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Query: UMLS:C0027960 (mole)
 21,279 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

We treated three patients with inflammatory linear verrucose epidermal nevus. The lesions were either verrucose or psoriasiform and all had an inflammatory component. Histologic features included psoriasiform acanthosis with spongiosis and parakeratosis. Features that distinguish this entity and the differential diagnoses are discussed.
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PMID:Inflammatory linear verrucose epidermal nevus. 62 80

Skin lesions of three patients with inflammatory linear verrucose epidermal naevus (ILVEN) were examined. Histologically, orthokeratosis and parakeratosis were alternately seen in the acanthotic epidermis. By N-(7-dimethylamino-4-methyl-3-coumarinyl)maleimide staining, the horny cells in the parakeratotic epidermis showed a cytoplasmic SH pattern and a weak membranous SS pattern. The orthokeratotic epidermis revealed an increased involucrin expression, whereas the parakeratotic epidermis showed almost no involucrin expression. Ultrastructurally, in the parakeratotic epidermis, the living keratinocytes had prominent Golgi apparatuses and vesicles in the cytoplasm. In the intercellular spaces in the upper spinous layer through to the lower horny layer, an electron dense, homogeneous substance was deposited. The cytoplasm of the horny cells was filled with keratin filaments and contained remnants of nucleus and cytoplasmic membrane structures, and some lipid droplets. The marginal band formation was incomplete. Most of these ultrastructural abnormalities were not found in the orthokeratotic epidermis. There are both similarities and differences in histopathogenesis of the parakeratotic epidermis between ILVEN and psoriasis. A unique finding was the lack of involucrin expression in the ILVEN parakeratotic epidermis.
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PMID:Histopathogenesis of inflammatory linear verrucose epidermal naevus: histochemistry, immunohistochemistry and ultrastructure. 172 70

A 7-month-old boy came to use with flat papules and small erosions on the extensor aspect of his left forearm and a linear arrangement of verrucous papules on the dorsum of his left hand. Histological examination revealed hyperkeratosis, parakeratosis, irregular acanthosis, and marked acantholysis from suprabasal through upper epidermis. This is the first reported case of linear epidermal nevus with acantholytic dyskeratosis in Japan.
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PMID:Linear epidermal nevus with acantholytic dyskeratosis in an infant. 227 48

We report on a patient with verruciform xanthoma lesions in an epithelial nevus and developmental anomalies since birth that may be classified as CHILD syndrome (congenital hemidysplasia with ichthyosiform erythroderma and limb defects). Histological analyses of the lesion revealed verrucous features with acanthosis, hyperkeratosis, parakeratosis and the typical infiltrate of xanthomatous cells in dermal papillae. To our knowledge only 5 cases of verruciform xanthoma on epidermal nevus have been reported to date. In our view, the present case of CHILD syndrome is the first reported in which xanthomatous transformation has been revealed in biopsies taken when the girl was a baby.
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PMID:Another CHILD syndrome with xanthomatous pattern. 235 7

Verruciform xanthoma is an unusual lesion that was initially reported in 1971. Clinically, it is a verrucous, vascular, epithelial plaque or papillomatous growth commonly found on oral or mucosal tissues. Histologically, it is characterized by a verruciform epithelium with abundant parakeratosis and by foam cells occupying the vascularized dermal papillae. This is the second extramucosal verruciform xanthoma reported. Our patient had several unique features. She is the youngest patient described to date, her lesion is the largest reported, and the lesion occurred in an epithelial nevus-type eruption of a phocomelic extremity. Light and electron microscopic studies disclosed abnormal keratinization, mast cell proliferation, and the previously described foam cells. We believe that the verruciform xanthoma in our patient represents part of a reaction to the epithelial nevus occurring in association with a developmental anomaly.
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PMID:Verruciform xanthoma in an epithelial nevus. 681 Jul 62

