dyskeratosis


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dyskeratosis

 [dis″ker-ah-to´sis]
abnormal, premature, or imperfect development of the keratinocytes. adj., adj dyskeratot´ic.

dys·ker·a·to·sis

(dis'ker-ă-tō'sis),
1. Premature keratinization in individual epithelial cells that have not reached the keratinizing surface layer; dyskeratotic cells generally become rounded and they may break away from adjacent cells and fall off.
2. Epidermalization of the conjunctival and corneal epithelium.
3. A disorder of keratinization.
[dys- + G. keras, horn, + -osis, condition]

dys·ker·a·to·sis

(dis-ker'ă-tō'sis)
1. Premature keratinization in individual epithelial cells that have not reached the keratinizing surface layer; dyskeratotic cells generally become rounded, and they may break away from adjacent cells and fall off.
2. Epidermalization of the conjunctival and corneal epithelium.
3. A disorder of keratinization.
[dys- + G. keras, horn, + -osis, condition]

dyskeratosis

Abnormal, excessive or imperfect KERATINIZATION of cells of the EPIDERMIS. DYSPLASIA of a squamous epithelium.

dyskeratosis

Abnormal process which, in the eye, results in hornification of the epithelial layer of the conjunctiva or cornea. It may be hereditary or due to irritation (e.g. radiation) or to prolonged drug administration in the eye. It appears as a dry white plaque (called leucoplakia or leucokeratosis). It may be benign or malignant, in which case it must be surgically excised. See pterygium.

dys·ker·a·to·sis

(dis-ker'ă-tō'sis)
Premature keratinization in individual epithelial cells that have not reached the keratinizing surface layer.
[dys- + G. keras, horn, + -osis, condition]
References in periodicals archive ?
Germline mutations in the human TERT and TERC cause autosomal dominant dyskeratosis congenita, a rare hereditary disorder associated with premature death from aplastic anemia and pulmonary fibrosis.[18] TERT variants are the most frequently identified rare mutations related to pulmonary fibrosis, which were found in up to 15% of familial interstitial pneumonitis and in 1-3% sporadic cases.[8] This Ala716Thr variant of TERT was firstly reported in children with severe aplastic anemia and a family history of lung fibrosis.[19],[20] Thus, it was considered that within a single family carried this mutation, older generations were more likely to affected by adult-onset pulmonary fibrosis, whereas bone marrow failure was the first presentation in subsequent generations at a younger age.
Out of the 4 patients who presented with hyperpigmentation of skin, 3 had megaloblastic anaemia and one had dyskeratosis congenita.
Vedi, "Survival after hematopoietic stem cell transplant in patients with dyskeratosis congenita: systematic review of the literature," Biology of Blood and Marrow Transplantation, vol.
Also, the neoplastic cells presented prominent intercellular bridges with variable degree of keratinization, either individual cell dyskeratosis or in clusters (Figure 3 inset).
Histopathologically, PNP is most often seen as suprabasal acantholysis, dyskeratosis, and cellular autoimmune phenomena: vacuolar interface change and lichenoid infiltrates.
Gemahsis' devastated mother Tamar Srondy has told how her young boy was diagnosed with a disease called dyskeratosis congenita - rare and progressive bone marrow failure syndrome.
Pulmonary cutaneous syndromes, such as sarcoidosis, Hermansky-Pudlak syndrome, tuberous sclerosis, neurofibromatosis, dyskeratosis congenita, scleroderma, systemic lupus erythematosus, and dermatomyositis, are reported to be associated with interstitial lung involvement (4).
(3) reported a case of Graphium basitruncatum fungemia in a two-year-old child with dyskeratosis congenita who underwent stem cell transplantation two months prior to infection.
Dyskeratosis congenita or Zinsser-Cole-Engman syn-dromeis is a rare inherited bone marrow failure syndrome (IBMFS) characterized by diagnostic triad of reticulated skin hyperpigmentation, nail dystrophy, and oromucosal leukoplakia (1).
Other general features include: Asteatosis (absence of sebaceous glands), normal general mental development, dry skin, nail dystrophy, palmoplantar dyskeratosis, decreased sebaceous and nasal secretions, lack of dermal ridges, and fragile-appearing skin.
A telomerase component is defective in the human disease dyskeratosis congenita.
An extensive variety of cutaneous conditions bearing a resemblance to CARP were considered in the differential diagnosis, including acanthosis nigricans (AN), tinea versicolor, Darier disease, terra firma-forme dermatosis, prurigo pigmentosa, flagellate dermatosis, and dyskeratosis congenita.