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Results for “KERATOSIS FOLLICULARIS”

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314 records · Page 18Linked to original sources

PubMed1991

A case of erythromelanosis follicularis faciei with a unique distribution.

A 16-year-old Japanese boy with erythromelanosis follicularis faciei (EFF) with an unusual distribution is described. The clinical features of classic EFF consist of reddish-brown discoloration of the skin with fine follicular hyperkeratosis in the preauricular areas, and occasionally on the temples and the lateral aspects of the neck. The present case is unique in that similar lesions also occurred on the auricles and eyebrows. Both light and electron microscopic findings of the lesions were consistent with the diagnosis of EFF.

Adolescent↗

PubMed2006

Glabellar pomade crust mimicking dyskeratosis follicularis.

A 79-year-old female patient presented with a slowly developing crusting, itching verrucous lesion of the forehead of 3 months' duration. She had no personal or family history of skin disease. On examination she presented with a hyperpigmented plaque of the glabellar region that resembled dyskeratosis follicularis Darier. A detailed medical history was taken and a skin biopsy was performed. Epidermal acanthosis and enlargement of follicle ostia with foreign material were found. The patient reported use of an ointment twice daily. She had a single cosmetic treatment where powder of unspecified composition had been used. The diagnosis of pomade crust was confirmed. Follicular material was enucleated mechanically and the area was treated with a metronidazole cream. The lesions completely disappeared.

Aged↗

PubMed2000

[Hyperkeratosis follicularis et parafollicularis in cutem penetrans (Kyrle's disease). Description of a case].

A case of hyperkeratosis follicularis et parafollicularis in cutem penetrans Kyrle's disease in a 41-year-old woman is described. It was a recurrence of a lesion surgically removed and originally diagnosed as invasive squamous cell carcinoma. Kyrle's disease is a rare dermatosis and should not be confused with neoplastic conditions i.e. squamous cell carcinoma. Pathologists should be aware of such conditions in order to avoid misdiagnosis, which can imply over treatment for the patient.

Adult↗

PubMed2002

Ichthyosis follicularis with atrichia and photophobia (IFAP) syndrome in two unrelated female patients.

The IFAP syndrome is characterized by the congenital onset of ichthyosis follicularis, absence of hair, and photophobia. A limited number of patients with the disorder have been described, and X-linked recessive inheritance has been proposed. Two unrelated female patients with a complete IFAP syndrome are reported. Both patients show a diffuse distribution of the disorder without linear arrangement. Because the suggested X-linked recessive pattern of inheritance is unlikely in these patients, a different way of transmission or, alternatively, genetic heterogeneity of the disorder has to be considered.

Abnormalities, Multiple↗

PubMed1992

Cell kinetics in skin disorders with disturbed keratinization.

A relatively simple immunohistochemical method was developed and used on cryostat sections. The monoclonal antibody Ki67 was used as marker for actively cycling cells and Pab601 for germinative cells. Counts were expressed as Ki67- or Pab601-positive cells/mm. In order to improve our understanding of the pathogenetic mechanisms in skin disorders with disturbed keratinization we have measured cell kinetic values in dyskeratosis follicularis, pemphigus benigna familiaris chronica, autosomal dominant ichthyosis vulgaris, X-linked recessive ichthyosis, atopic dermatitis and psoriasis and compared them with previous values derived with autoradiography using tritiated thymidine. The results showed that microscopical acanthosis is related to an increase of the germinative population, while the increased epidermal turnover is associated with increased numbers of cycling cells. The cell kinetic changes seem to be all secondary except in psoriasis where a dysregulation in the epidermal growth may cause the epidermal changes. This simple method allows quick evaluation of drug efficacy which might be useful in atopic dermatitis and psoriasis.

Cell Count↗

PubMed1999

[Acquired reactive perforating dermatosis. Successful treatment with allopurinol in 2 cases].

