Monoclonal Gammopathy of Undetermined Significance and Neutrophilic Dermatosis

A 62-year-old female presented with a three-day history of painful purple-red nodules on her face and extremities [Figure 1] associated with generalized malaise and arthralgia. The patient had a significant medical history of monoclonal gammopathy of undetermined significance diagnosed in 2008, ovarian cancer, which was treated with surgery and chemotherapy in 2010, and thyroid cancer for which she underwent a thyroidectomy in 2011.

Laboratory investigations showed normal complete blood counts, high C-reactive protein levels (27.4 mg/L; normal range 0–5 mg/L), normal renal and liver function, normal CA 125 level, and normal bone marrow biopsy. Serum protein electrophoresis showed monoclonal immunoglobulin (Ig)-A with lambda peak. A skin biopsy revealed diffuse neutrophilic dermatitis with no evidence of vasculitis or stainable microorganisms.

Figure 1.

Erythematous, tender nodules, and papules, some with central crusting distributed asymmetrically over the face, dorsum hands, arms, and thighs.

Questions

What is the diagnosis?

1. Sweet’s syndrome

2. Leukaemia cutis

3. Neutrophilic eccrine hidradenitis

Is there any association between monoclonal gammopathy of undetermined significance and this condition?

1. Yes

2. No

What is the first-line treatment?

1. Systemic corticosteroid treatment

   1. Ciclosporin

      1. Thalidomide