Hydantoin-Induced Cutaneous Pseudolymphoma With Clinical, Pathologic, and Immunologic Aspects of Sézary Syndrome
71
Citation
23
Reference
10
Related Paper
Citation Trend
Abstract:
• Background.—
The phenytoin-induced hypersensitivity syndrome is characterized by the development of fever, rash, lymphadenopathy, and hepatitis associated with leukocytosis and eosinophilia. This article describes the unusual occurrence of a pseudo-Sézary syndrome in the days following the introduction of phenytoin treatment.Observation.—
A phenytoin-induced erythroderma developed in a 60-year-old woman the histologic, cytologic, and immunologic characteristics of an erythrodermal cutaneous T-cell lymphoma of the Sézary syndrome type with lymph node involvement. The dramatic improvement after withdrawal of drug therapy and the absence of recurrence 5 years after led us to consider it as a hydantoin-induced pseudolymphoma.Conclusions.—
Although lymph node pseudolymphomas induced by phenytoin are well known, few cases of hydantoin-induced mycosis fungoides have been reported in the literature. We present herein the first case of a Sézary-like syndrome associated with phenytoin therapy. Such a patient must be monitored regularly because of the risk of a true malignant lymphoma developing even many years later. (Arch Dermatol.1992;128:1371-1374)Keywords:
Pseudolymphoma
Erythroderma
Hydantoin
Toxic Epidermal Necrolysis
Toxic Epidermal Necrolysis
Procalcitonin
Bacteremia
Body surface area
Cite
Citations (0)
Mycosis fungoides is a low-grade lymphoproliferative disorder caused by CD4+ lymphocytes. It is the most common type of cutaneous T-cell lymphoma. Typically, neoplastic T cells localize to the skin and produce patches, plaques, tumors, or erythroderma. Diagnosis of early mycosis fungoides can be difficult due to the nonspecific nature of cutaneous and histologic findings. However, recent advances in the application of histologic criteria, coupled with molecular biology tools such as immunophenotyping and polymerase chain reaction, have improved diagnostic accuracy. Independent prognostic factors include the extent and nature of skin involvement, the presence of extracutaneous disease, blood involvement, age > or = 60 years, and lactate dehydrogenase elevation. Accordingly, patients with limited patches and/or plaques (stage IA or IIA) experience long-term survival comparable to that of matched controls. The median survival is 11 years for patients with extensive patch/plaque (stage IB or IIA), 3.2 years for those with cutaneous tumors (stage IIB), 4.6 years for those with erythroderma (stage III), 1.2 years for those with pathologic nodal involvement (stage IVA), and 0.9 years for those with visceral disease (stage IVB). Over time, mycosis fungoides may progress to Sézary syndrome or transform to large-cell histology.
Erythroderma
Cutaneous T-cell lymphoma
Immunophenotyping
Peripheral T-cell lymphoma
Cutaneous lymphoma
Cite
Citations (13)
Erythroderma
Cutaneous T-cell lymphoma
Cite
Citations (13)
Toxic Epidermal Necrolysis
Body surface area
Mucous membrane
Cite
Citations (18)
A patient developed lymphadenopathy while receiving diphenylhydantoin therapy that histologically resembled malignant lymphoma. Adenopathy regressed when diphenylhydantoin was discontinued but subsequently recurred as a fatal, malignant lymphoma. Previous reports of hydantoin-induced lymphadenopathy were reviewed and classified. It was found that no consistent clinical or histologic criteria existed to differentiate benign from malignant lymphoid reactions.
Pseudolymphoma
Hydantoin
Malignant lymphoma
Cite
Citations (128)
Abstract Toxic epidermal necrolysis is a rare, life-threatening, drug-induced disease that was first described by a Scottish dermatologist named Dr. Alan Lyell in 1958. After nearly 60 years, the mortality rate remains at approximately 30%. Stevens–Johnson syndrome and toxic epidermal necrolysis are differentiated by the percentage of skin involvement, with greater than 30% being classified as toxic epidermal necrolysis. Patients with Stevens–Johnson syndrome and toxic epidermal necrolysis should be managed in a burn or intensive care unit. Prompt identification is crucial to the effective management and treatment of the disease. This article presents a case study of a 10-year-old boy diagnosed with toxic epidermal necrolysis with 94% skin involvement who was effectively treated and discharged without major complications or infections.
