| Background:Mycosis fungoides (MF), the most common primary cutaneous T-cell lymphoma, has an upward trend in incidence for the past few years. It usually has an indolent course and multiple variants and subtypes. The diagnosis is difficult especially in the early stages, thus we need to confirm the diagnosis combined with clinical forms, histopathological, immunohistochemical and T-cell receptor gene rearrangement so as not to delay the treatment.Case presentation:A 25-year old female presented to our hospital with the complaint of generalized rashes and itching for five years and aggravated for two months. The coin-sized rashes companied with itching appeared on the body five years ago without clear reasons; some rash-like patches could be enlarged into annular shape. The patient was diagnosed as’erythema annulare centrifugum’ a year ago in other hospital. Then the patient was suspected to be’mycosis fungoides and lupus erythematosus’seven months ago. The pathological histology test showed the mild hyperkeratosis, epidermal hyperplasia, cellular edema, liquefaction degeneration in the basal layer, mild edema in the dermal papilla layer, and moderate amount of lymphocytes infiltration around the superficial vessels; though the result of serum immunological examination was normal. Various folk prescriptions were used by patient herself; however, the symptom was not improved. The rapid spreading of rash around the body was appeared in recent two months; the affected skin area was enlarged with partial ulceration and accompanied with severe itching. Therefore the patient was admitted in our hospital for further diagnosis and treatment. There was no other particular symptoms except the occasionally fever, with usual vigor and diet; the urine and feces were normal, and the body weight had no obvious alteration. The patient was unemployed and healthy without long-term sun exposure history or abnormal hobbies. In addition, the patient had no systemic disease or tumor; there was no family history of her disease alike, no radioactive material contact history or radiation experiences. Physical examination:Body temperature was 37.5℃; the liver, spleen, or the superficial lymph nodes were not tangible on the body surface. Dermatology examination showed that there were reddish patches with clear boundaries scattered around face, neck, and trunk, with fine scales on the surface. The red plaques with varied sizes were soft and scattered around the trunk, hips, and thigh, with dry, rough and ulcerative surface; some of the lesions showed papillomatosis or gyrus-like alteration. The varied sized annular rashes whose margin was uplift, thickening, and desquamation were scattered around the limbs. Laboratory Tests:The blood routine test revealed WBC at 4.64×10e9/L, NEUT 56.80%、MONO 12.5%、 HB111g/L、PLT199×10e9/L. No abnormal lymphocytes were found in peripheral blood film. The liver functions showed total protein 60.8g/L and albumin 38.1g/L. Urine routines test showed weakly positive for protein while negative for BLD. The renal fuctions showed normal. Virus tests for HIV, HCV and HBV were also negative. Immunological examinations showed that the antinuclear antibodies were 1:100 with extractable nuclear antigens and ds-DAN negative. Whole abdomen CT, thorax CT and lymphonodes mandibulares color doppler ultrasound respectively observed the lymphadenopathy in bilateral groin, axillary, and lower mandible. Reactive lymphoid hyperplasia was likely diagnosed after lymph node aspiration cytology. The histopathology specimens from the continuous lesions in the skin of back, buttock, and thigh showed epidermis hyperkeratosis, mild acanthosis, abnormallymphctyes and formed Pautrier’s microabscesses. Multiple mononuclear cell infiltration was found in the dermis. Immunohistochemistry revealed that the skin lesions in the back were positive for CD3, CD4, but negative for CD8, CD20, or CD79a, while the skin lesions in the thigh were positive for CD2, CD3, CD4, CD5, CD7(scattered+),CD8(scattered+),CD30(scattered+),CD38(scattered+),CD68(scattered +),Ki67(+)<15%,TIA(scattered+),Gran-B(scattered+),PD-1 (scattered+),negative for CXCL-13, CD20, CD21, CD79-α. Lymphoma gene rearrangement revealed that instead of B cells, monoclonal hyperplasia of T cells was found in the specimens. qRT-PCR showed that the EBV DNA level was normal, and EBER was negative.Diagnosis:Mycosis fungoides (MF). Treatment:whole-body NB-UVB irradiation, oral acitretin capsule 20 mg/d, and intramuscularly injected with thymopentin and IFN-α2b every other day. However, The plaques and infiltration were improved after 2 months, but new lesions developed and part of rashes had been enlarged. It revealed uncertain prognosis and expected further observation.Conclusion:1. MF, a primary cutaneous T-cell lymphoma, is difficult to diagnose in its early stages because the symptoms and skin biopsy findings are various and similar to those of other skin conditions.2. It takes multiple skin biopsy samples and long-time observations, combine with various testing methods, such as histopathology, Immunohistochemistry, and TCR gene rearrangement to make a confirmed diagnosis of MF.3.In addition to cure, the treatment of MF now aims to maximize periods of remission or stable disease and minimize treatments and toxicities to avoid hasten death. |
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