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PubMedDate not supplied

Vitamin A acid in Darier's disease.

The etiology in Dariers disease (keratosis follicularis) is unknown, but it seems to be that the skin changes are related to a variation of nutritional vitamin A deficiency caused by processes within the organism. The skin condition, in which the main histological change shows follicular dyskeratosis, has a certain similarity to hypovitaminosis A. Several follicular keratoses are influenced by vitamin A or vitamin A acid therapy. The use of vitamin A is limited by its toxicity, but vitamin A acid being less toxic could be an important therapeutic advance in follicular keratoses. In Darier's disease vitamin A acid topically or systemically applied leads to distinct improvement of the skin lesions. Clinically and histologically, some of the treatment effects are similar to effects obtained when using vitamin A. Systemic vitamin A acid (retinoic acid) administration results in elevation of serum levels vitamin A (retinol) and provitamin A (carotene) and after high dosage clinical symptoms develop nearly identical with hypervitaminosis A. Several possibilities regarding the mode of action of vitamin A acid are mentioned.

Administration, Topical↗
PubMed1975

Hypopharyngeal and laryngeal involvement with Darier disease.

The presence of mucosal Darier disease (keratosis follicularis) was evaluated clinically and histologically in four consecutive patients with moderate to extensive cutaneous manifestations of this disease. Fiberoptic endoscopy demonstrated hypopharyngeal or laryngeal involvement or both in each patient. A dearth of corps ronds and grains in these anatomical regions was observed histologically. The routine evaluation of mucosal involvement in Darier disease should result in a higher incidence of hypopharyngeal and laryngeal lesions than is currently known. Furthermore, the recognition of hypopharyngeal and laryngeal involvement with Darier disease must be emphasized because of the clinical similarity to leukoplakia and squamous cell carcinoma.

Adult↗
PubMed2002

Pruritic linear papules on a 75-year-old woman: a case of localized Darier-White disease.

Darier-White disease (DWD), commonly called Darier disease or keratosis follicularis, is a genodermatosis seen in clinical practice. It exists more commonly in the generalized form but can present as a localized condition. Localized DWD is a rare entity characterized by epidermal changes that are like those seen in the generalized form but that are confined to a small area of skin. This entity is postulated to result from a postzygotic mutation and has the potential to be transmitted to offspring, which may result in the severe generalized form. We report a case of localized DWD diagnosed after biopsy of a recalcitrant linear dermatitis and discuss the salient features of this condition.

Aged↗
PubMed2002

Darier's disease associated with bipolar affective disorder: a case report.

Darier's disease, also known as keratosis follicularis, is an uncommon autosomal dominant disorder that may also occur as a sporadic mutation. It is characterized by multiple eruptions of hyperkeratotic or crusted papules at seborrheic areas with histologic acantholysis and dyskeratosis. It usually begins in the first or second decade of life and is equally prevalent in men and women. Darier's disease is caused by mutations in the ATP2A2 gene, which maps to chromosome 12q23-q24.1 and encodes the sarcoplasmic/endoplasmic reticulum calcium ATPase (SERCA2). The co-occurrence of various neurologic and psychiatric diseases with Darier's disease has been reported, including mood disorders, epilepsy, mental retardation, slowly progressive encephalopathy, and schizophrenia. Linkage studies using the association between these disorders and Darier's disease to determine the gene locus of these psychiatric disorders inferred the presence of a bipolar susceptibility gene on chromosome 12q23-q24.1 in the region of the Darier's disease gene (DAR). We report a case of Darier's disease of more than 40 years' duration and bipolar I disorder of 30 years' duration in a 52-year-old man, and provide a brief review of the literature.

Bipolar Disorder↗
PubMed1976

Cornifying Darier disease--a unique variant. II. Surgical treatment.

A case of severe cornifying Darier disease (keratosis follicularis) was successfully treated by deep dermal excision of diseased skin and subsequent dermabrasion, resulting in a remission lasting more than four years to date. Persistent loss of the papillary dermis was observed in the surgically treated, disease-free areas.

Adult↗
PubMed1979

Synergism of vitamins A and E with dermatologic applications.