Perforating disorders represent a heterogenous group of dermatoses characterized by transepithelial elimination of dermal structures. Primary perforating disorders should be distinguished from secondary perforating disorders in which perforation with transepithelial elimination is a rare component of a variety of dermatoses. The primary perforating disorders are hyperkeratosis follicularis et parafollicularis in cutem penetrans (Kyrle's disease), elastosis perforans serpiginosa and perforating folliculitis. Acquired reactive perforating dermatosis (also known as acquired reactive perforating collagenosis) together with the hereditary variant of the reactive perforating collagenosis represent further examples of the primary perforating disorders. We report on 84 year old and 96 year old female patients with an acquired perforating dermatosis. Both of the patients additionally showed diabetes and hyperuricemia. Oral administration of allopurinol (100 mg daily) led to a healing of the disseminated skin lesions in 1-2 weeks. After a follow-up period of 6 months, both patients were in complete remission. On one hand, these results prove again the existence and the severity of this disease, and on the other hand suggest an immunomodulating or differentiation-promoting action in addition to the uricostatic effect of allopurinol.

Aged↗

PubMed1993

Barrier function parameters in various keratinization disorders: transepidermal water loss and vascular response to hexyl nicotinate.

In this study, we characterized the stratum corneum barrier function in 39 patients with various keratinization disorders (autosomal dominant ichthyosis vulgaris [ADI] [n = 7], X-linked recessive ichthyosis [XRI] [n = 6], autosomal recessive congenital ichthyosis [CI] [n = 10], dyskeratosis follicularis [Darier's disease; DD] [n = 8], erythrokeratoderma variabilis [EKV] [n = 8]), and 21 healthy volunteers, using two non-invasive methods: transepidermal water loss (TEWL) measuring outward transport of water through the skin by evaporimetry, and the vascular response to hexyl nicotinate (HN) penetration into the skin as determined by laser-Doppler flowmetry. Significantly increased TEWL values were found on the volar forearm in all three forms of ichthyosis, compared with the healthy control group, with the highest TEWL values in the CI group. The penetration of HN on the volar forearm was accelerated in patients with ADI, XRI and CI, as indicated by a shorter lag time (t0) between HN application and initial vascular response. However, differentiation between CI and the other ichthyoses was not possible by this method. When using both methods in DD and EKV, no differences compared with the healthy controls could be detected on the volar forearm, where the skin was principally unaffected; only the measurements from the affected skin on alternative sites demonstrated significantly increased TEWL values. In ADI and CI, however, normal-appearing skin also showed impaired values. We conclude that both TEWL and the vascular response to penetration of HN are suitable methods to monitor the skin barrier function in keratinization disorders, and are helpful in discriminating between these disorders.

Adult↗

PubMed2001

Quantitative histopathologic findings of erythromelanosis follicularis faciei et colli.

BACKGROUND: Even though only 33 cases of Erythromelanosis follicularis faciei et colli (EFFC) have been reported around the world, there have been no quantitative clinicopathological studies yet. The purpose of this study is to examine quantitatively clinical and histopathological evaluations of EFFC using image analysis system. METHOD: Clinical grading of its severity was assessed. Many parameters of the horny layer, granular layer, epidermis and the dermal components were measured and compared to controls by image analysis. The correlation between the histological findings and clinical grading was assessed. RESULT: Thickness and compactness of the horny layer was significantly increased in EFFC. There were no significant differences in the number of melanocytes and severity of pigmentation between EFFC and controls; however, melanization was slightly increased in EFFC. The percent area of the superficial blood vessels was significantly increased and correlated with visual grading of erythema. Diameters of hair shafts and outer root sheaths, and thickness of inner root sheaths were decreased. Basal layer pigmentation was correlated with the clinical features of pigmentation. CONCLUSION: This study supported the findings of hyperkeratosis and dilatation of superficial dermal blood vessels in EFFC. The pigmentation of the basal layer and the percent area of the inner spaces of the superficial dermal blood vessels may reflect the clinical severity more than the other parameters. Although follicular plugging is a characteristic clinical feature of EFFC, enlargement of hair follicles and hair shafts were not characteristic pathologic changes EFFC in our study.

Adolescent↗