Chronic urticaria versus dermatomyositis in a case of T- cell large granular lymphocytic leukemia

Floria Bertolini MD1*, Luigi Clauser, MD2

1Rovigo City Hospital-Dermatological Clinic Via G. Falcone, P. Borsellino, 69 45100 Rovigo, Italy.
2Istituto Stomatologico Italiano, Via Pace, 21-20122 Milano, Italy.

Aim: The reported case involved a complicated diagnostic path, not only because of the usual difficulties specific to chronic urticaria but, also because the trigger was a T-cell large granular lymphocytic leukemia (T-LGLL), a rare type of leukemia with an indolent course whose etiology is still not well known. This leukemia is also known for its propensity to cause autoimmune diseases.

The aim of this study was to identify whether the muscle damage was caused by dermatomyositis or by T-LGLL.

Methods: After elevated muscle enzyme levels had been discovered magnetic resonance imaging (MRI) revealed muscle damage. Consequently, a muscle biopsy was performed in a targeted manner. In addition to muscle biopsy, transmission electron microscopy and anti-CN1A antibody testing were performed.

Results: MRI of the lower limbs and pelvic girdle indicated moderate fibroadipose substitution in many muscles, moderate edema in others. No involvement of the shoulder girdle and upper limbs.

Histological examination of the muscle fibers showed an “inflammatory myopathy with isolated phagocytotic fibers.” Inclusion-body myositis, which is known to be associated with chronic T-LGLL was excluded. On the same biopsy transmission electron microscopy confirmed inflammatory myopathy and anti-CN1A antibodies were positive. DNA extracted from the muscle of the micro-rearrangement for the y-chain of the TCR identified on the DNA extracted from peripheral blood was positive.

Conclusions: Conclusions: Chronic urticaria was an indication of immunoproliferative disease. Myositis was the pathology due to T-LGLL, and dermatomyositis was excluded.

Keywords: Chronic urticaria, myositis, immunoproliferative disease, T- cell large granular lymphocytic leukemia, muscular biopsy

Free Full-text PDF

How to cite this article:
Floria Bertolini, Luigi Clauser. Chronic urticaria versus dermatomyositis in a case of T- cell large granular lymphocytic leukemia. American Journal of Dermatological Research and Reviews, 2021; 4:43. DOI: 10.28933/ajodrr-2021-05-0205


1. McKenna RW, Parkin J, Kersey JH, GajlPec-zalska KJ, Peterson L, Brunning RD. Chronic lymphoproliferative disorder with unusual clinical, morphologic, ultrastructural and mem- brane sur-face marker characteristics. Am. J Med. 1977, 62(4): 588-596. doi: 10.1016/0002 -9343(77)90422-3.
2. Barilà G, Teramo A, Gattazzo C, Zambello R, Semenzato G. Leucemia a grandi linfociti granulari. Ematologia “Diagnosi difficili e rare”. Dynamicon ed. Vol 1 – n.3 – 2014, 49-57.
3. Dearden C. Large granular lymphocytic leuke- mia pathogenesis and management. Br J Hae- matol. 2011; 152(3):273-283. doi: 10.1111/j. 1365-2141.2010.08494.x
4. Zhang R, Shah MV, Loughran TP, Jr. The root of many evils: indolent large granular lymphocyte leukemia and associated disorder. Hematol. Oncol. 2010; 28(3): 105 – 117. doi: 10.1002/ hon.917.
5. Rose MC, Berliner N. T-cell large granular lym-phocyte leukemia and related disorders. Oncolo-gist,2004; 9(3): 247- 258.
6. Bohan A, Peter JB. Polymyositis and dermato- myositis (first of two parts). N Engl J Med. 1975 Feb 13;292(7):344-7. doi: 10.1056/NEJM19750 2132920706.
7. Bohan A, Peter JB. Polymyositis and dermato- myositis (second of two parts). N Engl J Med. 1975 Feb 20;292(8):403-7. doi: 10.1056/NEJM 197502132920706
8. Braun-Falco O, Plewig G, Wolff HH, Burgdorf WHC. Dermatologia ed. Italiana a cura Gelmetti C, Springer-Verlag Italia 2002, 814-818.
9. Dion E, Cherin P, Payan C, Fournet JC, Papo T, Maisonobe T, Auberton E, Chosidow O, Godeau P, Piette JC, Herson S, Grenier P. Mag-netic resonance imaging criteria for dis- tinguishing between inclusion body myositis and polymyositis. J Rheumatol 2002 Sep; 29(9): 1897-906. PMID: 12233884
10. Tawara N, Yamashita S, Xiao Z, Korogi M, Zhang Z , Doki T, Matsuo Y, Nakane S, Maeda Y, Sugie K, Suzuki N, Aoki M, AndoY. Pathomec- hanisms of an-ti-cytosolic 5′- nucleotidase 1A autoanti-bodies in sporadic inclusion body myositis. Ann Neurol. 2017 Apr;81(4):512- 525. doi: 10.1002/ana.24919. Epub 2017 Apr 3. Doi:10.1002/ana.24919

Terms of Use/Privacy Policy/ Disclaimer/ Other Policies:
You agree that by using our site, you have read, understood, and agreed to be bound by all of our terms of use/privacy policy/ disclaimer/ other policies (click here for details)

CC BY 4.0
This work and its PDF file(s) are licensed under a Creative Commons Attribution 4.0 International License.