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At least 991 records · Page 55Linked to original sources
PubMed1978

Reticulate pigmented anomaly of the flexures. Dowing Degos disease, a new genodermatosis.

Acquired reticulate pigmentation of the flexures developed in ten patients. Additional features in some cases were pitted scars near the angles of the mouth and scattered dark comedone-like lesions on the neck (dark dot follicles). The disease affects both sexes, usually develops in early adult life, and is slowly progressive. The abnormality is characterized by pigmented filiform epidermal downgrowths closely resembling an adenoid seborrheic wart, but similar proliferations also develop around the variably dilated pilosebaceous follicles. The occurence of the anomaly in siblings and in mother and daughter in two families suggests the condition in a new genodermatosis. Reticulate pigmented anomaly of the flexures bears a spurious clinical resemblance to acanthosis nigricans and, thus, the recognition of this new genodermatosis should spare patients undergoing unnecessary investigations to exclude visceral malignancy.

Acanthosis Nigricans↗
PubMed1982

Reticulate pigmented anomaly of the flexures. Case reports and genetic investigation.

Reticulate pigmented anomaly of the flexures is a pigmented macular disease characterized by punctate, hyperpigmented macules on the flexural areas. Histopathologic findings include acanthosis, keratinization of the follicular infundibulum, and filiform downgrowths of epidermal cells. Examination of a family has revealed the presence of the disease in six members. Analysis of these data suggests that reticulate pigmented anomaly of the flexures is an autosomal dominant genodermatosis with possibly variable penetrance, variable expressivity, and delayed onset.

Acanthosis Nigricans↗
PubMed1980

Pseudoatrophoderma colli. A familial case.

A case of familial pseudoatrophoderma colli was found in a 22-year-old Indian woman whose sister and father had similar eruptions. Pseudoatrophoderma colli is an unusual papillary dermatosis in which the lesions appear atrophic. The condition responded very well to treatment with 5% lactic acid ointment. It is likely that pseudoatrophoderma colli is more common than has been reported. It should be considered in the differential diagnosis, along with acanthosis nigricans and confluent and reticulated papillomatosis of Gougerot and Carteaud when a pigmented and papillary dermatosis is present around the neck and upper part of the thorax.

Acanthosis Nigricans↗
PubMed1994

[Effect of chronic blockade of the opiodergic receptor on insulin resistance in a hyperandrogenic woman].

We report a woman with insulin resistance associated with hyperandrogenism and acanthosis nigricans (HAIR-AN syndrome) treated during 30 days with the prolonged action opioid antagonist Naltrexone. During its administration, decreases in basal blood glucose and serum insulin, insulin and glucose response to a glucose load and plasma testosterone were observed. These findings suggest that opioid activity could play a critical role in the physiopathology of hyperinsulinemia in hyperandrogenic women.

Acanthosis Nigricans↗
PubMed1994

[Extreme insulin resistance type A].

Extreme insulin resistance Type A is defined as the syndrome with decreased insulin sensitivity associated with decreased insulin binding to the receptor. The patients usually present with acanthosis nigricans, hirsutism and polycystic ovary. The mechanism for deceased insulin binding includes decreased affinity of insulin receptors, abnormal biosynthesis of the receptor i.e. defect of processing from proreceptor to mature receptor and increased degradation of the proreceptors. The same mutation in the insulin receptor does not always necessarily show the same phenotypes of skin lesion or the same glucose intolerance.

Acanthosis Nigricans↗
PubMed1994

[Rabson-Mendenhall syndrome].

Rabson-Mendenhall syndrome was initially reported in 1956 by Rabson et al., who described three children with familial hyperplasia of pineal gland and diabetes mellitus. Characteristic features of this syndrome are low birthweight, thickened nails, hirsutism, acanthosis nigricans, dental precosity and dysplasia, polycystic ovary, abdominal proturbance, phallic enlargement and insulin resistant diabetes mellitus. Most patients die of ketoacidosis and intercurrent infection associating with extreme insulin resistance during mid-childhood. This syndrome appears to show autosomal recessive inheritance. Recent reports provide evidence that mutations in the insulin-receptor gene are, at least in pant, the cause of this syndrome, and that recombinant IGF-I (insulin-like growth factor-1) reduces hyperglycemia in patients of this syndrome.

