Indian Journal of Dermatology
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E-IJD CORRESPONDENCE
Year : 2016  |  Volume : 61  |  Issue : 1  |  Page : 124
Myeloma associated primary systemic amyloidosis


1 Department of Dermatology, Faculty of Medicine, Universiti Teknologi MARA, Jalan Hospital, Selangor, Malaysia
2 Department of Dermatology, Universiti Teknologi MARA, Selangor, Malaysia
3 Department of Pathology, Universiti Teknologi MARA, Selangor, Malaysia
4 Department of Dermatology, Hospital Raja Permaisuri Bainun, Perak, Malaysia

Date of Web Publication15-Jan-2016

Correspondence Address:
Wan Syameen Afira Wan Ahmad Kamal
Department of Dermatology, Faculty of Medicine, Universiti Teknologi MARA, Jalan Hospital, Selangor
Malaysia
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0019-5154.174166

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How to cite this article:
Kamal WW, Taib T, Noor NM, Jong TJ. Myeloma associated primary systemic amyloidosis. Indian J Dermatol 2016;61:124

How to cite this URL:
Kamal WW, Taib T, Noor NM, Jong TJ. Myeloma associated primary systemic amyloidosis. Indian J Dermatol [serial online] 2016 [cited 2019 Sep 22];61:124. Available from: http://www.e-ijd.org/text.asp?2016/61/1/124/174166


Sir,

Primary systemic amyloidosis (AL amyloidosis) is rare, affecting 5 to 12 per million people per year. [1] Patients may have amyloidosis alone or in association with other plasma cell dyscrasias. Up to 30% of patients with multiple myeloma have coexisting subclinical primary amyloidosis. [2] Cutaneous lesions other than those related to intracutaneous hemorrhage are not frequently seen. [3] We report a case of multiple myeloma associated primary systemic amyloidosis presenting with uncommon cutaneous manifestations.

A 52-year-old Malay lady, with underlying hypertension and ischemic heart disease, presented with bilateral leg edema, weight loss, and lethargy for 6 months. She developed papules and purpuric rash on her face and chest for about a year. Examination revealed macroglossia with waxy papules on the tongue [Figure 1]. There were also multiple erythematous papules on the upper and lower eyelids bilaterally, face, abdomen [Figure 2], and groin area with multiple petechiae, purpura, and ecchymoses. Aside from bilateral pedal edema, examination of other systems was unremarkable. Investigations revealed anemia, renal impairment with proteinuria, and hypercalcemia. Serum electrophoresis showed a significant lambda light chain with lambda Bence-Jones protein in urine electrophoresis. A two-dimensional echocardiography revealed concentrically thickened ventricles with diastolic dysfunction and a hypokinetic basal septum. The left ventricular ejection fraction was 58%. Biopsy of the tongue showed pink acellular eosinophilic homogenous materials in the dermis on hematoxylin and eosin stain under × 400 magnification [Figure 3]. These amyloid deposits demonstrated apple-green birefringence when stained with Congo red [Figure 4]. Immunochemistry staining was positive for lambda light chains [Figure 5]. Biopsies of the antral gastric mucosa, rectal, and papules on the back also showed amyloid deposits. She, however, refused bone marrow examination and was managed with fluid restriction, diuretics, and beta blockers. She succumbed on day 5 of the admission due to cardiac complications.
Figure 1: An enlarged tongue with teeth indentation marks on the sides

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Figure 2: Papules and petechiae on the abdomen

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Figure 3: The amyloid deposits under higher power, H and E stain (tongue biopsy) under ×400

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Figure 4: Apple-green birefringence of amyloid deposits when stained with Congo red and viewed under polarizing microscopy (tongue biopsy) under ×400

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Figure 5: Positivity for lambda immunostains (tongue biopsy) under ×400

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The cutaneous signs of primary systemic amyloidosis are related to amyloid infiltration of the blood vessel walls. Our patient also had multiple papules due to dermal deposition of amyloid. Other rare cutaneous findings include hyperpigmentation, scleroderma-like skin infiltrates, alopecia, and cutis laxa. Amyloid deposition in the heart results in rapidly progressive heart failure because of restrictive cardiomyopathy.

The diagnosis of multiple myeloma in this lady was strongly suspected in view of the detection of serum and urinary monoclonal protein, and the associated end organ damage. The presence of multiple myeloma in primary systemic amyloidosis has been found to represent a poorer prognosis as compared to multiple myeloma alone, even if asymptomatic at diagnosis. [4] Other predictors of poor outcome include clinically apparent cardiac involvement and high levels of free light chains. [5] The amyloidogenic proteins expressed, in this case, are light chains of the λ isotype, which are expressed more frequently than the k isotype. The nonspecific presenting symptoms of this disease often result in a delayed diagnosis. Patients may suffer from complications such as organ dysfunction by the time, treatment is initiated leading to significant morbidity and mortality.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

 
   References Top

1.
Skinner M, Sanchorawala V, Seldin DC, Dember LM, Falk RH, Berk JL, et al. High-dose melphalan and autologous stem-cell transplantation in patients with AL amyloidosis : a0 n 8-year study. Ann Intern Med 2004;140:85-93.  Back to cited text no. 1
    
2.
Rajkumar SV, Gertz MA, Kyle RA. Primary systemic amyloidosis with delayed progression to multiple myeloma. Cancer 1998;82:1501-5.  Back to cited text no. 2
    
3.
Fernandez-Flores A. Cutaneous amyloidosis : a0 concept review. Am J Dermatopathol 2012;34:1-14.  Back to cited text no. 3
    
4.
Vela-Ojeda J, García-Ruiz Esparza MA, Padilla-González Y, Sánchez-Cortes E, García-Chávez J, Montiel-Cervantes L, et al. Multiple myeloma-associated amyloidosis is an independent high-risk prognostic factor. Ann Hematol 2009;88:59-66.  Back to cited text no. 4
    
5.
Palladini G, Campana C, Klersy C. Serum N-terminal pro-brain natriuretic peptide is a sensitive marker of myocardial dysfunction in AL amyloidosis. CJASN 2003;107:2440-5.  Back to cited text no. 5
    


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5]



 

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