Journal of Postgraduate Medicine
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Year : 2011  |  Volume : 57  |  Issue : 2  |  Page : 141-142  

EMO syndrome

KVS Hari Kumar1, YS Bisht2, P Prusty1,  
1 Department of Endocrinology, Command Hospital, Lucknow, India
2 Department of Dermatology, Base Hospital, Lucknow, U.P., India

Correspondence Address:
KVS Hari Kumar
Department of Endocrinology, Command Hospital, Lucknow
India




How to cite this article:
Hari Kumar K, Bisht Y S, Prusty P. EMO syndrome.J Postgrad Med 2011;57:141-142


How to cite this URL:
Hari Kumar K, Bisht Y S, Prusty P. EMO syndrome. J Postgrad Med [serial online] 2011 [cited 2019 Dec 7 ];57:141-142
Available from: http://www.jpgmonline.com/text.asp?2011/57/2/141/81881


Full Text

Pretibial myxedema is a rare manifestation of autoimmune thyroid disease. It is described commonly in Graves' disease and seldom in hypothyroidism. [1] Euthyroid individuals with autoimmune thyroiditis rarely have pretibial myxedema. [2] The triad of Exophthalmos, Myxedema, and Osteoarthropathy constitutes EMO syndrome. [3]

In August 2010, a 34-year-old man presented with swelling of legs and exophthalmos of 6-year duration. Individual noticed progressive painless swelling of legs involving the shin area with no diurnal variation. He denied symptoms of underlying cardiac/renal/hepatic/malabsorptive diseases. Patient noticed protrusion of eyeballs without any associated redness, pain, diplopia, or vision loss. Thyroid hormone estimation at the onset of exophthalmos was normal. He consulted various practitioners later on multiple occasions and thyroid hormonal profile repeatedly was normal. Other differential diagnoses leading to bilateral exophthalmos like orbital tumors, inflammation, and cavernous sinus disease were excluded by previous clinicians. He also noticed progressive thickening of skin involving hands, excessive sweating of palms and soles. He denied history of fever, weight loss, palpitations, heat intolerance, and neck swelling. There was no family history of thyroidal illness. He was reviewed by an ophthalmologist and neurophysician in 2009, who considered acromegaly. Serum growth hormone was undetectable after a glucose load and neuroimaging revealed normal sella. Symptoms continued to remain and he was referred to our department.

Examination revealed bilateral exophthalmos, multiple firm small subcutaneous nodules over face with swollen eyelids leading to mechanical ptosis [Figure 1], thick spade-like hands with clubbing, hyperhidrosis, and pretibial myxedema [Figure 2]. There was no tachycardia, goiter, myopathy, tremors, and ophthalmoplegia. Abdominal palpation revealed splenomegaly and rest of the systemic examination was normal.{Figure 1}{Figure 2}

The possibilities considered were cutaneous and visceral leishmaniasis, EMO syndrome, POEMS (Polyneuropathy, Organomegaly, Endocrinopathy, M band, and Skin changes) syndrome, and amyloidosis. Routine blood tests revealed normal hematological and biochemical parameters. Bone marrow and K39 antigen were negative for kala azar. Ultrasonography of abdomen showed hepatosplenomegaly without lymphadenopathy, and upper gastrointestinal endoscopy was normal. His thyroid profile showed total triiodothyronine-106 ng/dL (normal, 60-175), total thyroxine-8.6 μg/dL (4-11.5), thyroid stimulating hormone-1.3 mIU/mL (0.3-4.5), thyroglobulin antibody - 63.7 IU/mL (0-20), and thyroid peroxidase antibody-47.7 IU/mL (0-15). TSH receptor antibody estimation was not done due to financial constraints. Skin biopsy showed mucin deposition and perivascular lymphocytic infiltration in the dermis. Radiological examination of the extremities showed lamellar periosteal new bone formation around the shafts of distal ulna, radials, and metacarpal bones. M band was negative and Congo red stain of tissue from skin and rectal mucosa was negative for amyloid deposition. The patient hails from endemic area of kala azar and in view of cutaneous changes with splenomegaly, we ruled out underlying kala azar infection. Presence of osteoarthropathy precludes the diagnosis of euthyroid Graves' disease. The presence of all three components of EMO syndrome over a period of six years without ever having clinical or biochemical hyperthyrodisim leads us to the diagnosis of EMO syndrome. The patient is being treated with monthly intralesional triamcinolone injection and is under regular follow-up.

EMO syndrome is a rare extrathyroidal syndrome seen in less than 1% of patients with autoimmune thyroid disease. [2] Pretibial myxedema is localized thickening of the skin and occurs due to cross reaction of autoantibodies activating the pretibial fibroblasts. These activated pretibial fibroblasts react with T lymphocytes on thyrotropin receptors and release excess glycosaminoglycans. [4] A subgroup of orbital fibroblasts can differentiate into mature adipocytes with increased expression of thyrotropin receptor. [5] Thyroid dermopathy is seen mostly in females and is usually associated with ophthalmopathy. Dermopathy involves pretibial location in more than 90% of cases and rarely does it involve other areas like upper limbs and lids. The various clinical forms of lesion are nonpitting edema, plaque, nodule, and elephantiasis type. Our case had both nonpitting edema and nodular type of involvement. Treatment of pretibial myxedema is difficult and is done by topical or intralesional steroids. In resistant case, intralesional octreotide has been shown to be of benefit. [6]

References

1Walia R, Bhansali A, Shanmugasundar G, Upreti V, Dutta P. Primary hypothyroidism with infiltrative ophthalmopathy and dermopathy, BMJ Case Reports 2010. doi:10.1136/bcr.08.2009.2218.
2Senel E, Gulec AT. Euthyroid pretibial myxedema and EMO syndrome. Acta Dermatovenerol Alp Panonica Adriat 2009;18:21-3.
3Anderson CK, Miller OF 3rd. Triad of exophthalmos, pretibial myxedema, and acropachy in a patient with Graves' disease. J Am Acad Dermatol 2003;48:970-2.
4Daumerie C, Ludgate M, Costagliola S, Many MC. Evidence for thyrotropin receptor immunoreactivity in pretibial connective tissue from patients with thyroid associated dermopathy. Eur J Endocrinol 2002;146:35-8.
5Bahn RS. Graves' ophthalmopathy. N Engl J Med 2010;362:726-38.
6Felton J, Derrick EK, Price ML. Successful combined surgical and octreotide treatment of severe pretibial myxoedema reviewed after 9 years. Br J Dermatol 2003;148:825-6.

 
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