass spectrometry. Majority of these lesions do not require any treatment but surgical excision, shave excision, laser therapy, and radiotherapy can be considered for symptomatic nodular localized primary cutaneous amyloidosis (NLPCA). We present a case of recurrent NLPCA in a 64-year-old woman who was treated with bortezomib and dexamethasone after failing several local therapies with excellent response.

Localized Cutaneous Nodular Amyloidosis: A Specific Cutaneous Manifestation of Sjögren’s Syndrome

“…The subtype of amyloid deposits must then be determined, which reveals the κ and/or λ light chain [ 20 ]. For diagnosis, in addition to biopsies, soft-tissue imaging, preferably magnetic resonance imaging (MRI), should be requested in cases of extensive or deep involvement [ 21 ].…”

“…Plaques and nodules with a waxy appearance and a yellowish-orange color similar to those described in PLCNA are frequent [ 22 ]. In contrast to PLCNA, lesions are more common in mucocutaneous areas, such as the orbits and nostrils [ 21 ]. In primary systemic amyloidosis, it is frequent to find petechial, hemorrhagic, and purpuric lesions due to dermal involvement.…”

“…Some authors also suggest performing initial imaging studies, such as positron emission tomography (PET) scanning, to evaluate systemic involvement. In order to rule out cardiac involvement, an electrocardiogram may be ordered [ 21 ].…”

“…Systemic treatment may be considered in extensive cases or when local modalities fail to control the disease [ 21 ]. As discussed, PLCNA often does not respond well to immunosuppressive therapies, although intra-lesional methotrexate may be an option for patients who have localized lesions and are poor candidates for surgery or invasive procedures [ 56 ].…”

“…The patient had recurrent PLCNA and had previously undergone several local therapies with no response. The validity of this therapy was based on the good results obtained with the combination of proteasome inhibitors and corticosteroids in the treatment of systemic immunoglobulin light chain amyloidosis (AL) [ 21 ]. When taking into account the associated accompanying symptoms, interleukin-31 antibodies, as a novel antipruritic medication, may offer a new approach to the future treatment of this condition [ 50 ].…”