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[Actinic keratosis of the conjunctiva. Apropos of a clinical case].

We report a slowly progressive and whitish limbal conjunctival tumor that occurred in a 73-year-old man. An excisional biopsy specimen of this conjunctival tumor was submitted for histopathologic evaluation. The diagnosis of conjunctival actinic keratosis was based on following cellular abnormalities: epithelial hyperplasia acanthosis, keratosis or parakeratosis with discrete papillomatosis and some atypia. The basement membrane was intact. An area of elastotic degeneration in the subtantia propria, was considered as one of the characteristic features of this conjunctival precancerous condition related to excessive sun exposure. By hybridization in situ, the detection of human papilloma virus (HPV) was negative. Keratosis actinic needs to be distinguished from other precancerous conditions showing similar clinical features such as dysplasia and carcinoma in situ appearing to affect the prognosis.

Aged↗

Keratosis follicularis spinulosa decalvans. Report of two cases and literature review.

We report herein two cases of keratosis follicularis spinulosa decalvans (KFSD) and review the literature on this condition. The entity is one of a group of related disorders that shows keratosis pilaris with inflammation followed by atrophy. The clinical features and course of KFSD are characteristic. During infancy, keratosis pilaris begins on the face and, by childhood, progresses to involve the trunk and extremities. Sometime during childhood or up to the early teenage years, a cicatricial alopecia of the scalp and eyebrows develops and is the hallmark of this disorder. Hyperkeratosis of the palms and soles is a frequently associated finding and is usually manifested during adolescence. Other features occurring with this syndrome include atopy, photophobia, and corneal abnormalities. Sex-linked inheritance has been proposed by several authors.

Adult↗

Keratosis punctata and atopy. Report of 31 cases with a prospective study of prevalence.

We report 31 cases of keratosis punctata palmaris et plantaris in blacks. Eleven of these were found in a prospective study of 573 consecutive black private dermatologic outpatients, a prevalence of 1.9%, and 20 cases were randomly encountered over a three-year period in the same office. Five of the 30 patients able to give a history were symptomatic, and they and ten others came to the office because of this disease, where as 15 came for other reasons, two of whom were unaware of the disease. Four of the 31 patients also had foot lesions. Four patients were aware of relatives with palmar keratoses. The mean duration of disease was 4.3 years, the median was three years, and the range was zero to 38 years. The mean age at onset was 30.3 years, the median was 29 years, and the range was 12 to 70 years, with one case beginning in infancy. Each patient in the prospective study was examined for personal atopy or for a family history of atopy. Nine (82%) of the 11 patients with keratosis punctata had atopy and/or a family history of atopy. Statistical analysis confirmed the association between keratosis punctata and atopy in the prospective study.

Adolescent↗

Inverted follicular keratosis.

We reviewed 17 cases of inverted follicular keratosis. The median age of the patients at the time of surgery was 69 years. Follow-up in 14 cases showed no recurrences of inverted follicular keratosis, which is a benign skin lesion, often mistaken clinically and pathologically for a malignancy. Inverted follicular keratosis is characterized histologically by the presence of squamous eddies, acantholysis, acanthosis, and hyperkeratosis.

Aged↗

Papillated squamous cell carcinoma in situ arising in a seborrheic keratosis.

A 73-year-old man had developed a 27 x 60-mm seborrheic keratosis on his trunk. Some months previously, an 18 x 19-mm cauliflower-like area had developed within this seborrheic keratosis. Histologic examination showed a papillated squamous cell carcinoma in situ arising in a seborrheic keratosis. Seborrheic keratoses are the most common cutaneous neoplasms in man. Malignant changes are most unusual but should be suspected in lesions of unusual clinical appearance.

Aged↗

Alteration in stereological density parameters of the mucosal vascular bed during neoplastic transformation in sublingual keratosis.

