OBJECTIVE: To report the clinical characteristics, diagnosis, treatment and prognosis of one patient with primary cutaneous CD8⁺ aggressive epidermotropic cytotoxic T-cell lymphoma (CD8⁺ PCAECTL), and to strengthen the understanding of this extremely rare type of lymphoma. METHODS: The clinical manifestations, diagnosis, treatment course, and prognosis of one patient with CD8⁺ PCAECTL admitted to our hospital were retrospectively analyzed. RESULTS: The patient is a 42-year-old female, with infiltrative skin rash on naso-facial and back as the main clinical manifestations. After pathological examination of the affected skin tissue, immunohistochemistry, molecular biology, and imaging, the diagnosis was confirmed as CD8⁺ PCAECTL, T3aN₀M₀ stage. Alternating chemotherapy with CHOP/HD-MTX (methotrexate, 6 g/m²) regimen was administered, and achieved complete remission (CR) after 4 cycles. After undergoing chemotherapy with DHAP regimen (cisplatin 100 mg/m², d 1 + cytarabine 2 g/m², q 12h, d 2 + dexamethasone 40 mg/d, d 1-4), the patient was mobilized for peripheral blood stem cells using recombinant human granulocyte colony-stimulating factor (G-CSF), and a sufficient number of CD34⁺ cells were successfully collected. Preconditioning was conducted with the BEAM regimen, followed by consolidation therapy with autologous hematopoietic stem cell transplantation (AHSCT). The patient remained in a disease-free survival state after 20 months of follow-up post-AHSCT. CONCLUSION CD8⁺ PCAECTL is extremely rare in clinical practice, with insidious onset and difficult early diagnosis. It is mainly characterized by the proliferation of epidermotropic CD8⁺ cytotoxic T cells and aggressive clinical course. At present, there is still no unified standard for the optimal treatment regimen, and the prognosis is very poor. Consolidation therapy with AHSCT after achieving remission through induction chemotherapy can improve the survival and prognosis of the CD8⁺ PCAECTL patients.
Humans ; Female ; Adult ; Antineoplastic Combined Chemotherapy Protocols/therapeutic use* ; Lymphoma, T-Cell, Cutaneous/diagnosis* ; Retrospective Studies ; Prognosis ; CD8-Positive T-Lymphocytes ; Skin Neoplasms/diagnosis* ; T-Lymphocytes, Cytotoxic

For diagnosis of rare conditions, consistent follow-up on the part of the patient as well as a high index of suspicion on the part of the physician is needed. Evaluation of the management should be done in the event that patients do not respond to treatment.

This is a case of a 33 year old female who fifteen months prior, noticed erythematous scaly plaques on her cheeks with mild pruritus. She was treated for psoriasis with Halobetasol ointment and Petroleum Jelly, which had partial resolution. Twelve months prior, she was prescribed Methotrexate, again achieving partial resolution. Biopsy was done suggestive of Folliculotropic Mycosis Fungoides. Methotrexate was increased, and additional medications were prescribed, leading to lesion resolution. However, she was lost to follow-up and experienced worsening symptoms. One month prior, biopsy was repeated and again showed Diffuse Lymphocytic Dermatitis positive for CD3+, CD4+, CD5+, CD8-, CD20-, CD30-, and loss of staining for Pan-T cell markers, CD2- and CD7. She is currently managed with regular sessions of Narrowband UVB.

These rare cases are few but more often than not, they are easily missed and when caught are usually progressed and already difficult to treat. Physicians must be vigilant in treating patients, even if they initially diagnose it to be a commonly seen and easily managed disease. Skin Directed therapy is done with PUVA and NBUVB with complete response in 30-70%3. For prognosis, early stages are favorable with a 94% 5 year survival rate, decreasing to 69% after tumor development.

