PubMed Health⌕ Search

SEARCH · PubMed Health

Results for “KERATOSIS FOLLICULARIS”

Explore indexed PubMed citations for clinical trials, systematic reviews and public health research. Read source abstracts and follow each citation to its original PubMed record.

Quote a phrase for an exact phrase match. Source license links do not imply unrestricted reuse.

At least 145 records · Page 8Linked to original sources
PubMed1997

[Acrokeratosis verruciformis-like changes in Darier disease].

In the literature, a number of observers reported that acrokeratosis verruciformis (Hopf) often evolves in cases of keratosis follicularis. The relationship of these verruciform manifestations to keratosis follicularis is discussed. In this paper, we present three patients with Darier's disease, who have verruciform papules on the dorsal surfaces of the hands and feet. Histopathologically, the papules showed the distinctive structures of Darier's disease. Therefore, these lesions are regarded as an abortive form of keratosis follicularis. It is necessary to examine carefully the punch biopsies histomorphologically. We also recommend having a closer look at the development of other typical Darier's skin lesions in the follow-up of patients.

Acrodermatitis↗
PubMed1991

Ultrastructural changes in Darier's disease induced by ultraviolet irradiation.

A male patient with clinically and histopathologically verified Darier's disease and a history of deterioration after sun exposure was irradiated on uninvolved skin with 25, 50 and 75 J/cm2 of UVA once a week for five weeks. He also received 3 and 5 times his individually established MED of UVB. Since no signs of keratosis follicularis were detected one week after the last irradiation, he was then exposed to 10 times his MED with UVB, whereupon clinically characteristic lesions of keratosis follicularis developed. One week after this exposure, biopsies were taken from the UV-induced lesions and processed for light and transmission electron microscopic investigation. Light microscopy revealed suprabasal lacunae, corps ronds and grains. In the electron microscope, gaps in the basal lamina beneath the suprabasal lacunae were also observed, through which cytoplasmic processes of lymphocyte- and fibroblast-like cells and basal keratinocytes protruded. Parts of keratinocytes with disruptive cell membranes were localized in the gaps of the basal lamina. Also, multiplication of the basal lamina was frequent.

Adult↗
PubMed1982

Oral treatment of keratinizing disorders of skin and mucous membranes with etretinate. Comparative study of 113 patients.

This study reports comparative results of the effects of an aromatic retinoid, etretinate (RO 10-9359), on various disorders of the skin and mucous membranes. One hundred thirteen patients suffering from psoriasis, lichen planus, keratosis follicularis, and various other disorders were treated and examined. The patients received 25 to 100 mg/day of oral etretinate for up to 30 months. Patients with erythrodermic psoriasis, pustular psoriasis, and keratosis follicularis showed the best response. The conditions of patients with psoriasis vulgaris, palmoplantar psoriasis, and lichen planus also improved, but less impressively. The mucous membrane lesions of lichen planus and leukoplakia showed only moderate improvement. The most striking adverse clinical reactions observed were cheilitis (70%), dryness of the mucous membranes (27%), and hair loss (27%).

Administration, Oral↗
PubMed1985

Ichthyosis follicularis with alopecia and photophobia.

We treated two unrelated boys with ichthyosis follicularis, a rare skin disorder characterized by extensive noninflammatory spiny follicular hyperkeratoses, severe photophobia, and generalized noncicatricial alopecia. This disorder must be differentiated from keratosis follicularis spinulosa decalvans; ulerythema ophryogenes; keratosis pilaris rubra atrophicans faciei; atrichia with papular lesions; atrophodermia vermiculata; and keratitis, ichthyosis, and deafness syndrome, all of which share some clinical features. Ichthyosis follicularis with alopecia and photophobia appears to be a familial disorder, but too few cases have been reported to establish the exact mode of inheritance.

Alopecia↗
PubMed1992

[An inverse course of Darier dyskeratosis follicularis].

A 56-year-old female patient had suffered from isolated, unclassified palmoplantar keratosis since childhood. During the climacteric horny papules appeared for the first time on the extremities and trunk. Histologically, the efflorescences on the body and the palmoplantar keratosis were classifiable as keratosis follicularis. Predilection sites of Darier's disease were spared. Altogether, the usual pattern of involvement was reversed.

Biopsy↗
PubMed1981

[Siemens I syndrome--a case history].

Report on a patient suffering from keratosis follicularis spinulosa decalvans (Siemens I syndrome). This ia a very rare clinical picture, at least in its complete form. The patient's father and brother are suffering from an abortive Siemens I syndrome. The condition is a form of hereditary parakeratosis, a spinulose keratosis with typical localization in the eyes and on the skin. Causal therapy is not possible; attempts at conservative and surgical treatment are described. Prognostically, the condition will probably come to a standstill at puberty. The description of this case is intended to illustrate the necessity of close cooperation between dermatologist and ophthalmologist.

Adolescent↗
PubMed1987

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↗
PubMed1989

[Electron microscopic study of Darier's follicular dyskeratosis].

Ultrastructure of the skin biopsy specimens of 10 patients with Darier's keratosis follicularis (DKF), 6 of them with the vesicular form of the dermatosis, has been examined by electron microscopy. The study has revealed dyskeratosis with tonofilament homogenization and well-developed keratohyalin granules, round bodies and granules, and acantholysis less manifest than in Hailey-Hailey's chronic benign familial pemphigus. Ultrastructural features of the skin in vesicular DKF present as acantholysis and dyskeratosis more manifest than in typical DKF. Similarity of the morphologic signs of the typical and vesicular DKF permit considering the vesicular form of this keratosis as one of DKF variants.

