Sézary syndrome in an 11-year-old girl

Meister, Lynn; Duarte, Ana M.; Davis, Joanna; Pérez, Jorge Luis Estepa; Schachner, Lawrence A.

doi:10.1016/0190-9622(93)70017-n

Cited by 37 publications

(17 citation statements)

References 8 publications

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“…This estimate might be too low, because physicians often hesitate or delay performing skin biopsies in children [11]. Early primary of disseminated lesions might be mistaken for atopic dermatitis or insect bite reactions, resulting in delayed diagnosis [8, 9]. In our case, the patient was initially misdiagnosed as having psoriasis.…”

Section: Discussionmentioning

confidence: 78%

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Mycosis fungoides with Mucinosis follicularis in Childhood

et al. 1999

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Mycosis fungoides is a cutaneous T-cell lymphoma (CTCL) usually observed in mid to late adulthood. It occurs only rarely during childhood. Follicular mucinosis is a chronic dermatosis involving the sebaceous glands and outer root sheaths. It is normally differentiated into a juvenile benign form and an adult form possibly associated with mycosis fungoides. We report a 12-year-old boy who presented with an 8-month history of erythematous mucinous plaques on the scalp. Three months later, he developed erythematous patches and plaques on his whole body, accompanied by cervical lymphadenopathy. A biopsy showed follicular mucinosis and epidermotropism of the lymphocytic infiltrate. Immunophenotyping and a PCR clonality test were consistent with CTCL. The patient received PUVA treatment and local steroids, resulting in partial remission. Mycosis fungoides should be considered in the differential diagnosis of chronic, scaling dermatoses in childhood. Moreover, follicular mucinosis in childhood can be associated with mycosis fungoides.

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Section: Discussionmentioning

confidence: 78%

“…Although CTCL is primarily a disease of adulthood and occurs with the highest incidence in middle-aged men, its onset in childhood, adolescence and early adulthood with a preponderance in males has been reported [3, 4, 5, 6, 7, 8, 9]. Up to 6% of all mycosis fungoides cases [6, 10]are estimated to occur in childhood.…”

Section: Discussionmentioning

confidence: 99%

Mycosis fungoides with Mucinosis follicularis in Childhood

et al. 1999

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“…SS cases reported in the literature [26, 27]with histological diagnosis before 20 years of age were not included in this study. The parameters assessed were: gender, race, symptom duration before diagnosis, type of cutaneous involvement at diagnosis, TNM staging at diagnosis, clinical course, survival, immunophenotype of skin-infiltrating lymphocytes.…”

Section: Methodsmentioning

confidence: 99%

Mycosis fungoides in Patients under 20 Years of Age: Report of 7 Cases, Review of the Literature and Study of the Clinical Course

et al. 1999

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Background: Mycosis fungoides (MF) is rare in young patients. Its clinical behavior is still uncertain, as some reports have suggested that it has a more aggressive course than does the adult-onset type. Aim: To ascertain if early-onset MF represents a heterogeneous group of cutaneous T cell lymphomas. Materials and Methods: Clinical, immunohistopathological and follow-up data of early-onset (<20 years of age) MF cases reported in the literature (n = 42) plus 7 described herein were compared with those of a cohort of adult-onset MF patients (n = 252) diagnosed at our institution since 1975. Results: The majority of the 49 early-onset MF patients had patch-plaque stage disease at diagnosis. Ten had hypopigmented lesions. The predominant phenotype was CD3+ CD4+CD7–CD8–. Seven patients had a stage progression, 6 with extracutaneous involvement. Five- and 10-year survival rates were 93 and 74%, respectively. Conclusions: No statistically significant differences were found in the disease course between early- and adult-onset MF.

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“…This rate was significantly higher than the expected prevalence of atopy 7 . A relationship between Sezary syndrome and atopic diathesis was suggested in various case reports 8–10 . In a case report on a tumor stage mycosis fungoides, the patient had adult onset asthma and atopic‐like dermatitis in conjunction with increased IgE 11 .…”

Section: Introductionmentioning

confidence: 99%