1/41. Keratosis palmoplantaris varians of Wachters.We report a case of hereditary palmoplantar keratosis (HPPK) causing a progressive reduction of the prehension capacity of the fingers due to the presence of hyperkeratotic lesions which had appeared approximately 25 years earlier. These lesions, also involving the soles, appeared yellowish in color, linear or round in shape, symmetrical and often confluent, developed prevalently at the pressure points displaying a non-transgrediens pattern. The histological examination, clinical picture and careful analysis of the literature enabled us to define this form as 'keratosis palmoplantaris varians of Wachters'. As a contribution to a conclusive HPPK classification we discuss the differential diagnosis of this disorder most commonly identified through nummular-linear keratoses also known as Siemens' syndrome.- - - - - - - - - - ranking = 1keywords = keratosis (Clic here for more details about this article) |
2/41. costello syndrome with decreased glucose tolerance.We report a case of costello syndrome, which is an uncommon multisystemic condition with cutaneous manifestations on the palms and soles. In the literature there are 29 cases described, all the studies are published in the genetic literature with a few exceptions. We add a further case associated with impaired glucose tolerance. The diagnostic clinical signs are impressive, and highly characteristic. Cutaneous manifestations are: loose skin of the hands and feet "washer woman's hand", hyperkeratosis palmoplantaris, curly or sparse hair, acanthosis nigricans, papillomata nasi. Coarse, progeroid facial features with a bulbous nose, feeding difficulties in infancy, cardiac involvement with cardiomyopathy or conduction defect, and in our case impaired glucose tolerance also presented. Postnatal growth retardation, mental retardation, and a distinctive friendly personality is characteristic. Hyperextensible fingers with broad distal phalanges and joint contractures were observed, and peroneal hypertonicity required treatment by achilles tendon lengthening. The decreased glucose tolerance is interesting in the view of the acanthosis nigricans. No storage disease and no chromosomal abnormality were observed. Only in one case is a balanced translocation described in the literature.- - - - - - - - - - ranking = 0.5keywords = keratosis (Clic here for more details about this article) |
3/41. Squamous cell carcinoma arising from lesions of porokeratosis palmaris et plantaris disseminata.We report a 63-year-old Japanese man with numerous hyperkeratotic papules of porokeratosis palmaris et plantaris disseminata (PPPD) who developed multiple squamous cell carcinomas on the lesional sites of the palms and soles. The hyperkeratotic papules, which showed tightly packed columns of parakeratotic cells in the cornified layer (cornoid lamella), lost granular layer, and dyskeratotic keratinocytes in the epidermis below the cornoid lamella histologically, had been noticed on the palms and soles from the age of 28 and 43, respectively. He has no family history of such hyperkeratotic papules. Treatment with etretinate (10-50 mg/day) was given discontinuously, and the total dose of etretinate amounted to approximately 21 g over 14 years (average: 0.07 mg/kg/day). He noticed erosions on the hyperkeratotic papules on the left sole and palm more than 9 months after cessation of treatment with etretinate. Histological findings showed numerous atypical keratinocytes in the epidermis and upper dermis with mononuclear cell infiltration seen in the upper dermis. The diagnosis of squamous cell carcinoma arising from the lesions of porokeratosis palmaris et plantaris was made. Five erosions with histologically malignant changes were removed 1 cm from the margin of the erosions. These findings suggest that etretinate may have an inhibitory action on malignant changes in PPPD.- - - - - - - - - - ranking = 3keywords = keratosis (Clic here for more details about this article) |
4/41. Guess what! porokeratosis of Mibelli.A 72-year-old man had noticed, in his early forties, the appearance of well-defined papulous hyperkeratotic lesions, with increasing growth, located on both sides of his feet. After twenty-five years he consulted a dermatologist for the first time. physical examination showed annular papules and rose-coloured plaques with atrophic centres, some of them hypopigmented, with higher and irregular borders, separated from the surrounding skin by longitudinal and well-defined furrows. The lesions presented variable sizes and shapes, some of them punctate, involving exclusively and in a bilateral form, both sides, back and sole of the feet (Figs. 1 and 2). The patient did not report any subjective symptoms. He was immunocompetent and did not remember that any relative had the same disease, nor had he been subjected to radiation treatment.- - - - - - - - - - ranking = 2keywords = keratosis (Clic here for more details about this article) |
5/41. Disseminated superficial actinic porokeratosis associated with topical PUVA.Disseminated superficial actinic porokeratosis (DSAP) is associated with sun exposure and tanning bed usage and has been rarely reported in association with systemic PUVA. We report the first case of DSAP occurring after topical foot PUVA.- - - - - - - - - - ranking = 2.5keywords = keratosis (Clic here for more details about this article) |
