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


Considering the results of routine microscopic sections, immunohistopathological examinations, and immunoserological analysis, our case definitely has PV but not PNP which is known to be associated with multiple myeloma. To the best of our knowledge, there have been no reported cases of multiple myeloma revealed by PV in Madagascar.

Skin lesions are frequently encountered in clinical practice; they can be a presentation of systemic diseases not excluding an occult malignancy. The association of pemphigus with malignancy has been well documented.[2,3] PNP, a new disease entity, was first described by Anhalt et al. in 1990;[4] PNP is associated primarily with malignant and benign tumors of hematological tissue origin. However, PV associated with multiple myeloma is rare. Kridin et al. reported 16 cases of multiple myeloma in 1985 patients with pemphigus.[5] Absence of mucosal involvement, lack of vacuolar degeneration at the interface, and absence of apoptotic, dyskeratotic keratinocytes ruled out PNP in our case. Furthermore, our patient's skin lesion was not as severe or as polymorphous as those in PNP. PV and PNP have similar clinical presentations but have different pathophysiologies in that PV shows a more favorable prognosis than PNP, which often leads to pulmonary involvement. PNP is known to associate with diverse conditions such as non-Hodgkin's lymphoma, chronic lymphocytic leukemia, Castleman's disease, thymoma, and Waldenstrom's macroglobulinemia; however, PV should be considered even if the possible underlying diseases for which PNP is recognized are present. Table 1 shows salient features to differentiate between PV and PNP.

Scleromyxedema, lichen myxedematosus, and papular mucinosis are the skin disorders most associated with monoclonal gammopathy.[6,7] PV concomitant with multiple myeloma was first reported by Motoki Kurokawa et al. in 2005.[8] Furthermore, there are several reports of cases of myeloma associated with autoimmune diseases such as autoimmune hemolytic anemia,[9] acquired von Willebrand disease,[10] Sjögren's syndrome,[11] and Hashimoto's thyroiditis.[12] Although multiple myeloma is basically a B cell malignancy, the mechanism of autoantibody production in myeloma is still unclear. Fas mutations have been found in multiple myeloma, and there is a high incidence of autoreactive phenomena in patients with Fas mutations, such as systemic lupus erythematosus, Sjögren's syndrome, and Hashimoto's thyroiditis.