Abstract
“Shoulder pad” sign resulting from deposition of amyloid in the periarticular soft tissue is rare but it is pathognomonic for immunoglobulin amyloidosis (AL). Although the detection remains a challenge, it gives a strong clue leading to prompt diagnosis.
Keywords: AL amyloidosis, light chain, macroglossia, shoulder pad sign
“Shoulder pad” sign resulting from deposition of amyloid in the periarticular soft tissue is rare but it is pathognomonic for immunoglobulin amyloidosis (AL). Although the detection remains a challenge, it gives a strong clue leading to prompt diagnosis.
1. CASE PRESENTATION
Immunoglobulin amyloidosis is a well‐recognized cause for macroglossia and shoulder pad sign. “Shoulder pad” sign resulting from deposition of amyloid in the periarticular soft tissue is rare but it is pathognomonic for immunoglobulin amyloidosis (AL). Although the detection remains a challenge, it gives a strong clue leading to prompt diagnosis.
An 83‐year‐old woman on a background of diabetes mellitus, hypertension, bronchial asthma, and stable angina presented with a history of increasing lethargy and loss of appetite of 6 months duration. She also noted that her tongue was enlarging in size and increasingly stiff with difficulty in swallowing and talking over the course of the previous 2 years. Physical examination revealed macroglossia, visible enlargement of both anterior shoulders (shoulder pad sign), infiltration of upper lip, and multiple papules and nodules over the chest, neck, and arms (Figure 1). Laboratory studies showed normochromic normocytic anemia, elevated ESR, and mild renal impairment. Serum protein electrophoresis did not show M band but Bence Jones protein was found in urine. Ultrasound examination of abdomen revealed hepatomegaly with early chronic kidney disease, and transthoracic echocardiogram was unremarkable. The biopsy of the tongue was consistent with the diagnosis of amyloidosis (Figure 2A,B). Ultimately, she was diagnosed with AL amyloidosis, with amyloid fibrils derived from the monoclonal light‐chain deposit in the tissues. AL amyloidosis is a well‐recognized cause for macroglossia and “shoulder pad sign” resulting from deposition of amyloid in the periarticular soft tissue which is pathognomonic. 1
FIGURE 1.
It shows macroglossia with enlargement of both anterior shoulders (shoulder pad sign), infiltration of upper lip, and multiple papules and nodules on chest and arms
FIGURE 2.
A, This biopsy (H&E) shows subepithelial nodular extracellular deposits of eosinophilic homogenous material (black arrow). B, Amyloid deposition shows apple green birefringent (long arrow) under polarizing microscopy in contrast with the dermal collagen (short arrow)
The diagnosis of AL amyloidosis is usually established with an abdominal fat pad aspirate because of their ease, convenience, and high yield. Antiplasma cell therapy can be administered also to frail elderly patients. 2 However, considering age and other comorbid diseases, she was sent home on palliative care as per her decision.
CONFLICT OF INTEREST
The authors declare that they have no competing interests.
AUTHOR CONTRIBUTIONS
NS and PN: provided medical care and collected all data; NS, PN, and VS: involved in preparation of manuscript; all authors read and approved the final manuscript.
CONSENT FOR PUBLICATION
Informed written consent for the publication was obtained from the patient.
ACKNOWLEDGMENT
The authors are very grateful to the patient for her consent to publish the images and clinical data. The authors also wish to thank the histopathologists for helping to arrive at a diagnosis.
Suganthan N, Navaradnam P, Sujanitha V. Shoulder pad and macroglossia: “two signs of AL amyloidosis”. Clin Case Rep. 2021;9:1801–1802. 10.1002/ccr3.3782
REFERENCES
- 1. Liepnieks JJ, Burt C, Benson MD. Shoulder‐pad sign of amyloidosis: structure of an Ig kappa III protein. Scand J Immunol. 2001;54(4):404‐408. 10.1046/j.1365-3083.2001.00980.x [DOI] [PubMed] [Google Scholar]
- 2. Nuvolone M, Milani P, Palladini G, Merlini G. Management of the elderly patient with AL amyloidosis. Eur J Intern Med. 2018;58:48‐56. 10.1016/j.ejim.2018.05.004 [DOI] [PubMed] [Google Scholar]