Lymphomatoid papulosis (LyP) and primary cutaneous anaplastic large cell lymphoma (ALCL) are grouped under the category of cutaneous T-cell lymphomas as CD30+ lymphoproliferative disorders. Though LyP is clinically characterized by chronic recurrent papulonodular cutaneous lesions, it shows malignant features on the histologic findings. LyP is associated with lymphomas, including primary cutaneous ALCL, mycosis fungoides and other lymphoproliferative disorders. A few cases of LyP related to ALCL have been reported in Korea. We report here on an interesting case of primary cutaneous ALCL that developed in succession after LyP in a 38-year-old female.
Adult ; Female ; Humans ; Korea ; Lymphoma ; Lymphoma, Large-Cell, Anaplastic ; Lymphoma, Primary Cutaneous Anaplastic Large Cell ; Lymphoma, T-Cell, Cutaneous ; Lymphomatoid Papulosis ; Lymphoproliferative Disorders ; Mycosis Fungoides

Mycosis fungoides (MF) is the most frequent cutaneous T cell lymphoma (CTCL). Since the major tumor cell of MF is the helper T cell, positive markers are usually CD3, CD4 and CD45RO. Some MFs show CD30 positivity and the major differential diagnosis for MF with CD30 positivity includes transformed MF and MF concurrent with primary cutaneous anaplastic large cell lymphoma and lymphomatoid papulosis. As each disease shows a different prognosis, an exact diagnosis is crucial for proper treatment. We now report a case of 44-year-old male patient with mycosis fungoides which developed several papules on preexisting MF patches. On biopsy of the newly formed papules, CD 30 positive cells were observed and the histologic features were consistent with lymphomatoid papulosis. Both the lesions of lymphomatoid papulosis and MF responded well to narrow band ultraviolet B phototherapy.
Adult ; Biopsy ; Diagnosis, Differential ; Humans ; Lymphoma, Primary Cutaneous Anaplastic Large Cell ; Lymphoma, T-Cell, Cutaneous ; Lymphomatoid Papulosis ; Male ; Mycosis Fungoides ; Phototherapy ; Prognosis

Since the discovery of the CD30 molecule, its expression has been considered a reliable marker for CD30+ lymphomas, including lymphomatoid papulosis, primary cutaneous anaplastic large cell lymphoma and borderline cases. However, CD30+ cells can be observed in Hodgkin's lymphoma, as well as in T-cell and B-cell lymphomas or NK cell lymphomas. Furthermore, it can also be found in reactive inflammatory disorders or in non-lymphoid neoplasms. It is very important to differentiate between lymphomas and reactive inflammatory disorders using a combination of clinical, histological, phenotypic and molecular analyses. We report a case of atypical CD30+ lymphocytic hyperplasia in a 57-year-old man following tick bite.
Bites and Stings ; Hodgkin Disease ; Humans ; Hyperplasia ; Killer Cells, Natural ; Lymphoma ; Lymphoma, B-Cell ; Lymphoma, Primary Cutaneous Anaplastic Large Cell ; Lymphomatoid Papulosis ; Middle Aged ; T-Lymphocytes ; Ticks

CD30+ lymphoproliferative disorders (LPD) represent a spectrum of T-cell lymphoma including lymphomatoid papulosis and anaplastic large cell lymphoma (ALCL). Epidermis overlying cutaneous CD30+ LPD often shows epidermal hyperplasia, hyperkeratosis, crusting, and ulceration and it is difficult to distinguish from carcinoma such as keratoacanthoma (KA) or squamous cell carcinoma (SCC). Several cases of pseudocarcinomatous hyperplasia mimicking KA or SCC in CD30+ LPD have been reported. The relationship between CD30+ LPD and epithelial proliferations has not yet well understood. It was reported that a variety of mediators, including epidermal growth factor (EGF), transforming growth factor-α and EGFR from CD30+ LPD could attribute to epidermal hyperplasia. However, separate and distinct SCC occurring in CD30+ LPD has rarely been reported. Herein, we present a rare case of coexistence of SCC and cutaneous ALCL located on the same region.
Carcinoma, Squamous Cell* ; Epidermal Growth Factor ; Epidermis ; Epithelial Cells* ; Hyperplasia ; Keratoacanthoma ; Lymphoma ; Lymphoma, Large-Cell, Anaplastic ; Lymphoma, Primary Cutaneous Anaplastic Large Cell* ; Lymphoma, T-Cell ; Lymphomatoid Papulosis ; Lymphoproliferative Disorders ; Ulcer

