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Localized chrysiasis induced by laser therapy.

BACKGROUND: Chrysiasis is a rare blue-gray skin discoloration that occurs in sun-exposed sites of some patients who receive gold salts. A unique case of localized chrysiasis developed immediately after Q-switched ruby laser (694 nm) irradiation for postinflammatory hyperpigmentation secondary to granuloma faciale in a patient who was receiving long-term gold sodium thiomalate therapy for psoriatic arthritis. Skin biopsy specimens showed striking changes in the ultrastructural characteristics of cutaneous gold deposits following laser treatment. OBSERVATIONS: A blue-gray skin discoloration developed immediately after laser exposure and persisted unchanged after 1 year. Transmission electron microscopy of skin biopsy specimens showed electron-dense gold deposits. Before laser irradiation, these deposits were 106 +/- 35 (mean +/- SD) nm in diameter and faceted, consistent with a crystalline structure. Posttreatment deposits were round, smaller, measured 16 +/- 4 nm, and resembled colloidal gold. Identical findings were observed in an area of sun-protected skin treated with the Q-switched ruby laser; irradiation with a pulsed dye laser at 585 nm had no effect. CONCLUSIONS: Localized chrysiasis was induced in a patient receiving parenteral gold therapy who underwent treatment with a Q-switched ruby laser. This form of chrysiasis resulted from a structural alteration in dermal gold deposits. A similar physiochemical modification in gold deposits induced by UV light may explain the localization of chrysiasis to sun-exposed skin in affected patients.

Arthritis, Psoriatic↗

Photodynamic therapy in AIDS-related cutaneous Kaposi's sarcoma.

For evaluating the role of photodynamic therapy (PDT) in the local treatment of acquired immune deficiency syndrome (AIDS)-related cutaneous Kaposi's sarcoma (KS), nine treatments were performed in eight human immunodeficiency virus-positive homosexual men. The patients received 2 mg Photofrin/kg and either 120 J/cm2 (n = 5) or 70 J/cm2 (n = 4) laser light (630 nm). A total of 83 lesions were evaluable for response with a follow-up of 3-8 months. The overall response rates by patient for all treated lesions were 50-100% (120 J/cm2) and 83.3-90.3% (70 J/cm2), with a median duration of 3 months (range, 2-6 months). Tumors located at the head had higher response rates than those at the trunk or extremities (p = 0.005 and p - 0.015 respectively). The size of the KS showed a negative relationship with the probability of complete response (p = 0.047). Local and general side effects occurred, including pain, blisters, temperature increase, muscle stiffness, and severe edema. The cosmetic result was unsatisfactory because of a high prevalence of scars and long-lasting hyperpigmentation. Although the response rates of PDT are high, light dose of 70-120 J/cm2 cannot be recommended in the treatment of cutaneous KS in combination with 2 mg/kg Photofrin because of severe side effects and unsatisfactory cosmetic result.

Acquired Immunodeficiency Syndrome↗

Interdigital neuroma. Local cutaneous changes after corticosteroid injection.

Interdigital neuroma was diagnosed in a patient who was treated subsequently with a local corticosteroid injection. Two to 3 weeks after injection, a 2.5 x 1.5-cm area of hyperpigmentation, thinning of the skin, and subcutaneous fat atrophy developed at the site of the injection. Occurrence of these side effects depends on the solubility of the steroid preparation, the dosage, and the anatomic site and depth of the injection. When using local corticosteroid injections to treat interdigital neuromas, the physician should be familiar with the properties and recommended dosage of the given steroid. The injection should be deep enough so that the cortisone solution does not leak into the subcutaneous area. The possibility of skin atrophy and altered pigmentation should be discussed with all patients.

Adipose Tissue↗

Peutz-Jeghers syndrome.

Peutz-Jeghers syndrome is inherited as an autosomal dominant trait with variable incomplete penetrance. Patients with Peutz-Jeghers syndrome characteristically have hamartomatous polyps throughout their entire gastrointestinal tract, particularly in the small bowel, and mucocutaneous hyperpigmentation involving the lips, oral cavity, and skin. Although the intestinal hamartomatous polyps have a lower incidence of malignant change in the gastrointestinal tract than do adenomatous polyps, recent information suggests that the overall neoplastic transformation from Peutz-Jeghers syndrome is not a rare event.

