Case of multiple myeloma associated with extramedullary cutaneous plasmacytoma and pyoderma gangrenosum

Abstract

A variety of cutaneous manifestations has been described in multiple myeloma including extramedullary cutaneous plasmacytomas, cutaneous amyloidosis, pyoderma gangrenosum, leukocytoclastic vasculitis, necrobiotic xanthogranuloma, scleromyxedema, Sweet's syndrome, subcorneal pustular dermatosis, scleredema, and plane xanthomas etc. An 89-year-old Korean man, who had been suffering from multiple myeloma 1 year previous, presented for evaluation of two nodules on the right side of the forehead, left side of the chest (7th rib area), and multiple ulcers with papulopustules on both the thigh and the left side of the chest (2nd rib area) during 15 days, which developed at the same time. A biopsy of a lesion which manifested as a cutaneous nodule on the right side of the forehead revealed dermal infiltration by well-differentiated plasma cells, similar to those found on a bone marrow biopsy, and a biopsy of the lesion manifested as a painful ulceration on the right thigh area showing dermal neutrophilic infiltration. Histologic findings were consistent with plasmacytoma and pyoderma gangrenosum, respectively. We present a case of multiple myeloma which developed extramedullary cutaneous plasmacytoma and pyoderma gangrenosum simultaneously, which is very rare. The patient was treated with a systemic steroid and conservative therapy.

The histological and pathogenetic spectrum of cutaneous disease in monoclonal gammopathies

“…Pyoderma gangrenosum is associated with systemic diseases including inflammatory bowel disease, rheumatoid arthritis, seronegative polyarthritis, and monoclonal gammopathies. The latter, usually IgA, occurs in about 12% of cases 69–71 . The histopathologic findings are not specific or variable depending on the evolution of the lesion.…”

An Aggressive Extramedullary Cutaneous Plasmacytoma Associated with Extreme Alterations in the Innate Immune System

“…Theappearanceofimmatureplasmacellsinourpatient's bone marrow samples coincided with disease progression, a disturbanceinourpatient'simmunity,andclinicaldeterioration.WefoundverylownumbersofNKcellswithoutdisturbanceintheCD4/CD8ratioandextremelylowNKcellactiv-itylevels;invitrolymphocytecultureshadundetectablelevels ofIL-2.ThesefindingsmayreflectaninteractionbetweenNK cellsandplasmacells,andmayrelatetoimmunosuppressive cytokinesandotherinhibitoryfactorsproducedbythetumor cells [10].NKcellactivityhascertainlybeenseentobesimilarly suppressed in the advanced stages of different types of hematologicalmalignancyandmultiplemyeloma [7,8].Disturbances in the humoral and cellular systems have been showntocorrelatewiththeappearanceofopportunisticinfections in other plasma cell malignancies [5]. Our patient, as with the majority of cases of cutaneous plasma cell disease, diedshortlyafterdiagnosisdespitechemotherapyandradiotherapy [7][8][9]reflectingthedevastatingeffectsofthisdisease ontheinnateimmunesystem.…”

“…These conditions can be subdividedintothosetumorsoriginatingwithintheskeletalsystem and extramedullary plasmacytomas. Immunohistochemical analysesareinvaluablewhenestablishingaprecisediagnosis asthetumorsubtypeshavedifferingratesofprogressionand overallsurvival [4,5].Eachofthesetumorscanhavecutaneous involvement, though this occurs rarely and may reflect more aggressive disease [2,6]. Alterations in natural killer (NK)cellactivityhavebeenimplicatedasafactorinthepropensityforthesemalignantplasmacellstospreadtotheskin and other organs [7][8][9][10], though this role is not fully understood.…”

Cutaneous Plasmacytoma: Metastasis of Multiple Myeloma at the Fracture Site

“…MM occurring in tissues other than bone and bone marrow are called extramedullary plasmacytoma78910. It can also be defined as aggregates of plasma cells showing monoclonality occurring within soft tissue.…”