| lymphomatoid papulosis (LyP) is a chronic self-healing cutaneous eruption which is clinically benign but histologically malignant. Lesions occur episodically over the trunk and limbs. We describe four patients with regional LyP. All were male, with a range in age at onset from 12 to 47 years. In all cases, lesions were confined to a segmental unilateral area. Two patients had type A and two type B LyP. We have long-term follow-up on one patient whose lesions were limited to the right buttock for more than 20 years before more widespread lesions developed. Another patient with lesions on the left flank had mycosis fungoides limited to the same region. Only one other case of LyP presenting in a regional distribution has previously been described. ( info) |
2/57. Lymphoma- and leukemia-associated cutaneous atypical CD30 T-cell reactions. Cutaneous CD30 lymphoid infiltrates appear cytologically atypical and occasionally may be misinterpreted as recurrent disease when they occur in patients treated for other primary hematologic malignancies. We recently encountered two such cases and present our findings. One patient with B-cell lymphoma and another with myeloid leukemia developed cutaneous eruptions after chemotherapy displaying highly atypical perivascular lymphoid cells on histology that mimicked recurrent disease. In both cases, the lymphocytes were CD30 T cells by immunohistochemistry. The skin lesions spontaneously resolved and have not recurred. Because one case was initially misinterpreted as recurrent leukemia, we conclude that close clinical correlation and immunophenotypic confirmation should be done for atypical cutaneous lymphoid infiltrates in patients with primary hematologic malignancies. We discuss the differential diagnosis of atypical CD30 infiltrates in this setting, which include recurrent lymphoma or myeloid leukemia, primary cutaneous anaplastic large cell lymphoma (ALCL), lymphomatoid papulosis (LyP), carbamazepine-induced CD30 pseudolymphoma, viral infection and an atypical eruption of lymphocyte recovery. ( info) |
3/57. Is there a special relationship between CD30-positive lymphoproliferative disorders and epidermal proliferation? The relationship between CD30 lymphoma and epithelial proliferations is not well defined. CD30 lymphoma and lymphomatoid papulos (LyP) share immunohistochemical epitopes and some other features. A single case of LyP associated with multiple keratoacanthomas (KAs) was recently reported. We report two cases of atypical lymphocytic proliferation with features of CD30 lymphoma and LyP intimately associated to KA and squamous cell carcinoma (SCC), KA type. This similar combination of an epidermal tumor and apparent involvement with atypical lymphocytic infiltrates raises the possibility of an association between the two entities. We speculate that the association may be more than expected to occur by chance and suggest several mechanisms by which the association may evolve. ( info) |
| A 60-year-old woman was referred to the Department of Dental medicine at Long Island Jewish Medical Center for evaluation of multiple lesions of the tongue. She reported a long history of recurrent papular cutaneous eruptions that waxed and waned. A biopsy specimen of one of the cutaneous lesions was diagnosed as lymphomatoid papulosis. Sporadic, recurrent oral ulcers that resolved spontaneously were noted 10 to 14 days before the initial visit. These ulcers had recurred for the past 17 years. The most recent oral lesion was an erythematous, irregular, solitary ulcerated area on the middle third dorsum of tongue. The area quickly enlarged, ultimately developing extensive surface necrosis. Shortly after, a similar lesion on the posterior dorsum of the tongue developed. biopsy specimens of the former lesion showed numerous, large, atypical, pleomorphic, and hyperchromatic cells with abundant mitoses. The large, atypical cells were immunohistochemically proven to be T lymphocytes. A diagnosis of lymphomatoid papulosis was made. Two weeks later, the tongue lesions had spontaneously and totally resolved. The clinical, histomorphologic, and immunohistochemical features, as well as gene rearrangement studies of this rare entity, are presented. ( info) |
| BACKGROUND: Previous reports have found an association between lymphomatoid papulosis and hypereosinophilic syndrome, as well as lymphomatoid papulosis and lymphoma. In the current study the authors report what to their knowledge is the first reported case of these three diseases occurring simultaneously in the same patient. methods: The authors followed the clinical course of a 64-year-old man with lymphomatoid papulosis associated with severe hypereosinophilic syndrome complicated by involvement of the lungs and heart. RESULTS: After 6 years of follow-up, the patient developped a large T-cell, CD30 positive lymphoma. The bone marrow biopsy was typical of hypereosinophilic syndrome associated with fibrosis, with focal lymphomatous infiltrates comprised of large cells resembling the type A cells of lymphomatoid papulosis. Complete remission of the lymphoma was obtained with chemotherapy. CONCLUSIONS: This exceptional case report suggests a link between the three diseases. lymphomatoid papulosis belongs to the spectrum of CD30 positive lymphoproliferative disorders and CD30 positive lymphocytes of lymphomatoid papulosis are known to have a Th2 profile with possible secretion of eosinopoietic cytokines. ( info) |
