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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.