Adenomatous Polyps↗

[Idiopathic eruptive macular pigmentation].

BACKGROUND: Hyperpigmentation can be due to different cause or have no apparent cause. CASE REPORT: A 4 year-old boy was examined because pigmentation had appeared 5 months earlier. The cutaneous lesions were blue-grey, macular (diameter: 2-3 cm), with a predilection for the trunk, the lower part of the neck and the proximal part of the legs. There was no pruritus and the lesions did not urticate when rubbed. Histologically, there was epidermal acanthosis, moderate hyperkeratosis and dermal infiltration with numerous melanophages. The clinical lesions remained stable 6 months later. CONCLUSION: The cutaneous manifestations and their course are similar to those of idiopathic macular pigmentation usually reported in adolescents. Ultramicroscopic findings have led some authors to classify it as lichen planus.

Child, Preschool↗

[Cosmetic dermopigmentation. The pigment stays ... as do regrets].

INTRODUCTION: Cosmetic dermopigmentation designates tattooing of the superficial derma with pigments in order to obtain an aesthetic effect. We observed two cases illustrating the inconveniences of this technique. CASE REPORTS: Two women underwent dermopigmentation on the face in a beauty institution to create pseudo-freckles. Definite macular hyperpigmentation occurred with dyschromia in both subjects. COMMENTS: There is a major risk in subjects who undertake dermopigmentation of freckles and/or naevus, a practice which should be discouraged by dermatologists.

Adult↗

[Pigmented erythroderma in AIDS. 5 cases].

INTRODUCTION: We report five cases of pigmented erythroderma occurring during AIDS, noteworthy for its unusual hyperpigmented feature, its advent at the terminal stages of AIDS, and an CD8 cells dermal infiltrate. PATIENTS AND METHODS: It is a retrospective study of five patients infected with HIV: a woman infected by transfusion and four homosexual men, average 55 years old. No one was intravenous drug user. They were all severely immunocompromised; HTLV I/II serology was negative. Skin biopsies were studied with light microscopy (Hematoxylin-eosin) and immunohistochemical studies were performed on frozen sections. RESULTS: The patients had an erythroderma of particular interest because of the associated hyperpigmentation, the severe repercussion (pruritus, weight loss), and the difficulty in treating (except systemic corticosteroids). The histology demonstrated a mononuclear dermal lymphocytic infiltrate, without epidermotropism and atypical cytonuclear feature. The phenotype of the infiltrate was uniformly of the suppressor-cytotoxic subset (CD8+, CD4-). COMMENTS: Our cases are like those previously described as "Pseudo-Sezary", mimicking a lymphoma during AIDS. Numerous factors are probably the cause of this hyperpigmented erythroderma: HIV, CD8 cells... CONCLUSIONS: This severe skin disease, complicating AIDS, seems very particular, but not yet clearly defined. In practice, the problem remains the treatment of this severe erythroderma, because only the systemic corticosteroids are effective, but this is debatable during the treatment of AIDS.

Acquired Immunodeficiency Syndrome↗

Hormonal and genetic analysis of a patient with congenital adrenal hyperplasia.

We describe a patient with signs and symptoms of virilization caused by 21-hydroxylase deficiency. The patient, a Hispanic woman, first sought medical attention at age 24, when she presented to a medical clinic with an uncomplicated urinary tract infection. At that time several signs of virilization were noted and she was referred to the endocrinology clinic. Evaluation revealed temporal balding, hyperpigmentation, acne, absent breast development, a muscular habitus, and clitoromegaly. Radiological studies revealed bilaterally enlarged adrenal glands and ovaries. Laboratory evaluation revealed markedly increased concentrations of 17-hydroxyprogesterone, androstenedione, and testosterone. The patient was diagnosed with congenital adrenal hyperplasia (CAH) and received hormone therapy. In her sister, encouraged to undergo testing for this autosomal recessive disorder, HLA testing demonstrated that certain haplotypes in this family were associated with CAH. The case highlights key steps in the laboratory diagnosis and genetics of CAH.

