ABSTRACT
El linfoma de células T epidermotropo agresivo CD8+ o linfoma de Berti es un subtipo infrecuente de los linfomas primarios cutáneos, descripto en 1999. Aún se considera una entidad provisional en la última clasificación de linfomas cutáneos primarios de la Organización Mundial de la Salud. Predomina en los hombres adultos y se manifiesta con pápulas, placas hiperqueratósicas y tumores ulcerados, diseminados y de inicio agudo. En la histopatología, es característica la presencia de un infiltrado de linfocitos atípicos medianos CD8 +, con epidermotropismo de patrón pagetoide. Tiene un comportamiento agresivo y es de mal pronóstico, con una sobrevida a los 5 años menor del 40%. Si bien esta entidad es un reto terapéutico, el tratamiento inicial se basa en poliquimioterapia y/o trasplante de células hematopoyéticas.
The aggressive epidermotropic CD8+ T-cell lymphoma or Berti's lymphoma, is a rare subtype of primary cutaneous lymphomas, first described in 1999. It is still considered a provisional entity by the latest World Health Organization classification of cutaneous lymphomas. Patients are commonly adults with a male predominance and it is characterized by widespread rapid evolving papules, hyperkeratotic annular plaques and ulcerated tumors. Histopathologicfindingsarecharacteristic,withaninfiltrate of medium-sized CD8+ atypical lymphocytes involving the epidermis with a pagetoid pattern. Ithasanaggressivebehaviorandtheprognosisispoor with a 5-yearsurvival less than 40%. Instead these disease represents a therapeutic challenge, the initial treatment consists on polychemotherapy and hematopoietic stem cell transplantation.
Subject(s)
Humans , Skin Neoplasms , Lymphoma, Primary Cutaneous Anaplastic Large Cell/diagnosis , Carcinoma, Squamous Cell , Drug Therapy, Combination , Lymphoma, Primary Cutaneous Anaplastic Large Cell/therapyABSTRACT
Mycosis fungoides (MF) is the most common group of cutaneous T-cell lymphomas. It is a rare non-Hodgkin’s lymphoma of mature, skin-homing, clonal, malignant T lymphocytes, usually observed in mid to late adulthood, that initially presents in the skin as patches, plaques, tumors, or generalized erythema (erythroderma) and can involve the lymph nodes and peripheral blood. In this review, we survey the MF literature of the last decade and highlight the major trends.
ABSTRACT
Cutaneous metastases of malignant melanoma are usually confined to the dermis or subcutaneous fat. In some instances, however, they may involve the epidermis. A 68-year-old woman with a malignant melanoma on the subungual area of the right great toe presented with multiple blackish pinhead-sized macules surrounding an ulcerative lesion on the right great toe. Histopathological study of the macules showed atypical melanocytes and melanocytic nests in the papillary dermis and the dermoepidermal junction. A thinning of the epidermis, widening of the dermal papillae by aggregated atypical melanocytes, epidermal collarette formation, and angiotropism were also seen. A diagnosis of epidermotropic metastatic malignant melanoma (EMMM) was made. EMMM is a specific form of metastatic malignant melanoma that is associated with epidermotropism of melanoma cells and several histopathological features. The differential diagnosis between primary malignant melanoma and EMMM can be difficult because of their similar clinical and histological features. Here, we report a case demonstrating EMMM.
