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Keratosis punctata palmoplantaris controlled with topical retinoids: a case report and review of the literature.

Keratosis punctata palmoplantaris (KPPP) is a rare genodermatosis with an autosomal-dominant pattern of inheritance. We report the case of a 61-year-old woman who presented with a long history of multiple symptomatic hyperkeratotic papules on the palms and soles. In addition, we review the literature and present the current classification of the heterogeneous group of punctate palmoplantar keratoses, the cutaneous and histologic findings, the differential diagnosis, the possible association with various anomalies including malignancies, and the various treatment options.

Administration, Cutaneous↗

Linkage analysis of keratosis follicularis spinulosa decalvans, and regional assignment to human chromosome Xp21.2-p22.2.

Keratosis follicularis spinulosa decalvans (KFSD) is a rare X-chromosomal disorder. It consists of follicular hyperkeratosis of the skin, scarring alopecia of the scalp, absence of the eyebrows, and corneal degeneration. There is photophobia in childhood, but the symptoms tend to diminish after puberty, and prognosis for vision is good. Some heterozygotes do show clinical symptoms. In a large Dutch pedigree we performed DNA analysis in order to localize the KFSD gene. In 54 individuals, including 21 affected males, RFLP analysis was done using DNA probes covering the X chromosome. Two-point linkage analyses with 19 informative DNA markers revealed significant linkage to DNA probes on Xp21.1-p22.3. The highest lod scores of 5.70 and 4.38 were obtained with DXS41 and DXS16 at a recombination fraction of zero and 4 cM, respectively. Multipoint linkage data place KFSD between DXS16 and DXS269. Our data confirm X linkage of KFSD in this family and tentatively map the gene on Xp22.2-p21.2. Combined with clinical investigation, RFLP analysis may become an important tool in carrier detection.

Chromosome Mapping↗

Topical diclofenac: new preparation. Moderate efficacy in actinic keratosis.

(1) Many treatments are available for actinic keratosis. The most widely used is cryotherapy. Topical application of 5% fluorouracil cream is a second-line option. (2) Marketing authorisation has been granted for a topical gel containing 3% diclofenac, a nonsteroidal antiinflammatory drug. The excipients include 2.5% hyaluronic acid. (3) Animal pharmacology studies and in vitro tests show that hyaluronic acid delays the transcutaneous uptake of diclofenac, leading to higher concentrations in the epidermis. (4) No comparative trials with fluorouracil or other drugs have been published. Five trials comparing 3% diclofenac topical gel with its excipient are available. They show that it takes at least two or three months of treatment for the lesions to disappear in one-third to one-half of patients. Indirect comparison suggests that diclofenac is less effective than fluorouracil in terms of lesion disappearance one month after the end of treatment. The subsequent risk of relapse is unknown. (5) Local adverse effects are numerous and frequent, and include contact dermatitis, skin rash, dry skin, desquamation, pruritus, local pain, and paresthesia. Adverse effects can be due to either diclofenac or to the excipient, and seem to be less intense than with topical fluorouracil. (6) Trials have shown that topical diclofenac is effective at a dose of 0.5 g of gel applied twice a day, but this precise dose is unlikely to be used as no measuring device is included in the packaging. (7) In practice, diclofenac topical gel is less effective than fluorouracil, and is only a moderately effective option when fluorouracil is poorly tolerated and when physical treatments such as cryotherapy fail.

Administration, Cutaneous↗

[Bowenoid actinic keratosis: therapy with intralesional injection of recombinant beta-interferon].

Biopsy-proven bowenoid actinic keratosis located in the pretibial region in each of two elderly women (61 and 81 years) were treated with intralesional injections with a new recombinant beta-interferon. The treatment took the form of intralesional injections three times a week over 3 consecutive weeks. The dose per single injection was 1.5 or 1.0 MU interferon, respectively. Clinical and histological examinations showed a complete response in both patients. Controls up to 18 months showed no relapse. There were no flu-like side-effects. Leukocyte counts decreased by 2700 and 500 cells per microliter during therapy.

Aged↗

[Keratosis follicularis--new genetic aspects].

A hypothesis according to which two types of keratosis follicularis (below) should be differentiated is discussed with reference to clinical findings recorded during mass screening in kindergartens and in a dermatological practice: (1) condition improves, to normal in early adulthood, a type in which the for which heritability not cannot be identified by mass screening; (2) a type with growing incidence, primarily among women up to 30 years of age, caused by an X-linked dominant gene.

Adolescent↗

Keratosis punctata of the palmar creases.

We compared the prevalence of keratosis punctata of the palmar creases among populations living in Belgium, Togo, Peru and the United Arab Emirates. It appears that black Africans and Hindous (prevalence 1 to 2%) are more often affected than Latin Americans (prevalence less than 0.1%). The disease was not seen among Europeans and Arabs. Histologically, the lesions consist of a marked hyperkeratosis located at the site of acrosyringia. The disease should therefore be considered as a sweat gland disorder.