Stereological techniques have been used to study the vascularity in 110 sublingual keratosis lesions and 22 specimens of normal sublingual mucosa using an image analysis system "IBAS 1". The stereological parameters volume density (Vv), and surface density (Sv) were significantly increased in sublingual keratosis lesions as compared with those of normal sublingual mucosa. Lesions that eventually underwent malignant change showed the highest values and were highly significant (p less than .001) when compared with those which did not do so. The present results demonstrate that vascularity could be a supportive marker of impending malignant change in sublingual keratosis lesions.

Blood Vessels↗

Keratosis follicularis spinulosa decalvans and acne keloidalis nuchae.

A 27-year-old man presented with a 10-year history of scarring alopecia on the vertex of the scalp associated with follicular crusting and pustule formation, and a papular eruption on the posterior neck. Additionally, there was keratosis pilaris on the cheeks, eyebrows and thighs. Histology from the vertex showed scarring with a mixed perifollicular inflammatory infiltrate and foci of acute suppurative folliculitis. With clinical correlation, the diagnosis of keratosis follicularis spinulosa decalvans and concurrent acne keloidalis nuchae was made. The association of keratosis follicularis spinulosa decalvans with acne keloidalis nuchae has not previously been described. The patient responded to treatment with oral isotretinoin 20 mg (0.25 mg/kg) daily for 12 months.

Acne Keloid↗

Arsenic-related Bowen's disease, palmar keratosis, and skin cancer.

Chronic arsenical intoxication can still be found in environmental and industrial settings. Symptoms of chronic arsenic intoxication include general pigmentation or focal "raindrop" pigmentation of the skin and the appearance of hyperkeratosis of the palms of the hands and soles of the feet. In addition to arsenic-related skin diseases including keratosis, Bowen's disease, basal-cell-carcinoma, and squamous-cell carcinoma, there is also an increased risk of some internal malignancies. Arsenic-related diseases are common in areas of the world where the drinking water has a high arsenic content. In this paper, we describe a 35-year-old male patient who had arsenic-related keratosis, squamous-cell carcinoma in the palmar area of his left hand, and Bowen's disease on his left thigh. The patient worked in a borax mine for 15 years, so he was exposed to arsenic in drinking water, airborne arsenic in his workplace, and had direct contact. The patient was treated for 11 months for arsenic-related keratosis until an axillary lymph node metastasis occurred; the lesion was excised and diagnosed to be malignant. Bowen's disease was detected when the patient was being treated for cancer. No other malignancy was found. The patient is still receiving regular follow-up care.

Adult↗

Keratosis punctata of the instep.

We present a case of keratosis punctata involving the instep of both feet in addition to palmar and plantar creases, a finding not previously reported to our knowledge. We also discuss a closely related entity, keratosis punctata palmaris et plantaris (KPPP), and why we believe our case does not simply represent a variant of keratosis punctata of the palmar creases (KPPC).

Adult↗

[Keratosis follicularis spinulosa decalvans (Siemens' syndrome) associated with other abnormalities].

Keratosis follicularis spinulosa decalvans (ichthyosis follicularis or Siemens's syndrome) is considered a general form of keratosis pilaris decalvans. Localized types are keratosis pilaris atrophicans and atrophoderma vermicularis. A case of this unusual process is presented. Clinical, histological and scanning electron microscopic studies of the hair were performed. Clinically, a generalized hypotrichosis with hyperkeratotic follicular plugs is observed; especially in the scalp and the eyebrows. Other interesting clinical findings were cutis hyperelastica, gingival hypertrophy, mongoloid palpebral fissures, big pinnae and clinodactyly of the 5th finger. From the histological point of view we observed follicular plugging, dystrophic pilosebaceous follicles, absence of sebaceous glands, perifollicular fibrosis and minimal lymphomonocytic infiltrate. Scanning electronmicroscopy shows a brittle hair with cuticular abnormalities. Siemens's syndrome can be considered a specific pilosebaceous dysplasia because the absence or hypoplasia of sebaceous glands; which produces follicular hyperkeratosis and pilar atrophy with perifollicular fibrosis and alopecia.