Toxic Epidermal Necrolysis
Erythroderma
Cite
Citations (1)
Toxic epidermal necrolysis is an adverse immunological skin reaction secondary in most cases to the administration of a drug. Toxic epidermal necrolysis, Stevens-Johnson syndrome, and multiform exudative erythema are part of the same disease spectrum. The mortality rate from toxic epidermal necrolysis is approximately 30%. The pathophysiology of toxic epidermal necrolysis is similar in many respects to that of superficial skin burns. Mucosal involvement of the ocular and genital epithelium is associated with serious sequelae if the condition is not treated early. It is generally accepted that patients with toxic epidermal necrolysis are better treated in burn units, which are experienced in the management of patients with extensive skin loss. Treatment includes support, elimination, and coverage with biosynthetic derivatives of the skin in affected areas, treatment of mucosal involvement, and specific immunosuppressive treatment. Of the treatments tested, only immunoglobulin G and cyclosporin A are currently used in most centers, even though there is no solid evidence to recommend any specific treatment. The particular aspects of the treatment of this disease include the prevention of sequelae related to the formation of synechiae, eye care to prevent serious sequelae that can lead to blindness, and specific immunosuppressive treatment. Better knowledge of the management principles of toxic epidermal necrolysis will lead to better disease management, higher survival rates, and lower prevalence of sequelae.La necrolisis epidérmica tóxica es una reacción cutánea adversa de tipo inmunológico secundaria en la mayor parte de los casos a la administración de un fármaco. La necrolisis epidérmica tóxica, el síndrome de Steven Johnson y el eritema exudativo multiforme forman parte del mismo espectro de enfermedad. La mortalidad de la necrolisis epidérmica tóxica es alrededor del 30%. La fisiopatología de la necrolisis epidérmica tóxica es semejante en muchos aspectos a la de las quemaduras dérmicas superficiales. La afectación mucosa del epitelio ocular y genital se asocia con secuelas graves si no se trata de forma temprana. Se acepta en general que los pacientes con necrolisis epidérmica tóxica son tratados mejor en unidades de grandes quemados, donde existe experiencia en el manejo de enfermos con pérdida cutánea extensa. El tratamiento es de soporte, eliminación y cobertura con derivados biosintéticos de la piel de las zonas afectadas, tratamiento de la afectación mucosa, y tratamiento inmunosupresor específico. De los tratamientos ensayados sólo se usa actualmente en la mayor parte de los centros la inmunoglobulina G y la ciclosporina A, aun cuando no existe evidencia sólida para recomendar ningún tratamiento específico. Entre los aspectos particulares del tratamiento de esta enfermedad se encuentra la prevención de secuelas relacionadas con la formación de sinequias, los cuidados oculares para prevenir secuelas graves que pueden conducir a la ceguera, y el tratamiento específico inmunosupresor. Un mejor conocimiento de los principios del manejo de la necrolisis epidérmica tóxica llevará a un mejor manejo de la enfermedad, a una mayor supervivencia y una menor prevalencia de las secuelas.
Toxic Epidermal Necrolysis
Erythroderma
Erythema multiforme
Cite
Citations (26)
Toxic Epidermal Necrolysis
Body surface area
Drug reaction
Adverse drug reaction
Erythroderma
Cite
Citations (3)
Mycosis fungoides (MF) and Sézary syndrome (SS) are two subgroups of cutaneous T-cell lymphomas (CTCL), which belong to the extranodal non-Hodgkin lymphomas. These are rare diseases whose etiology is still not fully understood. Regarding pathogenesis, mycosis fungoides and Sézary syndrome derive from different T-helper cell types and are therefore considered two separate entities according to the current concept. Mycosis fungoides is clinically characterized by patch, plaque and tumor stages, although the disease can also manifest as erythroderma. Sézary syndrome is characterized by the presence of erythroderma, defined as redness of the skin covering >80% of the total body surface area. In the blood count of patients with mycosis fungoides, a T-lymphocytosis, especially with CD4-positive cells, can only be observed in advanced stage IV. One of the typical histological findings is an epidermotropic infiltrate of atypical, CD4-positive T-cells. Characteristic features of Sézary syndrome include atypical T lymphocytes (Sézary cells) in the blood and skin infiltrate. The diagnosis of both mycosis fungoides and Sézary syndrome is based on clinical, histological and hematological examinations as well as imaging techniques. Staging is based on the tumor-node-metastasis-blood (TNMB) classification and is a crucial factor in determining the prognosis. The treatment of mycosis fungoides and Sézary syndrome is carried out according to the stage, whereby local therapies such as UVB phototherapy are used as well as systemic forms of treatment, and in addition, in recent years, targeted therapies have increasingly become part of everyday clinical practice.
Erythroderma
Cutaneous T-cell lymphoma
Cite
Citations (4)