Acanthosis Nigricans↗
PubMed1994

[Donohue's syndrome (Leprechaunism)].

The syndrome of leprechaunism is characterized by the elfin-like face, hirsutism, acanthosis nigricans, absence of subcutaneous fat, and intrauterine and neonatal growth retardation. Most of the cases have severe insulin resistance with the mutation of the insulin receptor gene. In some cases, patient had two different mutations of the insulin receptor gene, and showed extremely severe insulin resistance. However, the genetic abnormalities responsible for the patient's phenotype, other than the insulin resistance, have not been found. Further study to define the additional genetic defects will be necessary to understand the abnormalities of this disorder.

Abnormalities, Multiple↗
PubMed1993

[Cutaneous complications of massive obesity].

Massive obesity leads to nonspecific skin disorders. Skin folds are more numerous and deeper in the obese subject, and can become the site of various disorders. Hence the difficulty of diagnosis and treatment is increased. Acanthosis nigricans should be recognized, but not solely attributed to obesity, since other causes, particularly cancer, may be involved. Finally, leg ulcerations, a frequent consequence of the venous insufficiency inherent in obesity, should not lead to overlook other underlying causes, particularly in diabetic patients.

Acanthosis Nigricans↗
PubMed1976

Insulin resistant diabetes mellitus without the presence of insulin antibodies. A case report.

A case of a 26-year old woman suffering from an insulin resistant diabetes mellitus for 14 years is described. Acanthosis nigricans was diagnosed in the patient's second year and the syndrome of Stein-Leventhal at the age of 15. Diabetes could not be properly controlled either with the daily dosis of insulin as high as 1140 U or with peroral tolbutamide. Fasting serum IRI concentrations were elevated, the secretoric response to the stimulation by glucose or tolbutamide was substantial and protracted. The hypoglycemic response to the i.v. application of commercial insulin was insignificant. Serum growth hormone levels were normal. No presence of insulin antibodies in the serum was detected.

Acanthosis Nigricans↗
PubMed2007

Clinical presentation and etiologic factors of hirsutism in premenopausal Iranian women.

BACKGROUND: Hirsutism is a common clinical condition with different etiologies. Many of these patients have frank or subclinical abnormalities in the adrenal and ovarian steroidogenesis. The disease may be associated with other clinical signs of hyperandrogenism. The objective of this study was to investigate the clinical features of hirsutism and its etiologic factors in premenopausal Iranian women. METHODS: In a cross-sectional study, 790 consecutive premenopausal women referred to the dermatology Clinics of Hazrat-e Rasool and Firoozgar University Hospitals and three private dermatology clinics during 2001 - 2003 with the clinical diagnosis of hirsutism were studied. All patients underwent detailed clinical assessment and transabdominal ultrasonography of the ovaries. Endocrinologic work-up was performed for 285 patients. RESULTS: Hirsutism was mild in 65%, moderate in 32.5%, and severe in 2.5% of the patients. Positive family history was found in 56.2%. Hormonal studies revealed some abnormalities in 35.2% of the patients. Coexisting medical conditions included acne in 70% of the patients, menstrual irregularity in 38.6%, androgenic alopecia in 21.3%, obesity in 6.5%, acanthosis nigricans in 4.9%, and diabetes in 0.6% of the patients. Etiology of hirsutism was identified as polycystic ovary syndrome (62.53%), idiopathic (35.19%), congenital adrenal hyperplasia (0.38%), prolactinoma (0.13%), and undetermined (1.77%). Polycystic ovary syndrome was diagnosed more frequently in women with menstrual irregularity than eumenorrheic patients (97.70% vs. 40.41%, P < 0.001). CONCLUSIONS: Hirsute patients frequently have either elevated androgen levels or clinical conditions associated with hyperandrogenemia. Eumenorrhea does not rule out endocrine abnormality and particularly polycystic ovary syndrome which is a common cause of hirsutism. We recommend performing endocrinologic work up, investigation of coexisting hyperandrogenic states, and evaluation of polycystic ovary syndrome in all patients with hirsutism.

