Perianal condyloma‐like lesions in multiple myeloma associated amyloidosis

ARTICLE

Auteur(s) : Alberto SCHIERA^a, Marco PINI^a, Pietro PIOLTELLI^b, Eugenio ROSSI^a, Maria Gabriella VALENTE^c, Dario CRIPPA^a

^a Department of Dermatology, San Gerardo Hospital, Via Donizetti 106, 20052 Monza, Italy 
^b Department of Medicine, Division of Haematology, San Gerardo Hospital, Via Donizetti 106, 20052 Monza, Italy 
^c Department of Pathology, San Gerardo Hospital, Via Donizetti 106, 20052 Monza, Italy

Article accepted on 14/1/2004

Amyloid deposition may occur throughout many organs of the body (systemic amyloidosis) or be restricted to a single tissue site (localized amyloidosis). Systemic types of amyloidosis include those associated with plasma cell dyscrasia, either overt as in multiple myeloma or occult as in “primary“ systemic amyloidosis, and amyloidosis secondary to a variety of chronic diseases [1].
We report a case of myeloma-associated systemic amyloidosis in a woman who presented, in addition to common skin signs of amyloidosis, perianal and vulvar pedunculated tumours resembling condylomas.

Case report

A 57-year-old woman presented in our department with large pedunculated condylomatous tumours in the perianal region (Fig. 1).
Four months earlier she was diagnosed as having lambda light chain myeloma, II A according to Durie and Salmon [2], and received three chemotherapy courses of DAV schedule (dexamethasone, adriamycin [doxorubicin], vincristine). Partial remission of bone marrow plasma-cell infiltration was obtained and the patient received high-dose cyclophosphamide and filgrastim to harvest peripheral autologous CD34+ cells. A further treatment with DHAP schedule (cisplatinum, cytosine arabinoside, dexamethasone) was administered to reduce the tumour burden.
At that point, mucocutaneous lesions began to appear, with ecchymoses in the body folds and eyelid and periorbital purpura. Macroglossia also developed. The tongue became enlarged and firm, showing papules and nodules on its surface.
In perianal area, widespread nodules occurred, coalescing to form large pedunculated condyloma-like tumours with a glossy and smooth surface.
Genital inspection did not reveal clinical significant abnormalities.
Histopathological examination of an excisional biopsy of one perianal nodule revealed in the papillary and reticular dermis the presence of faintly eosinophilic, amorphous masses corresponding to amyloid deposits. A marked thickening of blood-vessel walls due to amyloid infiltration was also present.
There were no histopathological features typical of condylomata acuminata. The dermal eosinophilic material showed an affinity with Congo Red (Fig. 2), and bright green birefringence under polarized light.
One month later, the patient underwent a double autologous stem cell transplantation after high-dose melphalan, but her general condition worsened with the appearance of cardiac failure symptoms. The patient died four months later of congestive heart failure.

Discussion

Amyloid deposition in myeloma-associated systemic amyloidosis occurs as a result of plasma cell dyscrasia, and the amyloid fibrils are composed of immunoglobulin light chain material (protein AL). Amyloidogenic immunoglobulin AL monoclonal proteins appear to be preferentially of lambda type.
Systemic amyloidosis shows a large variety of clinical features. Principal initial examination findings usually include macroglossia, mucocutaneous lesions, hepatomegaly and oedema. Clinically evident mucocutaneous involvement occurs in up to 40% of patients. The most common skin signs consist of petechiae, purpura, and ecchymoses, occurring spontaneously or after minor trauma, and are the result of amyloid infiltration of blood vessel walls. Waxy, smooth, shiny papules, nodules, and plaques are the most characteristic skin lesions [1] Condyloma-like lesions were first reported by S. Wolfram in 1942, in a patient with large pedunculated tumours resembling condylomata lata or lesions of pemphigus vegetans [3]. Ten years later, Goltz reported a patient with multiple myeloma-associated amyloidosis who presented with numerous fibrous pedunculated tumours in the intergluteal cleft and over the sacrum [4].
We have found only one other case of condyloma-like perianal lesions described by Buezo et al. in a 48-year-old woman with multiple myeloma IgA lambda and associated amyloidosis [5], although four cases of nodular vulval lesions, which could resemble condylomas, have been reported in association with systemic amyloidosis [6, 9] or with localized forms (primary localized cutaneous nodular amyloidosis) [7, 8], included in the spectrum of immunoglobulinic amyloidosis, with no evidence of systemic involvement.
Condyloma-like lesions as a consequence of amyloidosis have been described in the perianal region, the gluteal fold and the vulva. These regions are likewise areas of predilection for HPV-induced lesions. The correct diagnosis can be established by histopathological examination and is mandatory before initiation of systemic chemotherapy. n

References

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2. Durie BG, Salmon SE. A clinical staging system for multiple myeloma: correlation of measured myeloma cell mass with presenting clinical features, response to treatment, and survival. Cancer 1975; 36: 842-54.

3. Wolfram S. Lokale tumorfoermige Amyloidose der Haut bei atipish generalisierter (systematisierter) Amyloydose. Arch f Derm u Syph 1942; 184: 289-98.

4. Goltz RW. Systematized amyloidosis. A review of the skin and mucous membrane lesions and a report of two cases. Medicine 1952; 31: 381-409.

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