Vulvar amyloidosis mimicking giant condylomata acuminata in a patient with multiple myeloma

ARTICLE

The cutaneous amyloidoses can be classified into a localized form (i.e., the amyloid deposits are restricted to the skin) and a systemic type. The systemic amyloidoses are subdivided into a "primary" type, a myeloma-associated type and a type secondary to chronic diseases [1]. Patients with multiple myeloma are prone to develop systemic amyloidosis involving the skin due to deposition of immunoglobulin light chain material (protein amyloid L, AL). It has been estimated that 15% of patients suffering from multiple myeloma might demonstrate amyloidosis [2].

Cutaneous amyloidosis shows a large variety of clinical features. We report an unusual case of condyloma-like lesions as a manifestation of myeloma-associated amyloidosis. This type of papillomatous cutaneous amyloidosis has only exceptionally been reported in patients with multiple myeloma [3-5], and the degree of severity found in the present case appears to be unique. In patients with multiple myeloma, we consider it mandatory to differentiate condyloma-like lesions from eruptions induced by human papilloma virus before initiation of systemic chemotherapy.

Case report

A 40-year-old woman was referred to our outpatient clinic with a diagnosis of "genital condylomata acuminata". Four months earlier she was diagnosed as having multiple myeloma grade IIIa according to Durie and Salmon [6]. One month before the diagnosis of plasmocytoma was established she first noticed skin changes in the vulvar area. At approximately the same time she had undergone surgery for carpal tunnel syndrome. Quite recent to her presentation in our department, multiple osteolytic lesions of the skull, humerus and femur had been found on X-ray examination. Anemia and Bence-Jones proteinuria due to monoclonal lambda light chains were present.

Genital examination revealed a dense, widespread dissemination of rubbery, waxy nodules covering the vulva. In the peripheral sites cobblestone-like verrucous papules were seen, whereas pedunculated, condyloma-like tumors were centrally present. The lesions were slightly pigmented (Fig. 1). No other skin abnormality was detected.

Histopathological examination of an excisional biopsy (Fig. 2) revealed papillomatous structures covered by a relatively thin epidermal layer with elongated rete ridges. An amorphous eosinophilic material replaced almost the entire papillary dermis. No inflammatory infiltrate and no plasma cells could be detected. There were no histopathological features typical of condylomata acuminata. The dermal deposits showed a positive reaction to Congo red stain (Fig. 3). Immunohistochemical studies were performed on formalin-fixed paraffin-embedded biopsy material using mouse antisera to human kappa and lambda light chains (antibodies purchased from DAKO, Glostrup, Denmark, clones N10/2 and A8B5). An intense reaction of the dermal deposits was noted for lambda light chains (Fig. 4), whereas no staining was seen for kappa light chains (not shown). Immunohistochemical examination for human papilloma virus (HPV) was performed by the Department of Pathology, Philipp University, Marburg. There was no reaction to HPV screening markers, nor to the high-risk markers for HPV type 16 and type 33. A diagnosis of cutaneous myeloma-associated amyloidosis was established.

Four months later the patient developed renal failure and died within a few days.

Discussion

Condyloma-like lesions as a consequence of amyloidosis have been described in the perianal region [3], the sacral area and the gluteal fold [5] and the vulva [4, 7, 8]. These regions are likewise areas of predilection for HPV-induced lesions. Taylor et al. report a rare case of waxy, rubbery, papulonodular vulvar lesions due to systemic amyloidosis in a patient suffering from multiple myeloma [4]. The present case is similar but shows an even more pronounced involvement.

In cases of systemic amyloidosis the patients may also exhibit symptoms of carpal tunnel syndrome, macroglossia, mucocutaneous lesions and hepatomegaly. Cutaneous manifestations of multiple myeloma can be present in the form of extramedullary cutaneous plasmocytomas, leukocytoclastic vasculitis, pyoderma gangrenosum, and, as observed in the present case, amyloidosis [9]. The broad spectrum of skin manifestations due to myeloma-associated amyloidosis may vary from common eruptions such as purpuric lesions and waxy, translucent papules, nodules and plaques to less common clinical features such as bullous lesions, pigmentary changes and sclerodermal changes [1]. The diagnosis of amyloidosis in patients with multiple myeloma is associated with a poor prognosis. In 1983 Kyle et al. estimated an average survival rate in such patients of not more than 5 months [10].

In conclusion, vulvar amyloidosis should be added to the list of myeloma-induced skin eruptions. In the present case, the extensive dense agglomeration of vulvar condyloma-like lesions had been mistaken at a first examination as condylomata acuminata. The correct diagnosis was established only by histopathological and immunohistochemical examination.

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