Adolescent↗
PubMed1985

Heterogeneity in autosomal recessive ichthyosis. Clinical and biochemical differentiation of lamellar ichthyosis and nonbullous congenital ichthyosiform erythroderma.

Nonbullous congenital ichthyosiform erythroderma (CIE) and classic lamellar ichthyosis (LI) can be distinguished by clinical, histopathologic, and biochemical findings reported herein. Whereas all patients with CIE (n = 12) had fine white scales, they were heterogeneous with respect to degree of erythroderma, ectropion, and prognosis. In contrast, all patients with LI (n = 6) had large, dark, platelike scales, severe ectropion that showed no improvement with age, and minimal erythroderma. Although the stratum corneum of patients with LI was two to three times thicker than that of those with CIE, the latter group demonstrated more acanthosis, parakeratosis, hypergranulosis, and less prominent dermal capillaries. Studies of scale-lipid content have indicated biochemical correlates of this clinical heterogeneity. These clinical, histologic, and biochemical findings provide useful guidelines to differentiate CIE from LI and strongly suggest that autosomal recessive primary ichthyosis comprises two distinct diseases.

Adolescent↗
PubMed1986

Progressive symmetric erythrokeratodermia. Histological and ultrastructural study of patient before and after treatment with etretinate.

Progressive symmetric erythrokeratodermia is a rare inherited cornification disorder characterized by symmetric erythematous hyperkeratotic plaques. Approximately 25 cases have been reported, but the ultrastructural features have not been well characterized. In this article, we describe the clinical, histologic, and ultrastructural findings noted in our patient. Biopsy specimens taken before treatment with etretinate showed hyperkeratosis with a well-preserved granular layer and acanthosis. Ultrastructural examination disclosed that granular cells contained markedly swollen mitochondria and that corneocytes contained many lipidlike vacuoles. After initiation of treatment, considerable clinical improvement was observed, but without significant histologic modification. The principal posttreatment ultrastructural changes observed were the reduction of mitochondrial swelling in granular cells and the reduction in the number of lipidlike vacuoles in corneocytes.

Adult↗
PubMed1987

Oral hairy leukoplakia. A distinctive marker of human T-cell lymphotropic virus type III (HTLV-III) infection.

Oral hairy leukoplakia (HL) is a newly described lesion occurring principally on the lateral borders of the tongue in immunosuppressed homosexual men infected with human T-cell lymphotropic virus type III (HTLV-III). Clinically, HL appears as a slightly raised, poorly demarcated lesion with a corrugated or "hairy" surface. Histologically, the lesion is characterized by keratin projections on the surface (which often resemble hairs), parakeratosis, and acanthosis. In addition, large pale-staining cells with pyknotic nuclei are seen in the upper stratum malpighii, which appear similar to the koilocytes described in uterine condylomata. Candida organisms are frequently observed on the lesion surface. Little, if any, subepithelial inflammation is present. Human papillomavirus and Epstein-Barr virus have been identified in biopsy specimens from lesions of oral HL. The association of this lesion in patients with HTLV-III infection has been established. We saw a patient with HTLV-III infection and HL, in whom the immunochemical and ultrastructural findings revealed the presence of a mixed viral infection. Because oral HL may be of diagnostic value as an early indicator of HTLV-III infection, awareness of its characteristic clinical, histologic, immunochemical, and ultrastructural features is important.

Acquired Immunodeficiency Syndrome↗
PubMed1992

Molluscum contagiosum. Ultrastructural evidence for its presence in skin adjacent to clinical lesions in patients infected with human immunodeficiency virus type 1. Military Medical Consortium for Applied Retroviral Research.