A synergism between vitamins A and E has been demonstrated by a number of investigators, notably Stanley R. Ames, who showed that in rats on a vitamin E deficient diet, the serum vitamin A level remained low, no matter how much vitamin A was given by mouth, or even by injection, but that adding vitamin E to the diet restored the vitamin A serum level to normal. We have utilized these observations with a high degree of success where vitamin A alone had failed to control three dermatologic conditions involving a defect in keratinization, namely, keratosis follicularis (Darier's disease), pityriasis rubra pilaris, and acne vulgaris. It is possible that a number of additional dermatoses characterized by dyskeratosis or hyperkeratosis might also be benefited by this combination.

Acne Vulgaris↗
PubMed2005

A case of Kaposi varicelliform eruption in Darier-White disease.

Darier-White disease (DWD), otherwise known as keratosis follicularis, is a rare disorder of keratinization of the epidermis, mucous membranes, and nails. It is autosomal dominant in transmission. Patients with DWD are prone to frequent superinfection including the rare complication of Kaposi varicelliform eruption. It is postulated that a defect in cell-mediated immunity may contribute to this predisposition.

Acyclovir↗
PubMed2005

Alopecia areata in Darier disease and clinical findings in five affected family members.

A 32-year-old man known to have keratosis follicularis presented with a new problem of alopecia areata involving his scalp. His Darier disease had been present for 24 years and was manifested by pruritic eruptions that were more severe in the summer months. Other family members, including his father, brother, and two sisters suffered from similar skin manifestations.

Adult↗
PubMed2006

Darier disease: case report with oral manifestations.

Darier disease, also known as keratosis follicularis or dyskeratosis follicularis, is a rare autosomal dominant genodermatosis. It is clinically manifested by hyperkeratotic papules primarily affecting seborrheic areas on the head, neck and thorax, with less frequent involvement of the oral mucosa. When oral manifestations are present, they primarily affect the palatal and alveolar mucosa, are usually asymptomatic, and are discovered in routine dental examination. Histologically, the lesions present suprabasal clefts in the epithelium with acantholytic and dyskeratotic cells represented by corps ronds and corps grains. This paper reports a case of an adult male patient presenting clinical signs of Darier disease in the palatal mucosa and skin on the neck and upper limbs. Intraoral biopsy of the affected area, analysis of family history and evaluation by a multidisciplinary team led to the diagnosis of Darier disease. Dental professionals and pathologists should be aware of these lesions to allow correct diagnosis and proper management of this disease.

Adult↗
PubMed1988

[Localized Darier's disease. Topical treatment with retinoic acid].

We report a new case of localized Darier's disease or keratosis follicularis with successful response to topical retinoic acid treatment. We comment the clinical histopathological features and differential diagnosis of these uncommon variant which has only been reported in 10% of the patients.

Administration, Topical↗
PubMed1979

Unilateral, linear, zosteriform epidermal nevus with acantholytic dyskeratosis.

An infant has a nevus unius lateris with the histopathologic condition of acantholytic dyskeratosis. Similar cases have, in the past, been classified as unilateral Darier's disease. Since it is now recognized that the pathologic changes of acantholytic dyskeratosis are not specific for a given disease entity, we believe the concept of unilateral keratosis follicularis deserves reevaluation.

Acantholysis↗
PubMed1979

[Lichen striatus with nail involvement. Report of 4 cases and review of the 4 cases in the literature (author's transl)].

Four cases of lichen striatus (L.S.) with nail involvement were studied and the four cases published previously reviewed. There are several types of nail dystrophy: fraying, longitudinal ridging, splitting and shredding, onycholysis, and total nail loss. All of them are transient and can be explained by the pathologic changes observed, particulary the transitory disruption of the basal layer. Lichen striatus must be differentiated from systematized eruptions such as lichen planus (the simultaneous occurrence of L.S. and lichen planus is extremely rare and the resemblance of one dermatosis to the other can be so pronounced that a differential diagnosis is difficult) linear localized neurodermatitis, linear psoriasis (almost always associated with more typical lesions of psoriasis elsewhere on the body), linear keratosis follicularis (histologically typical of Darier's disease) and linear epidermal nevus which occasionaly does not appear at birth but later in life and may present nail involvement. In some cases, however, clinical similarity of the inflammatory linear verrucose epidermal nevus to lichen striatus is striking.

