Atopic Dermatitis-like Pre-Sézary Syndrome: Role of Immuno-sup-pression.
Summary of "Atopic Dermatitis-like Pre-Sézary Syndrome: Role of Immuno-sup-pression."
We describe here 4 patients with Sézary syndrome masquerading as adult-onset atopic dermatitis. The patients presented with a clinical picture compatible with wide-spread atopic dermatitis and did not fulfil the criteria for Sézary syndrome (lack of lymphoadenopathy and blood involvement, skin histology without presence of atypical cells). In our patients, overt Sézary syndrome developed after immunosuppressive treatment (including cyclosporine). These cases support the validity of the concept of pre-Sézary syndrome, which is a long-lasting, pre-malignant condition, and which may develop to true malignancy in a state of immunosuppression.
Affiliation
Department of Dermatology, Venereology and Allergology, Medical University of Gdańsk, 80-210 Gdańsk, Poland. E-mail: mwojd@gumed.edu.pl.
Journal Details
This article was published in the following journal.
Name: Acta dermato-venereologica
ISSN: 0001-5555
Pages:
Links
- PubMed Source: http://www.ncbi.nlm.nih.gov/pubmed/21681350
- DOI: http://dx.doi.org/10.2340/00015555-1149
Medical and Biotech [MESH] Definitions
Kaposi Varicelliform Eruption
A disseminated vesicular-pustular eruption caused by the herpes simplex virus (HERPESVIRUS HOMINIS), the VACCINIA VIRUS, or Varicella zoster (HERPESVIRUS 3, HUMAN). It is usually superimposed on a preexisting, inactive or active, atopic dermatitis (DERMATITIS, ATOPIC).
Dermatitis, Exfoliative
The widespread involvement of the skin by a scaly, erythematous dermatitis occurring either as a secondary or reactive process to an underlying cutaneous disorder (e.g., atopic dermatitis, psoriasis, etc.), or as a primary or idiopathic disease. It is often associated with the loss of hair and nails, hyperkeratosis of the palms and soles, and pruritus. (From Dorland, 27th ed)
Antigens, Dermatophagoides
Antigens from the house dust mites (DERMATOPHAGOIDES), mainly D. farinae and D. pteronyssinus. They are proteins, found in mite feces or mite extracts, that can cause ASTHMA and other allergic diseases such as perennial rhinitis (RHINITIS, ALLERGIC, PERENNIAL) and atopic dermatitis (DERMATITIS, ATOPIC). More than 11 groups of Dermatophagoides ALLERGENS have been defined. Group I allergens, such as Der f I and Der p I from the above two species, are among the strongest mite immunogens in humans.
Netherton Syndrome
Rare autosomal recessive disease with variable expressions. Clinical features of the disease include variable ICHTHYOSIFORM ERYTHRODERMA, CONGENITAL; bamboo hair (trichorrhexis invaginata); and ATOPIC DERMATITIS. The disease is caused by mutations in the SPINK5 gene.
Dermatitis, Atopic
A chronic inflammatory genetically determined disease of the skin marked by increased ability to form reagin (IgE), with increased susceptibility to allergic rhinitis and asthma, and hereditary disposition to a lowered threshold for pruritus. It is manifested by lichenification, excoriation, and crusting, mainly on the flexural surfaces of the elbow and knee. In infants it is known as infantile eczema.
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