Abnormalities, Multiple↗

Keratosis lichenoides chronica. Successful treatment with psoralen-ultraviolet-A therapy.

A man with keratosis lichenoides chronica is described who, unlike previously described patients, had had his eruption since infancy. Moreover, this patient had prominent oral involvement resembling lichen planus. Typically, keratosis lichenoides chronica is recalcitrant to all forms of treatment, but this patient responded substantially to psoralen-ultraviolet-A therapy. Several weeks after treatment was completed, he showed no signs of relapse.

Adult↗

The pathologic features of keratosis obturans and cholesteatoma of the external auditory canal.

The presence of a keratin plug occluding the deep external auditory canal was first noted and documented in the 19th century. It has subsequently been proposed that two different diseases can be responsible for the presence of this type of obstruction within the deep meatus: keratosis obturans and external auditory canal cholesteatoma. Keratosis obturans is characterized by a dense plug of keratin debris located primarily within the deep meatus. There is an associated hyperplasia of the underlying epithelium and evidence of chronic inflammation within the subepithelial tissue. There is no evidence of erosion or necrosis of the underlying bone. In external auditory canal cholesteatoma the significant finding is extensive erosion of the bony external auditory canal by a wide-mouthed sac, lined with stratified squamous keratinizing epithelium, that arises lateral to the tympanic membrane and is located in the inferior portion of the bony external canal. There is frequently evidence of sequestration of the underlying bone.

Aged↗

DNA repair synthesis in fibroblast strains from patients with actinic keratosis, squamous cell carcinoma, basal cell carcinoma, or malignant melanoma after treatment with ultraviolet light, N-acetoxy-2-acetyl-aminofluorene, methyl methanesulfonate, and N-methyl-N-nitrosourea.

Fibroblast strains derived from skin biopsies of patients with actinic keratosis (6), malignant melanoma (18), squamous cell carcinoma (11), and basal cell carcinoma (12) were investigated for DNA repair synthesis, with 16 fibroblast strains for normal donors as controls. Cells were exposed to UV light, the "UV-like" carcinogen (Ac)2ONFln, and the methylating carcinogens MeSO2OMe and MeNOUr. Dose-response experiments, which included 10 dose levels, were performed, the data analyzed by linear regression, and the slope of the regression line (term: G0) used as a measure of DNA repair synthesis. The mean experimental variability of G0 of individual fibroblast strains was 9.5%-15.4%, depending upon exposure. For comparison of all cell strains belonging to the same skin malignancy group with those of the control group, G0 values of the individual strains were combined to yield group-specific weighted mean G0 values. In addition, the capacity to incise UV-damaged DNA was measured in 24 cell strains from patients with skin tumors using the alkaline elution technique. For quantitating DNA-incising capacity, the initial velocities of the elution curves were plotted versus the UV dose, and the slope of the resulting regression line was used to obtain the characteristic value E0. The mean experimental variability of E0 of individual strains was +/- 22%. These E0 values were combined to yield weighted mean values of groups. The fibroblast strains in the groups of patients with actinic keratosis and malignant melanoma were found to have normal mean G0 values when DNA repair synthesis was challenged with UV light or one of the three carcinogens. However, the squamous cell carcinoma group exhibited significantly lower mean G0 values after treatment with UV light (82% that of normal donors), (Ac)2ONFln (70%), MeSO2OMe (70%), and MeNOUr (69%). The basal cell carcinoma group showed significantly diminished repair synthesis upon treatment with UV light (81% that of normal donors) and MeSO2OMe (67%). In contrast to these findings, in no skin malignancy group was post UV DNA-incising capacity (E0) significantly diminished, although it should be noted that group sizes were only half as large as for G0 determinations. These data may be interpreted as indicating that DNA excision repair is impaired in fibroblast strains from patients with squamous cell carcinoma and-to a lesser extent-basal cell carcinoma.(ABSTRACT TRUNCATED AT 400 WORDS)

Acetoxyacetylaminofluorene↗

Characterization of a new type of human papillomavirus (HPV) related to HPV5 from a case of actinic keratosis.

