181/719. Coexistence of Darier's disease and acrokeratosis verruciformis of Hopf. Darier's disease and acrokeratosis verruciformis are inherited with the same pattern and they share similar clinical properties. We present a patient with both Darier's disease and acrokeratosis verruciformis. The patient had had brownish and skin-colored papules, initially on her face and neck, since the age of 25. Similar lesions had gradually spread to various parts of her body and lastly the dorsal aspect of her feet. On dermatological examination, brownish and skin-colored, 2 to 5 mm, keratotic papules were observed on her face, neck, both axillae, inframammary regions, and dorsa of the hands and feet. The nail examination revealed "V" shaped notches and longitudinal ridges on the fingers of both hands. The biopsy taken from the neck showed typical findings for Darier's disease. The biopsy from the dorsa of the left foot revealed the distinctive structures of acrokeratosis verruciformis. In this article the clinical and histopathological findings of these diseases are reviewed based on our patient. ( info) |
182/719. Hyperkeratosis of the heels: treatment with salicylic acid in a novel delivery system. A 43-year-old woman presented with dryness and scaling of the lateral and posterior aspects of both heels, which was diagnosed as hyperkeratotic xerosis (Figure 1). Pertinent medical history included dry skin with winter exacerbation and painful hyperkeratosis of the heels present for many years. The patient applied a topical multivesicular cream formulation of 6% salicylic add (Salex, Healthpoint Ltd., Fort Worth, TX) to one foot b.i.d. The physician was blinded as to which foot was treated. After 2 weeks of treatment, it was apparent that the patient was applying the cream to the right foot, as evidenced by reduced dryness, scaling, and hyperkeratosis (Figure2). The patient continued treatment of the same foot for an additional 2 weeks, revealing a dramatic improvement of the right heel,which appeared smooth and soft and devoid of pain. No irritation was associated with treatment; the patient commented that this was the best her heel had been "in years." Subsequently, the patient treated both heels with salicylic acid 60%, multivesicular cream. A second patient, a 25-year-old woman, was treated for ichthyosis vulgaris and hyperkeratosis of both heels. She presented w ith multiple painful fissures and hyperkeratosis of the posterior heels bilaterally (Figure 3). After I week of topical treatment with salicylic add 6%, multivesicular cream applied b.i.d. to the left heel only, there was rapid resolution of both hyperkeratosis and pain (Figure 4). ( info) |
183/719. Primary cutaneous extraskeletal osteosarcoma under a previous electrodessicated actinic keratosis. Extraskeletal osteosarcoma represents approximately 1% of soft tissue sarcomas and approximately 4% of all osteosarcomas. It is therefore much rarer than osteosarcoma arising primarily in bone. skin as a primary site has seldom been reported. This report describes an extraskeletal osteosarcoma of the skin under a previously electrodessicated actinic keratosis. ( info) |
184/719. Reticular erythematous mucinosis and acral papulokeratotic lesions associated with myxoedema due to Hashimoto thyroiditis. A case is described of a patient with generalized myxoedema due to Hashimoto thyroiditis involving lesions of reticular erythematous mucinosis and acral papulokeratotic lesions with 'church spire' histological pattern. Substitutive treatment with thyroid hormone led to a rapid regression of the cutaneous lesions. The infrequent association of reticular erythematous mucinosis lesions and other forms of cutaneous mucinoses with hypothyroidism is discussed, with special emphasis on the fact that the underlying thyroid disease in all cases was Hashimoto thyroiditis. The association of distal keratotic papules with hypothyroidism has not been previously reported in the literature. ( info) |
185/719. Cannon's disease: clinical and diagnostic implications: a case report. Cannon's disease or white sponge naevus is a relatively rare genetically determined skin disorder. It is inherited as an autosomal dominant trait that displays a high degree of penetrance and expressivity. This article describes cases of Cannon's disease in a mother and her son. ( info) |
186/719. Cytogenetic studies of skin fibroblast cultures from a karyotypically normal female with dyskeratosis congenita. skin fibroblast cultures from a female patient with dyskeratosis congenita revealed markedly increased frequencies of chromosomal breaks, hypodiploidy, and premature centromere disjunction. The frequencies of mitotic disturbances, like ana- and telophase bridges, lagging chromosomes, and micronuclei were almost as dramatically elevated as in cultures from two severely affected patients with fanconi anemia. Provided that our patient is representative for an autosomal form of dyskeratosis congenita, this type of the disease seems to be characterized by chromosomal instability with a characteristic pattern of cytogenetic abnormalities. ( info) |
187/719. Pseudoainhum in porokeratosis of Mibelli. porokeratosis of Mibelli in an adult man with involvement of the skin and oral and genital mucous membranes is described. The unusual features in this case were the late onset and pseudoainhum resulting from a porokeratotic lesion in the right foot. The probable cause of pseudoainhum is reviewed. ( info) |
188/719. Keratosis lichenoides chronica and eruptive keratoacanthoma-like lesions in a patient with multiple myeloma. We describe a 72-year-old woman with a 13-year history of a lichenoid dermatitis, who developed multiple, papular keratoacanthoma (KA)-like lesions and few crater-like nodules on the extremities over a period of 6 months before our observation. Her medical history also recorded multiple myeloma diagnosed a few years before. The long-standing dermatosis was diagnosed, clinically, as keratosis lichenoides chronica (KLC), although, histologically, a lichenoid tissue reaction pattern was not evident. On the other hand, histology from papular and nodular lesions of recent onset was consistent with a possible early phase of KA and spinocellular carcinoma, respectively. Oral acitretin induced regression of KA-like lesions and improvement of KLC but had no effects on crater-like nodules, which required surgical excision. KLC is a chronic disorder of keratinization characterized by lichenoid hyperkeratotic papules arranged in a linear pattern, erythematosquamous plaques and seborrhoea-like dermatitis. We emphasize in our case the association between KLC and multiple possible KAs, never previously reported, and speculate that these two rare conditions may represent here a 'continuum' from a pathogenetic point of view. ( info) |
189/719. A new type of erythrokeratoderma. We describe a Dutch man suffering from a previously undescribed erythrokeratoderma associated with palmoplantar keratoderma and circular constrictions of the fingers. No mutations were identified in the genes encoding loricrin, connexin 26, 30, 30.3, 31 and 31.1, and ARS/complex B. There are some similarities between the disorder described here and other palmoplantar keratodermas and erythrokeratodermas, but assignment to a particular disease category is not possible. Hence we propose that we have delineated a novel type of keratoderma. ( info) |
190/719. scleredema adultorum associated with hyperkeratosis. scleredema adultorum is a rare skin disorder reported to occur usually following streptococcal infections. It has characteristic clinical and histopathologic findings; however, associated hyperkeratosis is not known. We report a 6-year-old girl with scleredema adultorum and hyperkeratosis. The patient's disease had a benign course. To our knowledge, this patient is the first reported with scleredema adultorum associated with hyperkeratosis. ( info) |