17-alpha-Hydroxyprogesterone↗

Quinidine-induced pigmentation.

Antimalarial agents have long been known to cause a variety of pigmentary disturbances. Quinidine, a cincha alkaloid and D-isomer of quinine, is widely used for the treatment of ventricular arrhythmias. A paucity of literature, however, exists concerning quinidine-associated hyperpigmentation. We describe a case of focal ceruloderma we believe to be secondary to quinidine therapy.

Anti-Arrhythmia Agents↗

Prominent pigmented fungiform papillae of the tongue.

Prominent pigmented fungiform paillae of the tongue are characterized clinically by prominence and pigmentation confined to these papillae and histopathologically by melanophages in the lamina opriae. A 45-year-old Asian woman had dark erythematous papules exclusively involved with fungiform papillae on the anterior lateral dorsal aspect of the tongue and hyperpigmentation on the lip. Histologic examination revealed many melanophages in the subepidermal area within the fungiform papillae. Her skin lesions improved moderately following two months of treatment for anemia.

Anemia, Iron-Deficiency↗

Treatment of capillary vascular malformation (port-wine stains) with photochemotherapy.

One-hundred and thirty patients (85 female, 45 male) with port-wine stains were treated with photodynamic therapy, also called photochemotherapy, which recently has become acknowledged as effective for a variety of malignant tumors. Probably based on the photochemical reaction with the generation of toxic species, photochemotherapy results in endothelial cell injury and death of abnormal capillaries under overlying epidermis. A retrospective review of 118 available patients with port-wine stains reveals that 98.3 percent responded to photochemotherapy with varying degrees of success after one-time treatment. Results were reported under a simple classification system ranging from ordinary to dilated to posttreatment type. In the ordinary group, the results evaluated as excellent, good, fair, and poor were 37.8, 53.7, 8.5, and 0 percent, respectively, before a second treatment; the treated area was an average of 9.8 (range 7 to 13) cm in diameter. In addition, hypertrophic scars, permanent hyperpigmentation, and hypopigmentation were not seen based on proper parameters. Photochemotherapy offers a potentially efficient and promising choice based on a completely different mechanism from that of selected photothermal therapy with the pulsed-dye laser.

Adolescent↗

Intradermal urate tophi.

OBJECTIVE: To analyze the clinical features and identify risk factors associated with the development of intradermal urate tophi. METHODS: Six patients (5 men and 1 woman, mean age 59.8 yrs) with intradermal tophi were studied between 1987 and 1996. RESULTS: Intradermal urate crystal deposits appeared as small, superficial, pustule-like, whitish lesions. All patients experienced superimposed inflammatory episodes with increasing pain, swelling, and erythema of the intradermal tophi. In one patient, the lesions were associated with a peculiar skin hyperpigmentation. Five had intermittent liquefaction and ulcerations of the lesions with drainage of white chalky matter from which monosodium urate crystals were recovered. Mean pre-treatment serum urate was 570.6 mumol/l (range 496-720). Risk factors for gout and intradermal tophi included renal failure in all 6, hypertension and chronic diuretic therapy in 4, and one patient each with alcohol abuse, chronic low dose acetylsalicylic acid, myeloma, and a positive family history. CONCLUSION: Intradermal urate tophi with superimposed inflammatory episodes, intermittent ulcerations, and possibly pigmentary changes, are rare skin manifestations of chronic tophaceous gout. Renal insufficiency, hypertension, and chronic diuretic use are factors associated with the development of hyperuricemia and gout in these patients.

Adult↗

Alkaptonuric ochronosis: report of two affected brothers.

Alkaptonuric ochronosis is a rare inborn metabolic disorder. Because of the deficient activity of the enzyme homogentisic acid oxidase, homogentisic acid accumulates in plasma, is deposited in various tissues and is excreted in large amounts in urine. Dark brown discoloration of urine on exposure to air or after addition of alkaline solution is characteristic. We describe two brothers with typical alkaptonuric ochronosis with dark urine, blue pigmentation of auricles and axillae, focal brown hyperpigmentation of sclerae, and anthropathy.

Ear Diseases↗

Cutaneous syndrome possibly caused by heartworm infestation in a dog.