Human papillomavirus (HPV) DNA sequences, related to the genomes of HPVs associated with epidermodysplasia verruciformis (EV), were detected in DNA samples extracted from biopsied lesions in 2 of 24 cases of actinic keratosis found in the general population. An HPV DNA was molecularly cloned from one of these samples. Blot hybridization experiments, performed under stringent conditions, revealed a significant cross-hybridization only between this HPV DNA and the DNAs of HPV5 and of the HPV5-related types. The extent of homology between them ranged from 7 to 30%, as evaluated by hybridization in liquid phase at saturation followed by nuclease S1 analysis. This showed that the cloned HPV represented a new type, tentatively named HPV36. HPV36 was not found in the other 22 cases of actinic keratosis, but was detected in scrapings of benign lesions of 7 of 18 (39%) EV patients.

Aged↗

Focal palmoplantar and gingival keratosis: a distinct palmoplantar ectodermal dysplasia with epidermolytic alterations but lack of mutations in known keratins.

Focal palmoplantar and gingival keratosis is a rare autosomal dominant disease whose clinical features, and in particular, pathologic alterations and molecular etiology remain to be well defined. Recently we observed a German family affected by the disease in at least 3 consecutive generations. The 4 patients examined showed circumscribed and painful hyperkeratosis at the weight-bearing plantar skin since infancy, rather mild palmar hyperkeratosis, and continuous leukokeratosis confined to the maxillary and mandibulary attached gingiva. There were no nail changes, subungeal keratoses, or follicular hyperkeratosis. Light and electron microscopy of the plantar and gingival lesions revealed alterations of epidermolytic hyperkeratosis. Mutations in the known keratin genes were excluded by linkage analysis using microsatellite markers. We conclude that focal palmoplantar and gingival keratosis is a clinically distinct palmoplantar ectodermal dysplasia that is pathologically characterized by epidermolytic alterations, but is most probably not caused by a mutation in a keratin gene.

Adult↗

Lichen planus-like keratosis. A clinicohistopathologic correlation.

We feel that it is possible to make a reasonably consistent clinical diagnosis of lichen planus-like keratosis on the basis of several criteria. These criteria include physical appearance, location and short duration of the lesion, clinical symptoms such as pruritus and pain, age and sex of the patient, and the frequent presence of a preceding lentigo. Clinical data on fifty-nine histologically confirmed cases are presented. Some interesting features of this curious condition are discussed. Because of the clinical similarity of lichen planus-like keratosis to other sometimes more ominous lesions, microscopic confirmation of the diagnosis is still recommended.

Adult↗

Spreading pigmented actinic keratosis.

We present four cases of spreading pigmented actinic keratoses, an only recently described pigmented lesion of sun-exposed areas, in which the histologic appearance is that of actinic keratosis with the additional feature of excessive melanin deposition in the lower epidermis and in the upper dermis. Clinically, it is a brown patch or plaque with a smooth surface, usually larger than 1 cm, that tends to spread centrifugally. Clinical differential diagnoses include seborrheic keratosis, melanocytic nevus, senile lentigo, lentigo maligna, and lentigo maligna melanoma. This pigmented lesion is probably much more common than the existing literature would indicate.

Aged↗

A kindred with alopecia, keratosis, pilaris, cataracts, and psoriasis.

Three members of a family with numerous ectodermal abnormalities are described. These anomalies primarily include patchy alopecia beginning in childhood, premature cataracts, widespread keratosis pilaris, and psoriasis. The alopecia and premature cataracts appear to follow an autosomal dominant inheritance pattern with incomplete penetrance and appear to be linked. Psoriasis also occurs in several members of this family and probably represents a separate but possibly related genodermatosis. This kindred has features of both keratosis follicularis spinulosa decalvans and ichthyosis follicularis, and the disorder seems to fit into the group of follicular hyperkeratosis disorders.

Adult↗