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THROUGH THE LENS
Year : 2019  |  Volume : 10  |  Issue : 1  |  Page : 87-88  

Primary systemic amyloidosis


1 Department of Dermatology, Dayanand Medical College and Hospital, Ludhiana, Punjab, India
2 Department of Ophthalmology, Dayanand Medical College and Hospital, Ludhiana, Punjab, India

Date of Web Publication14-Jan-2019

Correspondence Address:
Sukhjot Kaur Goraya
116 R Model Town, Ludhiana, Punjab
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/idoj.IDOJ_175_18

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How to cite this article:
Batra J, Goraya SK, Goel S. Primary systemic amyloidosis. Indian Dermatol Online J 2019;10:87-8

How to cite this URL:
Batra J, Goraya SK, Goel S. Primary systemic amyloidosis. Indian Dermatol Online J [serial online] 2019 [cited 2019 Apr 25];10:87-8. Available from: http://www.idoj.in/text.asp?2019/10/1/87/250071



A 65-year-old lady presented with asymptomatic soft-to-firm swelling of her bilateral eyelids for the past 4 years [Figure 1]. The lesions were initially discrete and had slowly become confluent in the past 1 year resulting in bulky and drooping upper eyelids. There were extensive purpura on her neck and arms [Figure 2]. Histopathology showed a dense homogenous acellular eosinophilic material throughout the papillary and reticular dermis with clefting and extensive red blood cell extravasation [Figure 3] and [Figure 4]. Congo red staining showed a characteristic apple green birefringence [Figure 5], diagnostic of cutaneous amyloidosis. The patients hemogram, liver function tests, and renal function tests were normal; however, albumin-to-globulin ratio was reversed. Skeletal survey was unremarkable. Bone marrow aspiration revealed a predominance of plasma cells which constituted approximately 15% of all nucleated cells, and serum protein electrophoresis showed a monoclonal M spike in the gamma globulin region. Hence, a diagnosis of primary systemic amyloidosis caused by multiple myeloma was made, and the patient was started on treatment with thalidomide and bortezomib in consultation with the oncologist.
Figure 1: Boggy, soft-to-firm swelling of both upper and lower eyelids bilaterally

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Figure 2: Extensive purpura present over the neck

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Figure 3: Dense homogenous eosinophilic material present throughout the dermis with clefting and extensive red blood cell extravasation (H and E, ×100)

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Figure 4: Homogenous eosinophilic colloid with red blood cell extravasation (H and E, ×400)

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Figure 5: Apple green birefirengence seen with Congo red staining

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Primary systemic amyloidosis (AL amyloidosis) is usually associated with plasma cell dyscrasia or multiple myeloma. It is composed of immunoglobulin light chains. Multiple myeloma is a bone marrow plasma cell dyscrasia diagnosed when there are more than 10% plasma cells in the bone marrow or a plasmocytoma and at least one of the following findings: (1) monoclonal serum protein; (2) monoclonal urine protein; and (3) lytic bone lesions.[1] The prevalence of amyloidosis associated with multiple myeloma varies from 13% to 26%.[2] Cutaneous involvement, seen in 40% of the cases,[3] depends on the site of amyloid deposited. Common cutaneous lesions are petechiae, purpura, ecchymosis, waxy and translucent papules, plaques, and tumefactive lesions. The classical triad of carpal tunnel syndrome, macroglossia, and specific mucocutaneous lesions is seen in half of the patients. In our case, the typical cutaneous manifestations in form of periorbital plaques caused by dermal deposition of amyloidosis were associated with underlying multiple myeloma. Histopathology with hematoxylin and eosin and Congo red staining confirmed the diagnosis of amyloidosis, whereas bone marrow finding of 15% plasma cells and a monoclonal M spike confirmed multiple myeloma. Rectal biopsy, the gold standard for diagnosing systemic amyloidosis, could not be performed.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
   References Top

1.
Y−cel A, Akman A, Denli YG, Acar MA, Karakas M, Hazar B, et al. Acase of systemic amyloidosis associated with multiple myeloma presented as macroglossia and purpura. J Eur Acad Dermatol Venereol 2004;18:378-9.  Back to cited text no. 1
    
2.
López L, González K, Navarrete G, Novales J, Guarneros A, Cortés B, et al. Multiple myeloma and systemic amyloidosis. Int J Dermatol 2008;47:165-7.  Back to cited text no. 2
    
3.
Alvarez-Ruiz SB, García-Río I, Daudén E. Systemic amyloidoses. Actas Dermosifiliogr 2005;96:69-82.  Back to cited text no. 3
    


    Figures

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



 

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