Biopsies from 7 patients with white sponge nevus were examined histologically. Two of them had family history. Light microscopic observation reveals that the epithelium was thickened; the spinous cell layers had enlarged and oedematous cells and ballon-like degeneration; the keratohyalin granules were increased in upper spinous cells; the superficial layers of epithelium exhibited parakeratosis and thickening. 3 specimens were examined by electron microscopy. The basal and parabasal cells were cylindrical. The cytoplasmic tonofilaments were aggregated into boundles. It was characteristics of keratinized epithelium. In middle and superficial stratum spinosum the tonofilaments were homogeneously distributed in the cell plasma and the electron-dense granulars were located near the cell boundary and attached to desmosomes by tonofilaments, unlike normal non-keratinized oral mucosa. Many Odland bodies with a lamellar structure and irregular in both size and shape were seen. It is concluded that this leasion have a dyskeratinized process. The characteristic features of keratinized oral mucosa in non-keratinized mucosa is appeared. The thickening of superficial layer is due to shedding disturbance relating to dysfunction of Odland bodies.
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PMID:[The ultrastructure study of white sponge nevus in oral mucosa]. 748 47

We report a healthy 16-year-old Caucasian boy, who consulted us for white, asymptomatic lesions in the mouth. The lesions were stable and had been present for 6 years. On physical examination, there were diffuse white, soft, corrugated plaques involving the buccal and labial mucosa, oral commissures, and floor of the mouth. No other mucosae were affected and there were no skin or nail abnormalities. The histologic findings revealed epidermal hyperplasia with parakeratosis and intracellular edema in the squamous cell layer. No nuclear atypia was observed. A differential diagnosis of three entities is proposed: white sponge nevus, leukoedema, and focal epithelial hyperplasia.
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PMID:Diffuse whitening of the oral mucosa in a child. 926 18

A 38-year-old woman presented with unilateral lesions on the left side of the body and in the genital area. Clinically, the lesions showed a polymorphic pattern: brownish papules in the axilla, keratotic comedo-like papules on the hand and foot, and whitish papular plaques on the labia majora and anal canal. There was no family history of skin diseases. Histologically, cutaneous and mucosal specimens were characterized by acantholytic and dyskeratotic cells, corps ronds and grains in the parakeratotic zone, and by hyperkeratosis and parakeratosis. A diagnosis of epidermal naevus with acantholytic dyskeratosis was made.
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PMID:Acantholytic dyskeratotic epidermal naevus localized unilaterally in the cutaneous and genital areas. 966 38

We report a unique case of a congenital keratinocytic naevus associated with severe alopecia, onychodysplasia and palmoplantar involvement in a 13-year-old girl. The lesions, consisting of scaly, spinous and verrucous papules and plaques, mainly followed Blaschko's lines and have remained unchanged since birth. The predominant histopathological picture was that of a column of parakeratosis overlying the eccrine ostia and hair follicles. This is the first case of a systematized keratinocytic naevus characterized by histopathology of eccrine and hair follicle porokeratosis and a widespread bilateral involvement. This may be a distinct entity to be included in the differential diagnosis of linear, hyperkeratotic dermatoses. We suggest its classification as systematized porokeratotic eccrine and hair follicle naevus.
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PMID:Systematized porokeratotic eccrine and hair follicle naevus: report of a case and review of the literature. 1060 58

White Sponge Nevus (WSN) is a rare, autosomal dominant disorder that predominantly affects noncornified stratified squamous epithelia. Clinically, it is characterized by the presence of soft, white, and "spongy" plaques in the oral mucosa. The characteristic histopathologic features are epithelial thickening, parakeratosis, and vacuolization of the suprabasal layer of oral epithelial keratinocytes. Mutations in keratin 4 (K4) and keratin 13 (K13) genes have already been demonstrated to be responsible for WSN; the identification of new keratin mutations in a stratified squamous epithelia closely related to epidermis is of relevance for the understanding of the biochemistry of intermediate filaments, and for genotype phenotype correlations. In this study we investigated a 27-y-old, female Italian patient, affected by white asymptomatic oral plaques. Sequence analysis revealed a 3 bp (ACA) heterozygous insertion localized in the helix initiation motif of the 1A alpha helical domain of K4. We report this new K4 gene mutation and describe an amino acid insertion, in the 1A domain, responsible for a keratin disease.
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PMID:A glutamine insertion in the 1A alpha helical domain of the keratin 4 gene in a familial case of white sponge nevus. 1065 3


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