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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