Mycosis Fungoides is the most common type of primary cutaneous lymphoma. Early in its course, it usually presents as erythematous patches and plaques similar to a lot of cutaneous conditions. A 68-year-old male presented with a 13-year history of multiple erythematous patches and plaques on the arms and trunk. The lesions were pruritic and chronically relapsing over the years, temporarily relieved by topical corticosteroids. Thereafter, there was onset of multiple nodules on the trunk, extremities and face. Initial biopsy was done by a different dermatologist, revealed Hansen’s Disease, Borderline Borderline spectrum. Fitefaraco stain was positive but no bacillary index was given. The patient was subsequently started on rifamcpicin 600mg/capsule once a day, Ofloxacin 400mg/tablet once a day, and Minocycline 100mg/capsule once a day. The lesions were noted to worsen, eventually developing ulcerations over the trunk and extremities prompting referral to our institution. The biopsy results were as follows: Cutaneous T-Cell Lymphoma, diffuse cluster of differentiation (CD) 3 staining, focal CD20 staining, and negative FiteFaraco stain. The histopathologic findings combined with the clinical presentation led to the diagnosis of Mycosis Fungoides. He was then referred to medical oncology for proper staging and definitive management. The prognosisof Mycosis Fungoides is generally dependent on the stage as determined by the extent of skin involvement as well as presence of lymph node invasion and/or metastasis.
Mycosis Fungoides ; Lymphoma ; T-Cell ; Cutaneous

Humans ; Lymphoma, T-Cell, Cutaneous/complications* ; Neuroglia ; Pruritus/pathology* ; Skin Neoplasms/complications* ; Spinal Cord/pathology* ; Sympathetic Nervous System

Subcutaneous panniculitis-like T-cell lymphoma (SPTCL) is a very rare cutaneous malignant lymphoma derived from cytotoxic T cells that mainly involves subcutaneous adipose tissue rather than epidermis and dermis. It usually occurs in young and middle-aged population, and the etiology is currently unclear. Clinically, SPTCL is characterized by subcutaneous plaques, nodules, and skin ulcers with swell and ache, mainly presenting in limbs and trunk. SPTCL has been restricted to cases that express α/β phenotype, whereas cases with γ/δ phenotype are categorized to cutaneous γ/δ
Aged ; Humans ; Lymphoma, T-Cell ; Lymphoma, T-Cell, Cutaneous ; Middle Aged ; Panniculitis ; Skin Neoplasms

Primary cutaneous anaplastic large cell lymphoma (C-ALCL) is rare among skin malignancies. C-ALCL usually manifests as reddish or violet nodules. Surgical excision or radiation therapy is generally considered as first-line therapy, but a clinically aggressive disease may require multiagent chemotherapy. Establishing a proper diagnosis of C-ALCL is challenging but should be made to avoid inappropriate treatment and its consequences. The authors report a case of medically resolved C-ALCL in an 81-year-old man presented with well-defined nodular lesions on the forehead.
Aged, 80 and over ; Diagnosis ; Drug Therapy ; Forehead ; Humans ; Lymphoma, Large-Cell, Anaplastic ; Lymphoma, Primary Cutaneous Anaplastic Large Cell ; Lymphoma, T-Cell ; Skin ; Viola

Primary cutaneous anaplastic large cell lymphoma (C-ALCL) is a rare subtype of primary cutaneous lymphoma with a favorable prognosis. Primary cutaneous CD30+ lymphoproliferative disorders, which include C-ALCL and lymphomatoid papulosis, are the second most common group of cutaneous T-cell lymphomas. C-ALCL is comprised of large cells with anaplastic, pleomorphic, or immunoblastic cytomorphology, and indeed, more than 75% of the tumor cells express the CD30 antigen. C-ALCL clinically presents with solitary or localized reddish-brown nodules or tumors, and sometimes indurated papules, and they may be with ulceration covering with dark eschar. Multifocal lesions are seen in 20% of the patients. Extracutaneous dissemination, which mainly involves the regional lymph nodes, occurs in 10% of patients. A 69-year-old man noticed a mild elevated cutaneous lesion containing central ulceration covering with brownish black necrotic tissue on the right lower lip, and the lesion was surgically removed. After the first operation, another skin lesion was developed and the histological examination confirmed the diagnosis, C-ALCL. Eight specimens were excised during the 7-month follow-up period. The patient started the treatment with low-dose oral methotrexate (15 mg/wk) and there was no recurrence for 11 months.
Aged ; Antigens, CD30 ; Diagnosis ; Follow-Up Studies ; Humans ; Lip ; Lymph Nodes ; Lymphoma ; Lymphoma, Primary Cutaneous Anaplastic Large Cell ; Lymphoma, T-Cell, Cutaneous ; Lymphomatoid Papulosis ; Lymphoproliferative Disorders ; Methotrexate ; Prognosis ; Recurrence ; Skin ; Ulcer

