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Results for “Pigmentation Disorders”

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At least 1,261 records · Page 70Linked to original sources
PubMed1994

Flashlamp-pumped pulsed dye laser treatment of port-wine stains.

BACKGROUND: Port-wine stains, or capillary malformations, occur in 0.3-0.5% of newborns and have been shown to be very responsive to treatment with a pulsed dye laser (585 nm, 450 microseconds). Lesional characteristics that may help predict the ease of response to therapy have not been delineated. There exists a great range of variation in response to treatment. OBJECTIVE: A retrospective study of 133 patients with 140 port-wine stain malformations being treated with the flashlamp-pumped pulsed dye laser was undertaken. Lesion characteristics such as color and location, patient age, and skin type were evaluated as to their correlation with clinical response. Adverse sequelae were evaluated as well. METHODS: Charts of treated patients were reviewed and results tabulated. The five treating physicians were each responsible for reviewing and evaluating their own patients. Patients were treated at 8-12-week intervals using slightly overlapping pulses with fluences from 5.5 to 7.5 J/cm.2 RESULTS: Factors favoring a positive clinical response were pink lesion color, lesion location of the head and neck, patient age less than 10 years, and patient skin types I-III. Factors having a more negative impact on clinical result include purple lesion color, lesion location on the distal extremities, patient age > 50 years, and skin types IV and V. CONCLUSION: Examination of various patient and lesional characteristics shows trends that may be useful in a predictive manner regarding ease of treatment with the flashlamp-pumped pulsed dye laser.

Adolescent↗
PubMed1994

Selective decrease of eumelanin in hypopigmented epidermis of hypomelanosis of Ito.

A Japanese infant had bilateral hypopigmented macules in a whorled or marble-cakelike configuration on her trunk and extremities. Her irides were bluish gray and her hair was dark brown. She had photophobia and macrocephaly with developmental delay. Analysis of peripheral lymphocytes and dermal fibroblasts disclosed a normal chromosomal pattern. A new finding was that the eumelanin content of the hypopigmented epidermis was decreased. We suggest that at least a portion of patients with this disorder have a defect in eumelanogenesis in hypopigmented skin.

Epidermis↗
PubMed1999

Melanotic pigmentation in excision scars of melanocytic and non-melanocytic skin tumors.

The appearance of pigmented lesions in melanoma surgical scars is a frequent finding that in some instances may cause confusion with a melanoma persistence. Nevertheless, only a few papers have dealt with this subject in the dermatologic literature. The melanoma surgical scars of 60 consecutive patients were reviewed with special attention to the presence of pigmentation and its clinical characteristics. Simultaneously, the scars of 60 consecutive patients who had been subjected to excision of a non-melanoma skin tumor were also studied. Biopsies were performed in representative clinical cases of pigmented lesions arising on the scars of both groups, as well as in non-pigmented scars, and processed for hematoxylin-eosin and immunohistochemistry. Pigmented lesions were present in a similar percentage in both groups (30% in melanoma scars (18/60) and 25% in non-melanoma scars (15/60)). Clinically, three types of clinical pigmentation were observed: lentigine-like lesions; pigmented streaks in scars after direct closure; and diffuse pigmentation in grafts. Histologically, two patterns emerged: one with lentiginous epidermal hyperplasia, hyperpigmentation, and a normal or moderately increased number of melanocytes; and a second one characterized by melanocytic hyperplasia of a variable degree. The scar process itself, irrespective of the tumor excised, seems to be responsible for the pigmentation. We suggest the existence of an induction process of scar tissue acting on melanocytes of the overlying epidermis.

Adult↗
PubMed1975

Fanconi's aplastic anaemia with short stature. Absence of response to human growth hormone.

A patient with idiopathic marrow hypoplasia associated with short stature and other anomalies (Fanconi's anaemia) is described: treatment with human growth hormone for one year did not accelerate his growth rate or significantly affect his anaemia: androgen treatment considerably improved both features. Endocrine studies suggest that though he had poor and insufficient production of endogenous growth hormone to insulin-induced hypoglycaemia, the major defect in this syndrome is determined more at the end-organ than at the pituitary or gonadal level.