BACKGROUND AND DESIGN: Molluscum contagiosum in acquired immunodeficiency disease, although not life threatening, is often a marker of late-stage disease and may lead to disfiguring cutaneous lesions. Although most current therapy results in at least temporary clearing of individual lesions, lesions frequently recur and new lesions arise. Examination of hematoxylin-eosin-stained histologic sections in two patients showed changes suggestive of viral infection in the epidermis 0.5 cm and 1 cm lateral to obvious clinical lesions. These areas were clinically free of any lesions. Both routine histopathologic examination and ultrastructural examination were performed in two patients infected with human immunodeficiency virus type 1 (HIV-1) and three non-HIV-1-infected patients. RESULTS: All patients showed histologic changes diagnostic of molluscum contagiosum. In addition, the sections from HIV-1-infected patients showed areas of acanthosis, hyperkeratosis, and nuclear atypia. Electron microscopy of these areas revealed rare viral organisms in these areas. Similar acanthotic, hyperkeratotic areas were not seen in the biopsy specimens from the non-HIV-1-infected patients and no viral particles were found in the epidermis around the lesions. CONCLUSION: Viral structures consistent with molluscum contagiosum are present within the clinically normal epidermis around lesions of molluscum contagiosum in some HIV-1-infected patients. This may explain the large number of lesions seen in these patients and the difficulty in controlling the spread and recurrence of molluscum contagiosum in HIV-1-infected patients.

Acquired Immunodeficiency Syndrome↗
PubMed1995

Cutaneous eruptions from suramin. A clinical and histopathologic study of 60 patients.

BACKGROUND AND DESIGN: During a trial of suramin for advanced solid tumors, a high rate of cutaneous adverse reactions was observed. We present a prospective study, describing the clinical, histopathologic, histochemical, and immunochemical findings of the cutaneous reactions observed in 60 patients treated with suramin. RESULTS: Forty-nine (82%) of the 60 patients studied experienced at least one cutaneous reaction attributable to suramin therapy. Although morbilliform reactions predominated, a wide variety of eruptions was observed. Most reactions occurred within the first 24 hours of therapy and were self-limited despite continued drug infusion. Distinctive cutaneous findings included scaling erythematous papules (suramin keratoses) and a predilection of many eruptions for previously sun-exposed areas. Six patients experienced severe cutaneous reactions, but no patient permanently withdrew from therapy because of cutaneous toxic reactions. Common histopathologic findings included hyperkeratosis, parakeratosis, spongiosis, acanthosis, exocytosis, apoptosis, a perivascular lymphohistiocytic infiltrate, upper dermal edema, and increased dermal mucin. Staining with S100 antigen was markedly positive in several specimens. Only trace amounts of suramin were detected in skin samples tested with high-pressure liquid chromatography. CONCLUSIONS: The incidence of cutaneous toxic reactions from suramin greatly exceeds that seen with other medications. A wide spectrum of skin manifestations were observed, including suramin keratoses and UV recall, although serious reactions were infrequent. Cutaneous toxic reactions neither predicted nor correlated with other toxic reactions from suramin. Suramin may soon become more widely used; practitioners should be aware of the high incidence and wide spectrum of cutaneous toxic reactions to this drug.

Adult↗
PubMed1998

Trimethylpsoralen bath PUVA is a remittive treatment for psoriasis vulgaris. Evidence that epidermal immunocytes are direct therapeutic targets.