6/57. Mucosal involvement in a patient with lymphomatoid papulosis. We report a 38-year-old woman with lymphomatoid papulosis (LyP) who had nodular and papulovesicular lesions develop on the genital area and oral commissure. Mucosal involvement in LyP is a rare event, and its clinical relevance is still unknown. Aggressive therapies are not recommended in patients with LyP with either cutaneous or mucosal involvement. ( info) |
7/57. CD30 lymphoproliferative disorder: primary cutaneous anaplastic large cell lymphoma followed by lymphomatoid papulosis. CD30 large anaplastic lymphoid cells are seen in anaplastic large cell lymphoma (ALCL), and also in lymphomatoid papulosis (LyP) and other lymphoproliferative disorders. It can be difficult precisely to categorize these disorders with CD30 cells. We report a case of primary cutaneous CD30 ALCL with systemic metastases in whom the clinical disease subsequently evolved into LyP. The patient was initially administered cisplatin and etoposide and made a good response. Eighteen months later, recurrent, self-healing cutaneous small nodules appeared around the original tumour site without any systemic involvement. Histopathological examination of the recurrent lesions revealed infiltration with a mixture of cells that included neutrophils, eosinophils and CD30 large anaplastic cells cytologically identical with those in the primary lesion. The anaplastic cells in both the primary and recurrent lesions were positive for monoclonal antibodies CD30, CD25 and a monoclonal antibody directed against the chimeric protein p80(NPM-ALK). These observations suggest the possibility that the ALCL and the subsequent LyP represent different clinical manifestations of proliferation of the same clone. ( info) |
8/57. lymphomatoid papulosis and Ki-1 anaplastic large cell lymphoma occurring concurrently in a pediatric patient. lymphomatoid papulosis (LyP) is a benign, self-healing, papular eruption that can wax and wane over the course of time. Transformation to T-cell lymphoma has been well documented in 10% to 20% of adults with LyP, but there are have been no cases reported in patients younger than age 26 years. We describe the first pediatric patient, a 16-year-old girl, who had clinical features of LyP and concurrently was found to have a lesion diagnosed as Ki-1 anaplastic large cell lymphoma. After treatment with chemotherapy, she has been in continuous remission for 16 months. ( info) |
| We report a case of lymphomatoid papulosis (LyP) that occurred in a 44-year-old Japanese male patient. Reddish papules with a small number of pustules and nodules were observed on the extremities, chest and upper back. Most lesions were also associated with central necrosis, ulceration and crusting, and regressed spontaneously within 4 to 6 weeks. Histopathological examination revealed wedge-shaped dense cellular infiltrate in the dermis, which was mixed with large atypical lymphoid cells, small lymphocytes, eosinophils and neutrophils. These large atypical cells expressed CD30 on their cell membrane and cytoplasm. Rearrangement of the T-cell receptor (TcR) beta-chain gene was detected in the skin lesion. Lymphadenopathy with histopathologic change similar to the skin lesions, but without TcR gene rearrangement, was found at the left inguinal area. Systemic administration of methotrexate (7.5-15.0 mg/week) was found to be dramatically effective in resolution of skin lesions and prevention of their recurrence. ( info) |
| lymphomatoid papulosis (LyP) is defined as a recurrent self-healing papulonodular eruption with the histological features of a (CD30 ) cutaneous T-cell lymphoma. The atypical cells usually have a CD3 /-, CD4 /-, CD8-, CD30 , CD56- T-cell phenotype. We report an unusual case of LyP, in which the atypical cells expressed a CD3-, CD4-, CD8-, CD30 , CD56 phenotype. Detailed phenotypic and genotypic analysis confirmed that these cells had a natural killer (NK)-cell phenotype. Lymphomas with an NK-cell phenotype usually have a poor prognosis. However, the waxing and waning of papular lesions for more than 20 years and the excellent response to low-dose oral methotrexate in this patient suggest similar clinical behaviour to LyP cases with a T-cell phenotype. ( info) |