Anemia, Aplastic↗
PubMed2004

Impaired gallbladder motility and delayed orocecal transit contribute to pigment gallstone and biliary sludge formation in beta-thalassemia major adults.

AIM: Gallbladder and gastrointestinal motility defects exist in gallstones patients and to a lesser extent in pigment gallstone patients. To investigated the role of gallbladder and gastrointestinal motility disorders in pigment gallstone formation in beta-thalassemia major. METHODS: Twenty-three patients with beta-thalassemia major (16 females; age range 18-37 years) and 70 controls (47 females, age range 18-40 years) were studied for gallbladder and gastric emptying (functional ultrasonography), orocecal transit (OCTT, H(2)-breath test), autonomic dysfunction (sweat-spot, cardiorespiratory reflex tests), bowel habits, gastrointestinal symptoms and quality of life (all with questionnaires). Gallbladder content (ultrasonography) was examined before and during 8-12 mo follow-up. RESULTS: Gallstones and/or biliary sludge were found in 13 (56%) patients. beta-thalassemia major patients had increased fasting (38.0+/-4.8 mL vs 20.3+/-0.7 mL, P = 0.0001) and residual (7.9+/-1.3 mL vs 5.1+/-0.3 mL, P = 0.002) volume and slightly slower emptying (24.9+/-1.7 min vs 20.1+/-0.7 min, P = 0.04) of the gallbladder, together with longer OCTT (132.2+/-7.8 min vs 99.7+/-2.3 min, P = 0.00003) than controls. No differences in gastric emptying and bowel habits were found. Also, patients had higher dyspepsia (score: 6.7+/-1.2 vs 4.9+/-0.2, P = 0.027), greater appetite (P = 0.000004) and lower health perception (P = 0.00002) than controls. Autonomic dysfunction was diagnosed in 52% of patients (positive tests: 76.2% and 66.7% for parasympathetic and sympathetic involvement, respectively). Patients developing sludge during follow-up (38%, 2 with prior stones) had increased fasting and residual gallbladder volume. CONCLUSION: Adult beta-thalassemia major patients have gallbladder dysmotility associated with delayed small intestinal transit and autonomic dysfunction. These abnormalities apparently contribute together with haemolytic hyperbilirubinemia to the pathogenesis of pigment gallstones/sludge in beta-thalassemia major.

Adolescent↗
PubMed1976

Hypopigmentation with punctate keratosis of the palms and soles.

Generalized discrete hypopigmented macules forming a camouflage pattern appeared on the skin of a man. The condition has been present and has not changed since the age of 6 months. Five other members of his family are reported to have the same abnormality. All affected members also have punctate keratosis of the palms and soles. An autosomal dominant pattern of inheritance is suggested.

Adolescent↗
PubMed1987

Hypopigmentation in the Prader-Willi syndrome.

Cutaneous and ocular pigmentation were evaluated in 29 individuals with the Prader-Willi syndrome (PWS). Criteria for hypopigmentation included the presence of type I or II skin, the lightest skin type in the family by history, and iris translucency on globe transillumination. On the basis of these criteria, 48% of the PWS individuals were hypopigmented. The presence of hypopigmentation correlated with a small interstitial deletion on the proximal long arm of chromosome 15; however, this deletion was also found in individuals who did not meet the full criteria for hypopigmentation. Hairbulb tyrosinase activity and glutathione content, as well as urine cysteinyldopa excretion, were low in PWS individuals with and without hypopigmentation and did not separate these two groups. We conclude that hypopigmentation is found in a significant proportion of individuals with PWS and that the hypopigmentation may be associated with a deletion of the long arm of chromosome 15. The mechanism for the hypopigmentation is unknown.

Chromosome Deletion↗