128
SHORT COMMUNICATION
ActaDV ActaDV
Advances in dermatology and venereology Acta Dermato-Venereologica
Primary Cutaneous Aggressive Epidermotropic CD8 + T-cell Lymphoma with Brain Involvement and Mimicking Dermatomyositis
Hitoshi TERUI , Kenshi YAMASAKI , Saaya YOSHIDA and Setsuya AIBA * Department of Dermatology , Tohoku University Graduate School of Medicine , Seriryomachi 1-1 , Aoba-ku , Sendai , Miyagi , 980-8574 , Japan . * E-mail : saiba @ med . tohoku . ac . jp Accepted Jun 8 , 2017 ; Epub ahead of print Jun 9 , 2017
Primary cutaneous aggressive epidermotropic CD8 + T- cell lymphoma ( PCAETL ) is a rare variant of cutaneous T-cell lymphoma that is characterized by epidermotropic infiltrates of CD8 + cytotoxic T cells , rapid progression , and poor prognosis ( 1 – 5 ). We report here an unusual case presenting with aggressive involvement , including skin , thigh muscle , and intracranial lesions .
CASE REPORT
A 17-year-old Japanese male , who had been healthy all his life , presented with a 3-month history of swelling of the left parotid gland followed by bilateral upper eyelid swelling with no systemic symptoms . There was no history of allergy , medication or familial medical history . The patient was referred to the Hematology and Rheumatology Department in our hospital for further evaluation . Meanwhile , the patient developed lower extremity myalgia while walking , and weight loss without anorexia . Laboratory examination revealed elevation of serum creatinine phosphokinase ( CPK ; 956 U / l ) and aldolase ( 31.7 IU / l ), no elevation of
C-reactive protein , and negative for anti-nuclear antibody , anti- Sm antibody , anti-Jo-1 antibody , anti-PL-7 antibody , anti-PL-12 antibody , anti-EJ antibody , and anti-KS antibody . Serum levels of immunoglobulins IgG , IgA and IgM were within normal range . An electromyogram , magnetic resonance imaging ( MRI ) of the thigh muscle , and thigh muscle biopsy revealed inflammation in the patient ’ s thigh muscles .
Our department was consulted based on suspicion of dermatomyositis due to those findings . Physical examination revealed facial swelling , especially on the bilateral upper and lower eyelids with some erythema , suggestive of heliotrope eyelids ( Fig . 1a ). Gottron ’ s sign or nail-fold capillary changes were not observed on the patient ’ s hands . The first skin biopsy of the left eyelid revealed vacuolar degeneration accompanied by lymphocyte infiltration into the dermo – epidermal junction and the follicular infundibulum . Since there were several atypical cells among the infiltrating lymphocytes , a conclusive diagnosis of dermatomyositis was suspended because we could not exclude the possibility of cutaneous lymphoma . At this time , the soluble interleukin-2 receptor level was elevated to 3,067 U / ml . Oral corticosteroid , 50 mg / day , had been administered to the patient for treatment , which was effective for the facial and eyelid swelling . However , the resolution of diffuse swelling revealed asymptomatic erythematous induration on the nasolabial fold and the chin ( Fig . 1b ). A skin biopsy was taken from the lesion . One day after the biopsy the patient suddenly developed systemic fatigue , headache , anorexia , nausea , difficulty moving the left arm , signs of meningeal irritation and cerebellar ataxia . Magnetic resonance imaging ( MRI ) of the brain demonstrated an increased fluid-attenuated inversion recovery ( FLAIR ) signal deep in multiple parts of the cerebral cortex ( Fig . 1c ), suggesting posterior reversible encephalopathy syndrome , although no abnormal signal had been found in the brain MRI that had been taken approximately 2 weeks previously . Electroencephalogram and cerebral scintigraphy revealed severe brain damage , especially in the right hemisphere . Whole-body screening with ( 18 ) F-fluorodeoxyglucose positron emission tomography / computed tomography showed no significant abnormalities . The skin biopsy from the lesion revealed massive infiltration of atypical lymphocytes into the dermo – epidermal junction and
Fig . 1 . Clinical and dermatopathologic features . ( a ) Facial swelling , especially on the bilateral upper and lower eyelids with erythema , on the first visit . ( b ) Erythematous induration on the skin of the nasolabial fold and the chin during treatment with oral corticosteroids . ( c ) A magnetic resonance imaging ( MRI ) study of the brain revealed an increased fluid-attenuated inversion recovery ( FLAIR ) signal in multiple parts of the cerebral cortex . ( d ) Skin biopsy of an erythematous induration revealed dense inflammatory infiltration from the dermoepidermal junction to subcutaneous tissue , and ( e ) vacuolar degeneration and epidermotropic infiltration of atypical lymphocytes ( haematoxylin-eosin staining . Low magnification × 40 , scale bar : 5 µ m . High magnification × 100 , scale bar : 500 µ m .). doi : 10.2340 / 00015555-2728 Acta Derm Venereol 2018 ; 98 : 128 – 129
This is an open access article under the CC BY-NC license . www . medicaljournals . se / acta Journal Compilation © 2018 Acta Dermato-Venereologica .