Subject(s)
Aged , Female , Humans , Dermis , Diagnosis , Diagnosis, Differential , Epidermis , Melanocytes , Melanoma , Neoplasm Metastasis , Subcutaneous Fat , Toes , UlcerABSTRACT
Background: Mycosis fungoides (MF) is cutaneous lymphoma of the T-cell lineage. Hypopigmented MF is a clinical variant of MF, described mainly in Asians. This is a retrospective clinicopathologic analysis of hypopigmented MF at a tertiary care center. Aims: To describe the clinicopathologic profile of hypopigmented MF. Methods: Records of clinicopathologic notes over a 5-year period ranging from January 2005 up to December 2009 were reviewed over a period of 3 months, of which 15 cases were diagnosed with hypopigmented MF based on clinicopathologic correlation. Results: Hypopigmented MF was found to be more common in males, and between second and fourth decades of life. The latent period between onset and diagnosis was around 3.83 years. Most of the patients were asymptomatic 80% (12/15), with skin changes of subtle atrophy in 46.66% (7/15), scaling in 20% (3/15) and focal changes of poikiloderma in 26.66% (4/15) patients. Most common sites of distribution of the lesions were the trunk and extremities. Many of the cases had been clinically mistaken for Hansen's disease prior to correct diagnosis. Marked epidermotropism and tagging of epidermis by large lymphocytes characterizes the condition histopathologically. Of the 15 cases, immunohistochemistry was possible in 10 cases, of which 8 showed predominant CD8 positive epidermotropic infiltrates and two cases showed absence of CD8 positive and CD4 positive lymphocytic infiltrate in the epidermis. Conclusion: Hypopigmented MF presents as hypopigmented asymptomatic patches without any erythema or infiltration in its early stage and mimics Hansen's disease. Skin biopsy clinches the diagnosis.
ABSTRACT
Lymphomatoid keratosis (LK) is considered to be a rare variant of cutaneous lymphoid hyperplasia, with epidermotropism. We herein report a case of LK which developed on the abdomen of an elderly Korean woman. A 60-year-old woman presented with a 10-year history of a pruritic, solitary, brown to black plaque on the abdomen. Histopathologically, the specimen showed hyperkeratosis, parakeratosis, acanthosis and Pautrier's micro-abscess in the epidermis, and a lichenoid infiltration of lymphocytes in the dermis, which expressed both B cell and T cell lineage on the immune-histochemical staining. Based on these clinical and histopathological findings, our case was diagnosed as LK. To our knowledge, this is the first case report of LK in the Korean dermatologic literature.
Subject(s)
Aged , Female , Humans , Middle Aged , Abdomen , Cell Lineage , Dermis , Epidermis , Hyperplasia , Keratosis , Lymphocytes , ParakeratosisABSTRACT
BACKGROUND: Mycosis fungoides(MF) is a representative of cutaneous T-cell lymphoma and progresses through clinical stages, such as initial pre-mycotic(macule or patch), plaque, and tumor stage. Although lesions of plaque and tumor stage show typical features, those of pre-mycotic stage are commonly non-specific that precise diagnosis of early MF remains elusive. OBJECTIVES: This study attempted to elucidate the clinical and histopathologic features of early MF. METARIALS AND METHODS: We investigated 11 cases of MF stage Ia(based on TNM staging system) that visited the department of dermatology at the Seoul National University Hospital for the past decade year(1988-1998). Medical records and biopsy slides were reviewed. RESULTS: The results were as follows : 1. Clinical characteristics: Lesions were relatively well-defined erythematous scaly patches or plaques on lower abdomen, buttocks, proximal extremities or whole body. Clinically initial diagnoses were large plaque parapsoriasis in nine cases and in the remainders, poikiloderma atrophicans vasculare and MF, respectively. Duration of disease ranged from one month to 41 years, averaging 15.2 years. 2. Treatments: Follow-up of 8 patients was continued and all showed partial responses to application of topical steroids or BCNU(carmustine) and PUVA therapy. 3. Histopathologic findings: Epidermotropism was found in all cases and can be the most important pathologic finding, but typical Pautriers microabscesses were found in a few cases. Mild spongiosis was seen in epidermis and superficial perivascular infiltration was the most common finding in dermis. Lichenoid infiltration with epidermal psoriasiform change was found in two cases. Atypism of infiltrating cells was found in all cases and more prominent in epidermis. CONCLUSION: Lesions of early mycosis fungoides were relatively non-specific erythematous patches/plaques but characteristically, patients had relatively long duration of stationary disease status and showed good responses to treatments. Histopathologically, variable degree of epidermotropism was the most constant finding.