Pemphigus Vulgaris as the First Manifestation of Multiple Myeloma

A Case Report

Fandresena Arilala Sendrasoa; Irina Mamisoa Ranaivo; Mendrika Fifaliana Rakotoarisaona; Onivola Raharolahy; Naina Harinjara Razanakoto; Malalaniaina Andrianarison; Lala Soavina Ramarozatovo; Fahafahantsoa Rapelanoro Rabenja


J Med Case Reports. 2018;12(255) 

In This Article

Case Presentation

A 55-year-old Malagasy man, a doctor, a non-smoker of tobacco, with no significant past medical history, presented with bullous and erosive skin lesions involving his trunk and scalp for the past 2 months. No toxic exposure was noted. He had a family history of cancer; his mother and sister presented breast cancer and multiple myeloma, respectively, the diagnosis of which were delayed. He had no personal or family history of any autoimmune disease. No medication was prescribed prior to diagnosis. A physical examination revealed multiple crusted erosions intermixed with erythematosus patches over his scalp (Figure 1), trunk (Figure 2a), and his back (Figure 2b). He had no mucous membrane involvement. General physical and systemic examinations were normal.

Figure 1.

Multiple crusted erosions intermixed with erythematosus patches over scalp

Figure 2.

a Multiple crusted erosions intermixed with erythematosus patches over trunk. b Multiple crusted erosions intermixed with erythematosus patches over the back

A complete blood count revealed microcytosis without anemia with mean corpuscular volume (MCV) of 76 fl and hemoglobin of 15.7 g/dL; his white cell count and platelet count were normal. Alanine and aspartate aminotransferase were normal (28 U/L and 25 U/L, respectively) but serum creatinine was high (121 umol/l; normal range: 53–115 umol/L). Other laboratory tests including corrected calcium level, phosphoremia, lactate dehydrogenase, and urine analysis were normal. His HIV status was negative.

A skin biopsy showed suprabasal blisters containing eosinophils and acantholytic keratinocytes. Direct immunofluorescence of perilesional skin revealed immunoglobulin G (IgG) deposition in the intercellular spaces in the epidermis. In an enzyme-linked immunosorbent assay (ELISA), his serum autoantibody index against desmoglein-1 and 3 was found to be 112 RU/mL and 34 RU/mL (normal range, < 20 RU/mL), respectively. Serum immunoelectrophoresis showed a monoclonal gammopathy with a markedly elevated IgG level (2880 mg/dL) in association with a lambda free light chain. Urine analysis was negative for Bence-Jones protein and beta2-microglobulin was 2.4 mg/L. Bone marrow aspirate showed 6% plasma cell infiltration. Further investigations, including creatinine blood test and whole body radiographic examinations, showed that he had clinical stage I multiple myeloma of the IgG-ƛ type.

At first, the skin lesions regressed significantly after topical applications of corticosteroid ointment and no specific therapy for myeloma was conducted. Three months later, the dermal affliction occurred again so systemic administration of prednisolone (1 mg/kg per day) was started. Six months later, bone tomography revealed vertebral compression fractures of the thoracic and lumbar spine that correlated with our patient's back pain topographically. However, his IgG level decreased (1080 mg/dL). Anti-myeloma treatment including melphalan and prednisone was started, which resulted in a rapid decline of monoclonal IgG concentration immediately. Skin and hematologic remission were maintained for 12 months.