A 9-year old German Shepherd bitch was presented with a recent onset of seborrhoea oleosa, hyperpigmentation, erythema, pruritus and alopecia along the neck, thorax, ventrum and the dorsal area of the carpus. The skin changes were believed to be caused by Dirofilaria immitis infection. A combination of topical and parenteral anti-heartworm therapy led to the resolution of the lesions.

Alopecia↗

An intense light source. The photoderm VL-flashlamp as a new treatment possibility for vascular skin lesions.

BACKGROUND: Up to now, vascular diseases were treated with various lasers, such as argon, pulsed dye, and copper vapor lasers, which can lead to side effects like hypopigmentations, hyperpigmentations, and scarring. We treated patients with vascular lesions with an incoherent intense light source, the PhotoDerm VL-flashlamp. OBJECTIVE: The aim of the study was to test the effectiveness and safety of the PhotoDerm VL for vascular skin lesions. METHODS: One hundred and twenty patients with facial or leg telangiectasias, spider nevi, erythrosis interfollicularis, and senile angiomas were treated with the PhotoDerm VL. RESULTS: In 73.6% of patients there was an immediate clearing, and in 84.3% a clearing after 1 month was found of leg telangiectasias up to 1 mm in diameter. Facial telangiectasias and erythrosis interfollicularis colli showed clearance up to 90%. Spider nevi and senile angiomas often only needed to be treated once. CONCLUSION: From our experience we conclude that the PhotoDerm VL is an excellent device to treat vascular lesions as there were hardly any side effects seen, however, the user needs a sufficient experience to get good results.

Cicatrix↗

Topical mechlorethamine. Cutaneous changes in patients with mycosis fungoides after its administration.

Six patients with mycosis fungoides were treated with topical mechlorethamine hydrochloride for periods of two to four years. Clinical and histological studies for radiomimetic and radiodermatitis-like effects failed to demonstrate any abnormalities. The only observed changes were generalized hyperpigmentation of the skin and melanin-containing melanophages in the papillary dermis. We consider that the long-term use of topical mechlorethamine may be a safe form of therapy, but that a continuous indefinite follow-up of patients on this medication should be mandatory.

Administration, Topical↗

Porphyria turcica. Twenty years after hexachlorobenzene intoxication.

Porphyria involving more than 4,000 persons occurred in Eastern Turkey from 1956 to 1961 due to ingestion of hexachlorobenzene (HCB), a fungicide added to wheat seedlings. During a site visit in 1977, 32 porphyric Turks (mean age, 29 years) were examined. Porphyrin concentrations were determined with samples of urine and stool from 29 porphyric and 10 control Turks and compared with results from 40 subjects in the United States. Distinctive clinical features were found in porphyric subjects, including hyperpigmentation, hirsutism, severe scarring, short stature, pinched facies, small hands, painless arthritis, weakness, and enlarged thyroids. Porphyrin levels were still substantially elevated in five subjects. Hexachlorobenzene, which is fat soluble, was detected in maternal milk of one porphyric patient and in one other patient's fat. The level of HCB was not considered sufficient to account for persistence of porphyria but may be a clue to the cause of pembe yara (pink sore), in which many children who were breast-fed by affected mothers died with associated weakness, convulsions, and annular erythema.

Adipose Tissue↗

Pyodermite végétante of Hallopeau. Immunofluorescence studies performed in an early disease stage.

A 50-year-old woman with autoimmune thyroiditis had an eruption resembling pyodermite végétante as described initially by Hallopeau. Direct immunofluorescence microscopy studies indicated deposition of IgG and C3 in the intercellular area of the epidermis. Circulating IgG antibodies to the intercellular areas of stratified epithelial tissue were demonstrable on indirect immunofluorescence microscopy. Treatment with prednisone and azathioprine resulted in the disappearance of vetetating lesions and in healing, with postinflammatory hyperpigmentation. Immunofluorescence microscopy studies, in an early stage of the disease, are of great value in the diagnosis of pyodermite végétante of Hallopeau. Our findings support the view that pyodermite végétante of Hallopeau belongs immunologically to the pemphigus group.

Animals↗