(PA-351) Cutaneous Manifestation of Monoclonal Gammopathy : Clinical Challenges and Management

A 59-year-old woman with a 3-year history of urticaria accompanied by fever, leading to a diagnosis of Schnitzler syndrome, associated with an IgM kappa peak. As anakinra was unavailable, she received two courses of rituximab, in addition to steroids and azathioprine, with success.

A 69-year-old man with indolent myeloma (SMM) with groin necrosis related to pyoderma gangrenosum. Despite treatment with steroids, the disease relapsed several times even after dapsone and MMF were administered. Ultimately, the disease never relapsed after starting anti-MM therapy.

A 57-year-old woman with scleroderma on her head and neck, leading to a diagnosis of Buschke scleroderma associated with an IgG kappa peak. Treatment with Vd resulted in the disappearance of the skin lesions and the monoclonal peak. She relapsed 10 years later and was successfully treated with Rd.

A 70-year-old woman with fever, necrotic purpura, arthralgia and acute renal failure, that led to a diagnosis of cryocrystalglobulinemia associated with SMM. Initial therapy consisted of plasma exchanges combined with VCD, followed by lenalidomide maintenance. The disease did not recur after plasma exchanges ceased, but renal function did not recover.

A 69-year-old man with a seronegative rheumatoid polyarthritis, with febrile urticaria related to Sweet syndrome. Further testing revealed an IgG lambda SMM. Although symptoms improved with infliximab, they did not resolve, raising the question of whether to start clone-directed therapy.

A 70-year-old woman with declive purpura and acute renal failure, leading to a diagnosis of rheumatoid purpura. Further testing revealed an IgA lambda SMM. Although symptoms improved with steroids, relapse occurred upon reducing the dosage. There was no recurrence of skin or renal problems under MM-directed therapy with DRd.

A 38-year-old woman with kidney failure, heavy proteinuria and microscopic hematuria, and an IgG kappa peak. She appeared much older than her age, with sagging skin on her face, neck, armpits, and groin. A skin and kidney biopsy revealed the same deposits of gamma heavy chains and C3, leading to a diagnosis of acquired cutis laxa due to heavy chain deposition disease. This was treated with VCD.

Conclusions:   This case series illustrates the range of cutaneous manifestations of MGCS. An accurate diagnosis is essential for effective management. It is also essential to raise awareness among clinicians to ensure timely diagnosis and effective treatment.