6/41. onychomycosis caused by alternaria spp. in Tuscany, italy from 1985 to 1999.Cutaneous phaeohyphomycosis due to alternaria spp. is reported with increasing frequency, especially in patients with immune deficiency. onychomycosis caused by this mould is still rarely observed. Here we report nine cases observed in Tuscany in the period 1985-99; the agent was alternaria alternata in eight cases and alternaria chlamidospora in one. diagnosis was made on the basis of repeated direct microscopic mycological examination and culture, confirmed by scanning electron microscope observation of fragments of colonies. In most cases, the clinical manifestations were dystrophy and distal subungual hyperkeratosis of one or two nails of the feet or hands. Seven cases were treated with oral itraconazole, successfully in six cases, as clinical and mycological recovery was confirmed at follow-up 1 year later.- - - - - - - - - - ranking = 0.5keywords = keratosis (Clic here for more details about this article) |
7/41. Verrucous form of chilblain lupus erythematosus.A 45-year-old woman had symmetrical livid plaques with yellowish hyperkeratoses for 5 years, which progressed on to the fingers and toes and on the soles of the feet. Two years later creamy, whitish areas and maceration appeared on the buccal mucosa and the lips. A skin biopsy revealed massive collagen hyaline degeneration in the perivascular area, hyperkeratosis and hypergranulosis, small lymphocyte infiltrates with several melanophages and extravasates of erythrocytes in the upper corium in perivascular areas and hydropic degeneration of basal keratinocytes. The findings using direct immunofluorescence were compatible with lupus erythematosus (LE). Laboratory investigation showed a slight leucopenia and thrombopenia, a slightly elevated erythrocyte sedimentation rate, hypocomplementaemia C3 and C4, a high titre of rheumatoid factor and antinuclear antibodies positivity of extractable nuclear antigen. The results reflected probably the development of a systemic form of the disease. The patient was successfully managed by methylprednisolone and hydroxychloroquine. After 1 year of therapy, a new skin biopsy revealed a substantial reduction of hyperkeratosis and hyaline degeneration of collagen tissue in the perivascular areas. The combination of the extensive hyperkeratosis and hyalinization thus seems to be features of the long-lasting, untreated lesions in chilblain LE.- - - - - - - - - - ranking = 1.5keywords = keratosis (Clic here for more details about this article) |
8/41. Generalized eruptive porokeratosis of Mibelli with associated psoriasis.A case of eruptive porokeratosis of Mibelli with diverse morphologic features, including circinate macular, circinate plaque and verrucous varieties is presented. No matter how variable the clinical presentation may be, the histologic hallmark of porokeratosis, the cornoid lamellae, is always present. The cornoid lamellae vary in height in relation to how prominent the thready ridge of the clinical lesion appears. Our patient also had psoriasis which initially masked the porokeratotic lesions both clinically and histologically. awareness of the various clinical expressions of porokeratosis of Mibelli would 1) make unnecessary the segregation of certain forms of porokeratosis into separate entities, and 2) help in the recognition of less classical forms of porokeratosis.- - - - - - - - - - ranking = 4.5keywords = keratosis (Clic here for more details about this article) |
9/41. sports-related pads.Knuckle pads, first described by Garrod in 1893, 1 are benign, asymptomatic, well- circumscribed, smooth, firm, skin colored papules, nodules, or plaques. They most commonly occur on the dorsal aspect of the proximal interphalangeal joint of the finger, 2 but also may occur on the dorsal aspects of the foot over joints. 3-5 Knuckle pads may be inherited or acquired. 1 While some authors suggest that trauma is not a significant factor, 6 acquired knuckle pads have been associated with repetitive friction and trauma, 2,7 and may resemble athlete's nodules (also referred to as collagenomas). 1 Histologically, knuckle pads are characterized by hyperkeratosis, hypergranulosis, proliferation of fibroblasts and capillaries, and thickened and irregular collagen bundles. 1 Few cases of knuckle pads involving the lower extremities have been reported. 7 Knuckle pads of the feet have been described in association with inherited syndromes, such as acrokeratoelastoidosis Costa, 3 a syndrome of knuckle pads, leukonychia and deafness, 4 and a syndrome of knuckle pads, leukonychia, deafness and keratosis palmoplantaris. 5 We report a case that illustrates an unusual presentation of acquired knuckle pads of the feet secondary to repetitive friction from athletic gear.- - - - - - - - - - ranking = 1keywords = keratosis (Clic here for more details about this article) |
10/41. Two siblings with tyrosinaemia type 2.We report on two siblings with tyrosinaemia type 2, a 6-year-old girl and her 3.5-year-old brother with a main complaint of painful palmo-plantar hyperkeratosis. The boy presented also conjunctival injection, photophobia, lacrimation and conjunctivitis. blood tyrosine levels were increased in both patients, 150.6 and 202.3 micro mol/dl, respectively (reference value: 5.8 /-2.2 micro mol/dl). After 1 month of dietary treatment with low protein, low phenylalanine and tyrosine, tyrosine levels decreased to 37.7 and 65.6 micro mol/dl, respectively; the cutaneous lesions improved in both of them, and conjunctivitis disappeared. CONCLUSION: the association of bilateral ulcero-conjunctivitis with photophobia and palmo-plantar hyperkeratosis since early infancy is the clue to the diagnosis of tyrosinaemia type 2.- - - - - - - - - - ranking = 1keywords = keratosis (Clic here for more details about this article) |
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