Paraneoplastic leukocytoclastic vasculitis mimicking ulcus cruris as rare initial manifestation of smoldering myeloma IgG kappa

Abstract

Leukocytoclastic vasculitis (LCV) has been reported as a rare paraneoplastic phenomenon associated with several hematologic disorders, including indolent lymphomas such as Waldenström macroglobulinemia. However, there are very few cases of LCV in the context of plasma cell disorders. We present the case of a 58-year-old female who developed a rapidly progressive, ulceronecrotic skin lesion on her left lower leg due to leukocytoclastic vasculitis. The lesion was initially suspected to be an ulcerative chronic wound (ulcus cruris) but represented an atypical manifestation of leukocytoclastic vasculitis as primary and only clinical sign of smoldering multiple myeloma IgG kappa. After standard induction chemoimmunotherapy with daratumumab, bortezomib, lenalidomide, and dexamethasone, the patient proceeded to high-dose chemotherapy with melphalan, followed by autologous stem cell transplantation for consolidation. Despite a bacterial skin superinfection, myeloma treatment was successfully completed without any major complications. The skin lesion healed concurrently with the reduction in paraprotein levels, and there was no need for plastic surgical intervention. LCV mimicking ulcus cruris can represent a rare and atypical initial manifestation of plasma cell neoplasia. In this case report, systemic myeloma treatment proved to be effective for inducing complete remission of advanced ulceronecrotic skin damage. This case extends the spectrum of reported monocloncal gammopathies of cutaneous significance.

Keywords: Leukocytoclastic vasculitis; Monoclonal gammopathy of cutaneous significance; Paraneoplastic phenomenon; Skin.

Fig. 1

Macroscopic and microscopic imaging of the skin lesion at initial patient presentation: (a) Clinical examination revealed livid confluent macules (10 × 15 cm) with a 2 × 2 cm ulceronecrotic lesion on the left lower leg. (b, c) Hematoxylin and eosin stain (HE) staining of the skin biopsy showing extravasation of erythrocytes. Blood vessel walls display perivascular infiltration of neutrophils, often with nuclear debris, which is typical for leukocytoclastic vasculitis (b: Magnification: 20x, c: Magnification: 200x). (d) In the chloroacetate staining, the numerous granulocytes appear red (Magnification: 200x)

Fig. 2

Monoclonal gammopathy IgG kappa as underlying condition of leukocytoclastic vasculitis: Protein electrophoreses revealed a significant M-gradient in the γ fraction (peak 23P: 24,1%, 18.2 g/l). Immunofixation confirmed monoclonal gammopathy IgG kappa with light chain (LC) kappa restriction. In line, the serum kappa/lambda (Κ/λ) quotient was increased. Albumin, β2-microglobuline and lactate dehydrogenase (LDH) were normal

Fig. 3

Time course of serum paraprotein levels (a) in relation to skin healing (b) and systemic myeloma treatment (c) After diagnosis of MM-associated leukocytoclastic vasculitis, the patient was treated with six cycles of Dara-V(R)D. After completion of standard induction chemotherapy, apheresis, high-dose chemotherapy with melphalan and consecutive autologous stem cell transplantation was performed. To protect the patient‘s privacy, name and date of birth were blacked out in the pictures. The scale shown indicates 7 cm. Figure 3a was created with GraphPad Prism (V8, GraphPad Software, La Jolla, CA)

Fig. 4

Macroscopic imaging of the skin lesion after completion of systemic myeloma therapy After completion of standard induction chemotherapy followed by consolidating high-dose chemotherapy with consecutive autologous stem cell transplantation, the skin lesion on the left lower leg was nearly completely resolved without the need of any plastic surgery intervention. The pictures were taken 4 months (a) and 5 months (b) after stem cell transplantation. To protect the patient‘s privacy, name and date of birth were blacked out in the pictures