Adolescent↗
PubMed1996

Localization of annexin I (lipocortin I, p35) mRNA in normal and diseased human skin by in situ hybridization.

Annexin I is a calcium- and phospholipid-binding protein that is involved in the regulation of cellular differentiation. The aim of the present study was to determine the localization of annexin I mRNA expression in normal and diseased human skin. In situ hybridization with a specific digoxigenin-labelled RNA probe was used throughout. We detected no annexin I mRNA signals in basal and suprabasal cells of normal epidermis, but positive signals were evident in the sudoriferous ducts. Annexin I mRNA expression was detected in the keratinizing squamous cells in keratotic type seborrhoeic keratosis and in keratinocytes at the periphery of the horn pearl in well-differentiated squamous cell carcinoma. Positive signals were also seen at the border between involved and noninvolved skin in psoriasis vulgaris and in dyskeratotic epidermal keratinocytes in keratosis follicularis Darier. By contrast, no annexin I mRNA signals were detected in tumour cells in basal cell carcinoma. The present results suggest that annexin I expression is related to, and may play a role in, keratinization disorders.

Annexin A1↗
PubMed1988

[Cutaneous signs of Noonan's syndrome. Apropos of a case with ulerythema ophyogenes, disseminated pilar and sudoral keratosis and progressive alopecia].

A case of typical Noonan syndrome (NS) with eye abnormalities, pterygium colli, cryptorchid testes, lymphoedema and asymmetrical cardiac septal hypertrophy is reported in a 8-month old infant. This case was particularly interesting since it included skin manifestations which enabled an early diagnosis to be made. Ulerythema ophryogenes has already been proposed as a cutaneous marker of NS, but the keratinization disorders in our patient also included disseminated keratosis of both hair follicles and sweat glands orifices. Abnormally brittle short curly hair has already been reported, but our patient exhibited progressive alopecia which is very rare in NS. Biochemical hair analysis did not show any abnormalities of aminoacids. All these features were suggestive of keratosis follicularis spinulosa decalvans. It therefore seems very likely that NS is associated with keratinization disorders but ulerythema ophryogenes might only be the limited form of these disorders. The other skin manifestations of NS are reviewed. Since the patient had 4 "café au lait" spots, the relation of NS with Von Recklinghausen syndrome, and neurofibromatosis-Noonan syndrome is discussed. Watson's Leopard and cardio-facial syndromes overlap with, and may represent subsets of NS.

Abnormalities, Multiple↗
PubMed1994

Clinical findings, cutaneous pathology, and response to therapy in 21 patients with keratosis pilaris atrophicans.

BACKGROUND: Keratosis pilaris atrophicans defines a group of cutaneous disorders characterized by follicular hyperkeratosis and scarring. X-linked dominant inheritance has recently been reported in a Dutch family with a form of keratosis pilaris atrophicans defined as keratosis follicularis spinulosa decalvans, with males more severely affected and having corneal involvement. The clinical manifestations observed in different families by others and ourselves did not follow that pattern, suggesting genetic heterogeneity. We report our experience with 21 unrelated individuals. RESULTS: There were 15 male and six female patients whose onset of the skin disease was in early childhood but with scalp involvement occurring in the teen years. The cutaneous lesions consisted of follicular papules with scalp involvement present in eight individuals. Half the women had scalp involvement, and one female and one male had eye changes. Familial involvement was observed in three patients and was compatible with dominant inheritance. Histopathologic examination revealed hyperkeratosis of the upper follicle with an inflammatory response that resulted in follicular destruction. Response to therapy including keratolytics, antibiotics, corticosteroids and retinoids was limited. CONCLUSIONS: Our findings support the hypothesis that there is genetic and clinical heterogeneity among the disorders represented by the term keratosis pilaris atrophicans. The cause of these diseases may be a disorder of the keratinocyte, which is responsible for inducing both the hyperkeratosis and inflammatory changes.

Adolescent↗
PubMed1999

Efficacy of erbium:YAG laser ablation in Darier disease and Hailey-Hailey disease.

BACKGROUND: Among different surgical approaches, dermabrasion and carbon dioxide laser vaporization have been used to treat Hailey-Hailey disease (HHD) (familial benign chronic pemphigus) and Darier disease (DD) (keratosis follicularis), with various results. Because of the erbium: YAG laser's unique absorption characteristics in tissue water, erbium:YAG laser ablation combines the advantages of both techniques, avoiding thermal injury of vaporization and also allowing selectively deeper tissue removal in the follicular lesions of DD. Therefore, good results should be expected in both types of acantholytic disorders. OBSERVATIONS: Four patients (2 with HHD and 2 with DD) with different affected areas were treated with laser ablation. During a follow-up period ranging from 8 to 20 months, complete remission was achieved in 3 patients--2 with DD and 1 with HHD--and significant improvement was achieved in 1 patient with HHD. Histological examination of control biopsy specimens after ablation in 1 patient with DD revealed no signs of the disease and only a slight fibrosis in the papillary dermis. CONCLUSIONS: Erbium:YAG laser ablation effectively removes lesions of both HHD and DD and can also yield excellent long-term results in chronic, recalcitrant cases.

Adult↗