Introduction: Folliculotropic mycosis fungoides is a rare and aggressive subtype of mycosis fungoides characterized by small to medium-sized malignant T-cells that typically infiltrate the hair follicle. It represents 4% of primary cutaneous lymphomas and less than 10% of patients with MF. Case Summary: We report 2 cases of 80-year old female, who presented with a 3-year history of an erythematous plaque on the left infraorbital area and forehead, and the other with an 8-month history of multiple erythematous indurated plaques on the left cheek, left eyebrow and malar area. Clinical presentation, histopathology and immunohistochemistry findings revealed a diagnosis of folliculotropic mycosis fungoides. Conclusion Folliculotropic mycosis fungoides has distinct clinical and microscopic features. Evaluation of clinical, pathologic, and immunophenotypic findings are important to identify this rare form of cutaneous T-cell lymphoma.
Mycosis Fungoides ; Lymphoma, T-Cell, Cutaneous ; Mucinosis, Follicular

BACKGROUND: Skin cancer is the most common other primary cancer in patients with lymphoma. However, an intriguing association between cutaneous lymphoma and other primary cancers has been suggested in a few studies. OBJECTIVE: This study investigated other primary cancers in patients with cutaneous lymphoma to evaluate the risk for occurrence of each type of cancer. METHODS: We screened for other primary cancers in 428 patients with cutaneous lymphoma. Clinical features were analyzed according to the lineage and origin of the lymphomas. We calculated the standardized incidence ratio with statistical analysis for each group according to age. RESULTS: Among 330 patients with cutaneous T cell lymphoma and 98 with cutaneous B cell lymphoma, a total of 43 cancers in 38 patients were finally included. Other primary cancers were prevalent in patients with cutaneous B cell lymphoma and patients with secondary cutaneous lymphoma. However, those differences were not significant when the age was calibrated by multiple logistic regression. Metachronously higher standardized incidence ratios were observed for primary lung (standardized incidence ratio [SIR], 14.81; 95% confidence interval [CI], 3.05~39.54), skin (SIR, 68.05; 95% CI, 14.03~181.62), and breast (SIR, 12.91; 95% CI, 1.56~41.41) cancers with statistical significance. CONCLUSION: Other primary cancers more preferentially occurred in patients with cutaneous lymphoma. Clinicians should carefully examine patients with cutaneous lymphoma for other cancers, especially lung, skin, and breast cancers.
Breast ; Humans ; Incidence* ; Logistic Models ; Lung ; Lymphoma* ; Lymphoma, B-Cell ; Lymphoma, T-Cell, Cutaneous ; Skin ; Skin Neoplasms

PURPOSE: We aimed to evaluate clinical outcomes including progression-free survival (PFS), overall survival (OS), partial response, and complete response in patients who underwent radiation therapy (RT) for mycosis fungoides (MF). Also, we sought to find prognostic factors for clinical outcomes. MATERIALS AND METHODS: Total 19 patients confirmed with MF between 1999–2015 were retrospectively reviewed. Clinical and treatment characteristics, clinical outcomes, and and toxicities were analyzed. RESULTS: Eleven patients were treated with total skin electron beam radiotherapy (TSEBT) and 8 patients with involved field radiation therapy (IFRT) with median dose of 30 Gy, respectively. The median time interval from diagnosis to RT was 2.6 months (range, 0.4 to 87.3 months). The overall response rate was 100%; 11 patients (57.9%) had a complete response and 8 patients (42.1%) a partial response. The presence of positive lymph node at the time of consultation of RT was associated with lower OS (p = 0.043). In multivariate analysis, PFS was significantly lower for patients with increased previous therapies experienced following RT (p = 0.019) and for patients showing PR during RT (p = 0.044). There were no reported grade 3 or more skin toxicities related with RT. CONCLUSION: Both IFRT and TSEBT are effective treatment for MF patients. Patients with short disease course before RT or complete response during RT are expected to have longer PFS. Positive lymph node status at the initiation of RT was associated woth poor OS, suggesting other treatment modalities such as low-dose RT for patients with low life-expectancy.
Diagnosis ; Disease-Free Survival ; Humans ; Lymph Nodes ; Lymphoma, T-Cell, Cutaneous ; Multivariate Analysis ; Mycosis Fungoides ; Radiotherapy ; Retrospective Studies ; Skin