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

The diagnosis of primary cutaneous lymphoma is based on a combination of clinical, histological, immunophenotypic and genetic criteria. Nineteen cases of primary cutaneous lymphomas were studied for clinicopathologic, immunophenotypic, and genetic features. Seventeen (89%) cases were T cell origin and two cases (11%) were B cell origin. CD30-positive cutaneous lymphoproliferative disorder was the most frequent subtype, occupying 42% (8 cases) of the cases. CD8 was positive in 5 cases consisting of 3 cutaneous T cell lymphomas and 2 anaplastic large cell lymphomas. CD4 was positive in 2 cases of mycosis fungoides and 3 cases of lymphomatoid papulosis. Six (67%) of 9 cases of cutaneous T cell lymphoma were positive for TIA-1. Ten (83%) out of 12 cases showed clonal rearrangements of TCR gamma genes, however, one T/NK cell lymphoma and one anaplastic large cell lymphoma did not. EBV association was detected only in T/NK cell lymphomas among 10 cases examined. In conclusion, our study showed higher proportion of CD30-positive lymphoproliferative disorders and less frequent mycosis fungoides in Korea compared to the incidences in Western countries. Our immunostaining results suggested that mycosis fungoides and lymphomatoid papulosis are CD4-positive T cell origin, however, the remaining primary cutaneous T cell lymphoma is predominantly CD8-positive cytotoxic T cell origin.
Diagnosis ; Genes, T-Cell Receptor gamma ; Herpesvirus 4, Human ; Incidence ; Korea ; Lymphoma* ; Lymphoma, Large-Cell, Anaplastic ; Lymphoma, T-Cell, Cutaneous ; Lymphomatoid Papulosis ; Lymphoproliferative Disorders ; Mycosis Fungoides

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

Cutaneous T-cell lymphomas other than mycosis fungoides and Sezary syndrome are heterogeneous; they deseve further scientific attention about their natural history and effective therapy. Pleomorphic T-cell lymphoma is a recently defined lymphoma type that can occur in the skin. We report the case of a man in whom such a tumor manifested itself with multiple subcutaneous lesions. The skin biopsy specimen showed diffuse dermal infiltration of atypical lymphocytes with highly pleomorphic nuclei. Testing for the antibody against HTLV-1 was negative and immunohistochemical staiiiing was compatible with pleomorphic T-cell lymphoma.
Biopsy ; Human T-lymphotropic virus 1 ; Lymphocytes ; Lymphoma ; Lymphoma, T-Cell* ; Lymphoma, T-Cell, Cutaneous ; Mycosis Fungoides ; Natural History ; Sezary Syndrome ; Skin ; T-Lymphocytes*

Primary cutaneous CD30 positive large cell lymphoma is a cutaneous T-cell lymphoma with a favorable prognosis, which is characterized by solitary or localized skin lesions with a tendency of spontaneous regression and frequent relapses. On the basis of the morphologic and immunophenotypic similarities between the large atypical cells in lymphomatoid papulosis and the neoplastic cells in primary cutaneous CD30 positive large cell lymphoma as well as their favorable prognosis, these two diseases are now regarded to be in a spectrum of primary cutaneous CD30 positive lymphoproliferative disorder. We present a borderline case of primary cutaneous CD30 positive lymphoproliferative disorder which recurred on the anatomic site different to the primary lesions despite multiple-agent chemotherapy.
Drug Therapy ; Lymphoma ; Lymphoma, T-Cell, Cutaneous ; Lymphomatoid Papulosis ; Lymphoproliferative Disorders* ; Prognosis ; Recurrence ; Skin

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