Adolescent↗
PubMed1982

[X-chromosome-linked hereditary dermatoses].

In X-linked inheritance, the difference between the terms dominant and recessive is blurred by the Lyon effect. In some X-linked recessive genodermatoses, the Lyon effect makes the detection of heterozygote females possible, either by clinical cromanifestations or by enzymatic demonstration of two functionally different populations of cells. The gene locus of X-linked recessive ichthyosis, however, escapes X-inactivation, but heterozygotes can be detected by enzyme analysis in this condition, too. X-linked dominant gene defects with manifestation in both sexes include keratosis follicularis spinulosa decalvans, and probably also the Bazex syndrome. The group of X-linked dominant gene defects with lethality in the male comprises incontinentia pigmenti, focal dermal hypoplasia, the oral-facial-digital syndrome and the CHILD syndrome. Prenatal diagnosis of severe X-linked conditions can be performed when the underlying defect of cell function is known (Fabry disease, Menkes syndrome). In other severe X-linked disorders, the possibility of prenatal determination of the sex may be considered (Wiskott-Aldrich syndrome, X-linked dominant chondrodysplasia punctata, oral-facial-digital syndrome).

Ehlers-Danlos Syndrome↗
PubMed1978

The genetics of follicular atrophoderma.

Follicular atrophoderma apparently does not occur alone but is associated with other abnormalities. It is present in three groups: (1) If it is associated with chondrodystrophia calcificans congenita, it occurs only in girls and is due either to an X-chromosomal gene causing the changes in the heterozygote (ie, in the female sex) and penatal death in the hemizygous state (ie, in the male sex), or to an autosomal dominant gene with expression almost wholly limited to the female sex; (2) If it is associated with Bazex's syndrome, it occurs in male and female persons in successive generations and is due either to an autosomal dominant gene or to an X-linked dominant gene; and (3) If it occurs in association with keratosis palmoplantaris dissipata, keratosis follicularis, and hyperhidrosis palmoplantaris, it may be due to a recessive gene or a mutation.

Atrophy↗
PubMed1997

Darier's disease associated with multiple café-au-lait macules.

Darier's disease, or keratosis follicularis, is an autosomal dominant inherited skin disease characterized by keratotic papules predominantly on the upper trunk and scalp, palmar pits, and nail dystrophy. It has been reported in association with basal cell carcinoma and other skin cancers, neuropsychiatric disorders, salivary gland obstruction, renal and testicular agenesis, bone cysts, and psoriasis. We describe the first report of a patient with multiple café-au-lait macules, but no personal or family history of neurofibromatosis, who developed Darier's disease.

Adult↗
PubMed1997

Subungual squamous cell carcinoma in Darier's disease.

We report the first case of subungual squamous cell carcinoma in a patient with Darier's disease (keratosis follicularis). The presence of human papillomavirus DNA was confirmed, consistent with its probable role in carcinogenesis at this site. The role of an altered immune state is discussed as an additional factor. Squamous cell carcinomas may be overlooked when they occur among the other cutaneous manifestations and complications of Darier's disease. A solitary, ulcerating nail bed lesion warrants biopsy to exclude squamous cell carcinoma.

Adult↗
PubMedDate not supplied

Erythromelanosis follicularis faciei et colli associated with keratosis pilaris in two brothers.

Erythromelanosis follicularis faciei et colli is characterized by well-demarcated erythema, hyperpigmentation, and follicular papules. Since the original description, it has seldom been reported in the literature. We present two adolescent brothers who had this disorder associated with keratosis pilaris on the shoulders and the extensor surfaces of the arms. Dermatologic examination found brown-red pigmentation, erythema, and follicular papules on both maxillary, preauricular regions, and the cheeks. The lesions of the older brother were more prominent. Histopathologic examination of skin biopsy specimens taken from both brothers revealed hyperpigmentation of the basal layer, follicular plugging, dermal vascular dilatation and congestion, and perivascular inflammatory infiltration. We suggest that the coexistence of these two conditions in brothers implies a genetic inheritance and a possible relationship between the disorders.

Adolescent↗