A case of nevus comedonicus syndrome associated with neurologic and skeletal abnormalities.
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Bone Diseases, Developmental↗
Biomedical subjects
K B Suhr
Publications and source records attributed to K B Suhr.
A case of childhood epithelioid hemangioendothelioma.
Epithelioid hemangioendothelioma (EHE) is a unique tumor, mainly of adults, which is characterized by epithelioid or histiocytoid endothelial cells. It is a low-grade malignant vascular tumor first described as a distinctive entity in 1982 by Weiss and Enzinger. An 8-year-old girl presented with an approximately 3-year history of an erythematous plaque on the left frontal area of the scalp. The diagnosis of EHE confined to the skin was made by clinicopathologic and radiologic evaluation. To our knowledge, there are only 5 reported cases of EHE with skin involvement, 2 of which showed concomitant systemic involvement, and all occurred in adults. Our case is the fourth reported case of EHE confined to the skin and the first to describe childhood onset of isolated cutaneous EHE.
Child↗
Sphingosylphosphorylcholine stimulates contraction of fibroblast-embedded collagen gel.
Sphingosylphosphorylcholine (SPC), a sphingolipid metabolite, has recently been reported to stimulate wound healing in an animal model. To clarify the mechanism of SPC on the healing process, we examined the effect of SPC on wound contraction using an in vitro model. A mixture of human dermal fibroblasts and porcine type I collagen in a serum-free medium was gelled, and then separated from the well after a 12-h incubation. Various reagents were applied to the medium, and its contractile activity was analysed by measuring the amount of contracted surface area. Among the sphingolipid metabolites, SPC and sphingosine-1-phosphate, but not sphingosine, C2-ceramide and C6-ceramide, stimulated collagen gel contraction. Maximal gel contraction, observed at 10 micromol L-1 of SPC, occurred as early as 1 h after the treatment and persisted for more than 48 h. The effect of SPC was not inhibited by pretreatment with antitransforming growth factor-beta or antiplatelet-derived growth factor-BB antibodies. Among the various signal transduction inhibitors, pertussis toxin, staurosporine and H7 were found to inhibit the action of SPC, whereas genistein and tyrphostin A47 were not, suggesting that fibroblast contraction induced by SPC is mediated by a trimeric guanosine triphosphate-binding protein (G protein)-coupled receptor and protein kinase. Our findings imply that the effect of SPC as a healing stimulant might be due in part to stimulation of fibroblast contraction in granulation tissue.
Carrier Proteins↗
Basal cell carcinoma of the nail bed in a Korean woman.
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Basal Cell Carcinoma↗
Malignant hidroacanthoma simplex: a case report.
Hidroacanthoma simplex is a benign tumor of the skin originating from or showing differentiation to the sweat glands. It grossly resembles seborrheic keratosis of Bowen's disease and histologically shows intraepidermal focal growth of epithelial cells. Malignant transformation of this tumor is rare. We report a case of pigmented hidroacanthoma with malignant transformation in a 67-year-old woman. There was a 20-year history of a skin lesion on the right thigh, which first appeared as a small verrucous papule, progressed to a dark-brown colored patch, and then to a pigmented plaque. Histologically, the primary tumor was composed of small squamoid cells with marked cellular atypia. Most of the tumor cells were located in the epidermis. Immunohistochemically, the cytoplasm of some tumor cells showed a positive reaction for epithelial membrane antigen, but not for either carcino-embryonic antigen or the S-100 protein.
Acrospiroma↗
Arginine in the beginning of the 1A rod domain of the keratin 10 gene is the hot spot for the mutation in epidermolytic hyperkeratosis.
Keratin intermediate filaments are expressed in specific type I/type II pairs in the stage of differentiation of keratinocytes. The mutations in the keratin genes expressed in the epidermis are etiologically responsible for several epidermal genetic skin diseases, such as epidermolysis bullosa simplex, epidermolytic hyperkeratosis (EHK), ichthyosis bullosa of Siemens, palmoplantar keratoderma, pachyonchia congenita and white sponge nevus. The mutations of keratins 1/10 which are expressed in spinous and granular layers are confirmed to cause EHK. There are several trials to correlate between the clinical phenotypes and sites of mutations of the keratin genes. One of these is that EHK is divided into two groups: the palms and soles involvement (PS) group and the non-palms and soles (NPS) group. So far the PS group had the mutations in the keratin 1 and the NPS group in keratin 10. Most of the mutations of the NPS group were reported in the beginning of the 1A rod domain and over 2/3 of the mutations in the 1A rod domain were the base pair substitution of arginine. Here we find two different mutations in two unrelated Korean kindreds classified as NPS group-R156C and R156H-in the 1A rod domain of keratin 10. Our results are compatible with the above classification and suggest that the arginine in the beginning of the 1A rod domain is the hot spot for the mutation of the keratin 10 gene.
Arginine↗
A clinical observation of scleredema adultorum and its relationship to diabetes.
Scleredema may occur secondarily to diabetes or independently. The course of scleredema is not known in either type. Twenty-one scleredema patients were included in this study (13 females, 8 males). The patients were divided into two groups according to the presence or absence of diabetes. In the group (11 patients) of scleredema which was secondary to diabetes, lesions were partially improved in 5 patients who had controlled diabetes, although it was difficult to control diabetes with insulin or oral hypoglycemics in such patients. Scleredema appeared insidiously in nine of these eleven patients. The posterior neck was involved, but the face was not. In patients without diabetes, the scleredema lesions began acutely in eight of ten of them. The course of the disease was usually stationary rather than showing acute improvement. Facial involvement was found in half of all the patients. In conclusion, scleredema patients with diabetes may improve if their diabetes is controlled, and facial involvement may be related to scleredema without diabetes.
Acute Disease↗
Mucinous nevus: report of two cases and review of the literature.
We report two cases of nervus mucinosis with papulonodules distributed dermatomally on the right T12 and L1 areas. Case 1 is a 61-year-old man who exhibited nodular lesions on the right abdomen in a zosteriform arrangement. Alcian blue staining showed the presence of mucin in the papillary dermis. Case 2 is a 17-year-old man who had a 10-year history of match-head to bean-sized skin lesions, multiple papules, and nodules distributed dermatomally on the right lower back.
Abdomen↗