BACKGROUND: Psoriasis vulgaris can be effectively treated with trimethylpsoralen (TMP) bath PUVA therapy (psoralen plus UVA), but no data exist on the extent to which psoriatic pathology is affected by this treatment, or on its cellular mechanism of action. OBSERVATIONS: Eleven patients with recalcitrant psoriasis vulgaris were treated with TMP bath PUVA therapy and observed through clinical and histological measures. Clinical resolution of psoriasis was achieved in 10 of 11 patients. Histopathological resolution of epidermal hyperplasia (marked by keratin 16 expression) was achieved in 90% of individuals treated with TMP bath PUVA. Epidermal acanthosis was reduced by 40% at 2 weeks and 66% by the end of treatment. Epidermal improvement correlated best with reduction in intraepidermal T lymphocytes, which were reduced by 76% at 2 weeks of treatment and 93% at the end of treatment. Furthermore following TMP bath PUVA therapy, the numbers of epidermal CD1a+ Langerhans cells were markedly reduced, and CD86+ cells were eliminated. Through in vitro assays, TMP was found to be about 10,000-fold more active as a lymphotoxic agent compared with 8-methoxypsoralen (8-MOP). Additionally, at physiologic concentrations, lymphocytes were killed more readily by TMP PUVA (TMP plus UVA) than were keratinocytes. CONCLUSIONS: Treatment with TMP bath PUVA was effective in treating moderate to severe psoriasis, even in darker pigmented individuals. It is likely that this treatment ameliorates psoriasis through direct effects on activated leukocytes in lesional skin.

Antigens, CD↗
PubMed1999

Migratory ichthyosiform dermatosis with type 2 diabetes mellitus and insulin resistance.

BACKGROUND: In addition to the well-defined hereditary primary ichthyoses, many sporadic or less well-defined keratinization disorders with or without systemic manifestations have been reported. Herein we describe ichthyosiform dermatosis associated with type 2 diabetes mellitus. OBSERVATIONS: The patients were members of a large Arab family with heavy consanguinity. Eighteen members were affected with a variously severe scaly disorder. They showed migratory polycyclic keratotic scaly plaques evolving into diffuse generalized scaling or complete remission. Acanthosis nigricans-like lesions were also noted, and there was an association with type 2 diabetes mellitus. A scarcity of intercorneocyte lamellae and reduction in lamellar body contents were observed. CONCLUSIONS: We could not find a report of a similar dermatosis. Furthermore, an association between ichthyosis and diabetes has not been documented. Therefore, we believe that this may constitute a new entity.

Adolescent↗
PubMed1999

Dermatologic manifestations of Hermansky-Pudlak syndrome in patients with and without a 16-base pair duplication in the HPS1 gene.

BACKGROUND: Hermansky-Pudlak syndrome (HPS) consists of oculocutaneous albinism, a platelet storage pool deficiency, and lysosomal accumulation of ceroid lipofuscin. Patients with HPS from northwest Puerto Rico are homozygous for a 16-base pair (bp) duplication in exon 15 of HPS1, a gene on chromosome 10q23 known to cause the disorder. OBJECTIVE: To determine the dermatologic findings of patients with HPS. DESIGN: Survey of inpatients with HPS by physical examination. SETTING: National Institutes of Health Clinical Center, Bethesda, Md (a tertiary referral hospital). PATIENTS: Sixty-five patients aged 3 to 54 years were diagnosed on the basis of the absence of platelet dense bodies in individuals with albinism and a bleeding diathesis. The presence of a 16-bp duplication in HPS1 was determined by polymerase chain reaction amplification; 40 patients were homozygous for the duplication and 25 lacked the duplication. All patients with the duplication were from northwest Puerto Rico; all patients without the duplication were non-Puerto Rican except 4 from central Puerto Rico. RESULTS: Both patients homozygous for the 16-bp duplication and patients without the duplication displayed skin color ranging from white to light brown. Patients with the duplication, as well as those lacking the duplication, had hair color ranging from white to brown and eye color ranging from blue to brown. New findings in both groups of patients with HPS were melanocytic nevi with dysplastic features, acanthosis nigricans-like lesions in the axilla and neck, and trichomegaly. Eighty percent of patients with the duplication exhibited features of solar damage, including multiple freckles, stellate lentigines, actinic keratoses, and, occasionally, basal cell or squamous cell carcinomas. Only 8% of patients lacking the 16-bp duplication displayed these findings. As a group, the patients with the duplication lived closer to the equator than those without the duplication. CONCLUSION: Patients with HPS exhibit wide variation in pigmentation and dermatologic findings.

Adolescent↗