631/719. A case of dermatofibrosarcoma protuberans on the right first toe. Reports of dermatofibrosarcoma protuberans on a toe are extremely rare. It frequently occurs on the trunk and extremities. A Japanese woman presented with a dark-brownish hyperkeratotic plaque on the dorsal skin of her first toe. The initial clinical diagnosis of verruca vulgaris prompted treatment with cryotherapy. After that a glossy milky-white tumor appeared. Only the results of the histopathologic examination resulted in a diagnosis of dermatofibrosarcoma protuberans. The unusual macroscopic finding was considered to be due to repetitive stimulation by foot movement. ( info) |
632/719. X-linked dominant chondrodysplasia punctata (Happle syndrome) with uncommon symmetrical shortening of the tubular bones. We describe the case of a 13-year-old girl suffering from chondrodysplasia punctata, associated with ichthyosis arranged along Blaschko's lines, follicular atrophoderma, cicatricial alopecia and coarse, lusterless hair. The patient also showed a congenital cataract of the right eye, dysplastic facial appearance and symmetrical shortening of the tubular bones. The pathogenetic concept of functional X-chromosome mosaicism is reviewed as well as the recent results obtained by molecular research that have failed, so far, to solve the problem of regional assignment of the underlying X-linked gene. ( info) |
633/719. Postirradiation multiple minute digitate hyperkeratoses. We report a new case of multiple minute digitate hyperkeratoses after postmastectomy irradiation therapy for mammary cancer. This is the first case of multiple minute digitate hyperkeratoses in an Asian country as well as the third report of the disorder after irradiation. The characteristic eruption, the consistent trigger of irradiation and its occurrence in patients of different racial groups indicate that multiple minute digitate hyperkeratoses are a distinct clinical entity. ( info) |
634/719. Primary cutaneous angioplasmocellular hyperplasia. Two cases of solitary nodules on the skin of the trunk of young adults showing a peculiar pattern of proliferation of blood vessels and a mixed inflammatory infiltrate with predominance of polyclonal plasma cell infiltration are presented. Follow-up of these patients after complete surgical excision revealed no recurrence. We interpreted the lesions within the morphologic spectrum of plasma cell infiltrates of the skin to be reactive and/or hyperplastic, which we term primary cutaneous angioplasmocellular hyperplasia. ( info) |
635/719. A particular subtype of ichthyosis congenita type III. Clinical, light, and electron microscopic features. We report the case of a second patient with the extraordinary ultrastructural findings of vacuolated structures intermingled with membranes in the perinuclear part of the upper epidermal cells. Clinical, light microscopic, and electron microscopic features of this particular presentation of ichthyosis congenita type III have already been presented by K. M. Niemi and L. Kanerva in 1989. Although our patient has more or less the same light and electron microscopic findings, the clinical picture is more severe. The patient was born as a collodion baby. Later, he showed signs of generalized severe involvement with large scales, erythrodermia, and itching. Successful therapy with retinoids resulted in complete removal of the hyperkeratosis but left the striking reticulate skin pattern. Noting the heterogeneous clinical presentation, the specific electron microscopic findings are diagnostic. No biochemical data on this disease are known. ( info) |
636/719. Changes of verruciform xanthoma in an hiv-1 patient with diffuse psoriasiform skin disease. Verruciform xanthoma occurs most commonly in the oral mucosa; however, rare cutaneous lesions have been described. Although the pathogenesis of this entity is not known, dysregulation of epithelial proliferation and degenerative changes in the epithelium may explain the occurrence of this lesion in association with inflammatory dermatoses, epithelial hamartomas, and epithelial dysplasia. We report an hiv-1 patient with diffuse psoriasiform skin lesions that showed histologic changes of verruciform xanthoma. ( info) |
637/719. Focal acantholytic dyskeratosis on the lip. A case of focal acantholytic dyskeratosis (FAD) in a 55-year-old woman is reported. The lesion was located on the lip and manifested as a painful, oozing, crusted patch lasting for 20 years. Although FAD has been recently reported in the mucosa of the anal canal without cutaneous lesion, we report a case of FAD that appeared on the lip with clinical manifestation. ( info) |
638/719. pemphigus vulgaris--the potential for error: a case report. The importance of correlation, referral, and collaboration between dermatology, or any other medical department, and the oral health profession is reported through a case presentation. A case of pemphigus vulgaris diagnosed from a tongue biopsy and confirmed by immunofluorescence is reported. The patient was treated for actinic keratosis of the scalp for 16 months due to inadequate biopsy material. The diagnostic value of proper biopsy material is stressed. The clinical manifestations leading to a misdiagnosis between actinic keratosis and pemphigus vulgaris are discussed. ( info) |
639/719. A radiologic sign of epidermolytic hyperkeratosis. The radiologic finding of innumerable fine ridges in the outer soft tissues of a patient with severe generalized epidermolytic hyperkeratosis is presented as a sign of the disease. The radiodensity results from the density of keratin and low water content in the scales of epidermolytic hyperkeratosis. ( info) |
| A girl is reported with a hitherto apparently undescribed ectodermal dysplasia syndrome. The main findings include: alopecia, onychodysplasia, hypohidrosis, sensorineural deafness, skin with a tan color and hyperkeratosis (involving also plams and soles), unusual facies (with slight auricle and nose abnormalities), pectus excavatum, severe hyperopia, EEG abnormalities, and retarded bone age. The patient also presents mongoloid palpebral slanting, narrow palpebral fissures, bilateral esotropia, photophobia and dermatoglyphics with extensive ridge dissociation. The etiology is unknown but presumed to be genetic, possibly due to the homozygous state of an autosomal recessive mutation. ( info) |