- Email: [email protected]
Macroglossia and periorbital ecchymosis
EJINME-03138; No of Pages 2 European Journal of Internal Medicine xxx (2016) xxx–xxx
Contents lists available at ScienceDirect
European Journal of Internal Medicine journal homepage: www.elsevier.com/locate/ejim
Internal Medicine Flashcard
Macroglossia and periorbital ecchymosis Nely Mercy Díaz Mejía a,⁎, Eduardo Josué Flores Umanzor b, Gala Caixal b a b
Department of Medical Oncology, Hospital Universitari Vall d'Hebron, Paseo de la Vall d'Hebron, 119-129, Barcelona 08035, Spain Department of Cardiology, Hospital Clinic, Universitat de Barcelona, Villarroel 170, Barcelona 08036, Spain
a r t i c l e
i n f o
Article history: Received 4 January 2016 Accepted 4 February 2016 Available online xxxx
A 70-year-old woman with past medical history of idiopathic adult hydrocephalus syndrome was admitted to our center due to malaise of one year of evolution without any other symptomatology. Macroglossia with indentation, spontaneous periorbital purpura and cervical-thoracic ecchymotic cutaneous lesions were notable at physical examination. (Figs. 1–3). Hemogram and urine analysis were within normal limits. M protein (IgG) in serum was identified as well free light chain Kappa and Lambda. A lytic lesion was observed in a cranial radiography (Fig. 4). What is the diagnosis?
Fig. 2. Shows peri-orbital haemorrhage (raccoon eyes), one of the classic signs of AL amyloidosis.
Fig. 1. Shows macroglossia with indentation due to amyloid depositions.
⁎ Corresponding author. E-mail address: [email protected] (N.M. Díaz Mejía).
Fig. 3. Shows cervical-thoracic ecchymotic cutaneous lesions due to amyloid infiltration of vessel wall causing capillary wall fragility.
http://dx.doi.org/10.1016/j.ejim.2016.02.010 0953-6205/© 2016 European Federation of Internal Medicine. Published by Elsevier B.V. All rights reserved.
Please cite this article as: Díaz Mejía NM, et al, Macroglossia and periorbital ecchymosis, Eur J Intern Med (2016), http://dx.doi.org/10.1016/ j.ejim.2016.02.010
e2
N.M. Díaz Mejía et al. / European Journal of Internal Medicine xxx (2016) xxx–xxx
Amyloidosis coexists with multiple myeloma in approximately 10% of cases. Mucocutaneous involvement can be the first manifestation of amyloidosis. Macroglossia can occur in almost 20% of cases [1]. Amyloid purpura appears in a minority of patients with amyloidosis. The purpura typically occurs above the nipple line and is often seen in the webbing of the neck, the face, and the eyelids. Raccoon eyes are present in only a minority of patients, but are highly characteristic of AL amyloidosis [2]. Treatment has been delayed awaiting surgery due the idiopathic adult hydrocephalus syndrome, given that treatment involves high risk of neutropenial infections and increased bleeding from thrombocytopenia. Conflict of interest The authors state that they have no conflicts of interest. Fig. 4. Shows a lytic lesion in cranial radiography which is typical of myeloma.
Due to M protein, mucocutaneous and radiographic findings, AL amyloidosis associated with multiple myeloma was suspected. Fibrillar amyloid and bone marrow clonal plasmocytosis (15%) were demonstrated confirming diagnosis.
References [1] Hazenberg BP. Amyloidosis: a clinical overview. Rheum Dis Clin North Am 2013;39: 323–45. [2] Eder L, Bitterman H. Amyloid purpura. N Engl J Med 2007;356:2406.
Please cite this article as: Díaz Mejía NM, et al, Macroglossia and periorbital ecchymosis, Eur J Intern Med (2016), http://dx.doi.org/10.1016/ j.ejim.2016.02.010
Contents lists available at ScienceDirect
European Journal of Internal Medicine journal homepage: www.elsevier.com/locate/ejim
Internal Medicine Flashcard
Macroglossia and periorbital ecchymosis Nely Mercy Díaz Mejía a,⁎, Eduardo Josué Flores Umanzor b, Gala Caixal b a b
Department of Medical Oncology, Hospital Universitari Vall d'Hebron, Paseo de la Vall d'Hebron, 119-129, Barcelona 08035, Spain Department of Cardiology, Hospital Clinic, Universitat de Barcelona, Villarroel 170, Barcelona 08036, Spain
a r t i c l e
i n f o
Article history: Received 4 January 2016 Accepted 4 February 2016 Available online xxxx
A 70-year-old woman with past medical history of idiopathic adult hydrocephalus syndrome was admitted to our center due to malaise of one year of evolution without any other symptomatology. Macroglossia with indentation, spontaneous periorbital purpura and cervical-thoracic ecchymotic cutaneous lesions were notable at physical examination. (Figs. 1–3). Hemogram and urine analysis were within normal limits. M protein (IgG) in serum was identified as well free light chain Kappa and Lambda. A lytic lesion was observed in a cranial radiography (Fig. 4). What is the diagnosis?
Fig. 2. Shows peri-orbital haemorrhage (raccoon eyes), one of the classic signs of AL amyloidosis.
Fig. 1. Shows macroglossia with indentation due to amyloid depositions.
⁎ Corresponding author. E-mail address: [email protected] (N.M. Díaz Mejía).
Fig. 3. Shows cervical-thoracic ecchymotic cutaneous lesions due to amyloid infiltration of vessel wall causing capillary wall fragility.
http://dx.doi.org/10.1016/j.ejim.2016.02.010 0953-6205/© 2016 European Federation of Internal Medicine. Published by Elsevier B.V. All rights reserved.
Please cite this article as: Díaz Mejía NM, et al, Macroglossia and periorbital ecchymosis, Eur J Intern Med (2016), http://dx.doi.org/10.1016/ j.ejim.2016.02.010
e2
N.M. Díaz Mejía et al. / European Journal of Internal Medicine xxx (2016) xxx–xxx
Amyloidosis coexists with multiple myeloma in approximately 10% of cases. Mucocutaneous involvement can be the first manifestation of amyloidosis. Macroglossia can occur in almost 20% of cases [1]. Amyloid purpura appears in a minority of patients with amyloidosis. The purpura typically occurs above the nipple line and is often seen in the webbing of the neck, the face, and the eyelids. Raccoon eyes are present in only a minority of patients, but are highly characteristic of AL amyloidosis [2]. Treatment has been delayed awaiting surgery due the idiopathic adult hydrocephalus syndrome, given that treatment involves high risk of neutropenial infections and increased bleeding from thrombocytopenia. Conflict of interest The authors state that they have no conflicts of interest. Fig. 4. Shows a lytic lesion in cranial radiography which is typical of myeloma.
Due to M protein, mucocutaneous and radiographic findings, AL amyloidosis associated with multiple myeloma was suspected. Fibrillar amyloid and bone marrow clonal plasmocytosis (15%) were demonstrated confirming diagnosis.
References [1] Hazenberg BP. Amyloidosis: a clinical overview. Rheum Dis Clin North Am 2013;39: 323–45. [2] Eder L, Bitterman H. Amyloid purpura. N Engl J Med 2007;356:2406.
Please cite this article as: Díaz Mejía NM, et al, Macroglossia and periorbital ecchymosis, Eur J Intern Med (2016), http://dx.doi.org/10.1016/ j.ejim.2016.02.010