Asia, Western↗

Simultaneous occurrence of junctional nevus and seborrheic keratosis.

A 45-year-old man presented with a pigmented lesion on his face. A clinical diagnosis of melanocytic nevus was made and the lesion was excised. Histopathologic examination showed a reticular seborrheic keratosis associated with a junctional nevus. We discuss the relation of nevocellular nevi with epidermal and adnexal tumors.

Cheek↗

[Keratosis follicularis decalvans: nosological discussion of Siemens' disease. Apropos of 3 cases].

We describe 3 cases of keratosis follicularis decalvans (Siemens' disease): a 15 year old boy and a 7 year old boy and his father. They represent 2 different patterns of the disease with different clinical courses and genetic background: an autosomal dominant type of good prognosis with elevated argininemia and absence of follicular atrophy in both father and son, a sporadic type, clinically severe, with follicular atrophy. This raises the question of the nosology of the so-called Siemens' disease, since it includes actually different diseases of variable prognosis.

Adolescent↗

[Chronic lichenoid keratosis].

A 34-year-old female patient presented with the classical features of keratosis lichenoides chronica, a very rare dermatosis. Typical hyperkeratotic papules in a linear and reticular pattern were located on the upper extremities and the trunk. The nails and the perionychial areas showed marked vegetating lesions. After administration of a combined retinoid-PUVA therapy impressive remission was observed. The clinical picture, the differential diagnosis, in particular from lichen ruber planus, and the therapeutic aspects are discussed.

Administration, Oral↗

Inverted follicular keratosis. A report of two recurrent cases.

Few cases of recurrent inverted follicular keratosis (IFK) have been reported in the literature, none with histopathologic confirmation. The authors report two cases of IFK that recurred within weeks of primary excision. Histologic confirmation was obtained on all four specimens. By establishing the benign nature of the recurrent lesions, unnecessary, possibly drastic surgery was avoided.

Aged↗

Unilateral keratosis follicularis.

Unilateral keratosis follicularis is considered a localized variant of Darier's disease and should be included in the differential diagnosis of zosteriform keratotic eruptions. The authors present a case report and review the treatment; topical retinoic acid appears to be the treatment of choice.

Adult↗

Syndrome of cystic eyelids, palmo-plantar keratosis, hypodontia and hypotrichosis as a possible autosomal recessive trait.

Description is given of two sisters, offspring of a first cousin marriage, who exhibited the following identical lesions: 1) cysts of the borders of upper and lower lids; 2) hypodontia; 3) hypotrichosis; 4) palmo-plantar keratosis and 5) onychodystrophy. It is suggested that this combination represents a previously unrecognized autosomal recessive trait in man.

Aged↗

Keratosis punctata palmaris et plantaris. A morphological study on the relation to the epidermal sweat duct unit.

The palmar and plantar pits of keratosis punctata palmaris et plantaris were examined histologically in both vertical and horizontal sections. Pits were located in the crista superficialis epidermis, especially in the orifice of the sweat duct, but no iodine-starch reaction was detected in pits by perspiration testing. In the center of a pit was a parakeratotic wedge, deflecting the squamous cell layers downwards. The sweat duct was found in this parakeratotic wedge, and was occasionally obstructed.

Aged↗

[Diagnostic value of keratosis palmaris as indicative sign of visceral cancer].

Palmar keratosis is studied in patients with visceral cancer. It is related to sex and localization. Its higher frequency can be observed in these patients than in controls. Their incidence is larger in the male and in patients with cancer localized in oesophagus, lungs and prostate. In woman the incidence is less than in male and in cancers localized anywhere but breast and uterus. This cutaneous lesion is a very important phenotypical sign in oncology and can be considered as paraneoplasic cutaneous marker.

Adult↗

Cytogenetic investigations on Werner's syndrome, Acrogeria and Keratosis Palmo-Plantaris.

The frequencies of sister chromatid exchanges and of aspecific chromosome aberrations have been investigated in the lymphocytes from a Werner patient, from an Acrogeria patient and from three members of a family with Keratosis Palmo-Plantaris. These investigations point out that: 1) the SCE frequency is significatively enhanced in Werner as in KPP lymphocytes, 2) the frequency of aspecific chromosome aberrations is increased only in Werner lymphocytes, without evidence of variegated translocation mosaicism. The findings confirm that SCE and chromosome aberrations do not necessarily result from the same genetic damage and that SCE may represent the cytological evidence of unexcised non fatal DNA lesions, which occasionally may be responsible for carcinogenesis.

Chromosome Aberrations↗

Keratosis punctata of the palmar creases.

We determined the prevalence of keratosis punctata of the palmar creases in 534 individuals. The group screened included patients, visitors, and staff in clinics and in a private office setting. The seven cases discovered were all found in black patients, representing 3.1% of this racial group. No relationship to arsenical agents, syphilis, or genetic factors could be ascertained by history.

Adolescent↗