11/54. Hypomelanosis of Ito: report of two cases.Hypomelanosis of Ito is an uncommon syndrome where bizarre, systematized hypopigmentation is often associated with neurological and other non-cutaneous abnormalities. We report two cases, one which was associated with laryngomalacia and sudden death (in which the parents were consanguineous), and one which was associated with episodes of loss of consciousness.- - - - - - - - - - ranking = 1keywords = hypopigmentation (Clic here for more details about this article) |
12/54. Leukoderma in association with giant congenital nevi: report of two cases.Two patients are presented in whom giant congenital nevi were associated with hypopigmentation. One patient has had no associated melanoma. The second patient developed hypopigmentation years before a melanoma was excised, and increased hypopigmentation was noted years later without evidence of melanoma recurrence. While the mechanism for the development of the hypopigmentation noted in these two patients in uncertain, an immunologically mediated systemic process may be responsible.- - - - - - - - - - ranking = 4keywords = hypopigmentation (Clic here for more details about this article) |
13/54. Oculocerebral syndrome with hypopigmentation (Cross syndrome): report of a new case.A syndrome of ocular and cutaneous hypopigmentation, severe mental retardation with spastic tetraplegia and athetosis was first observed by Cross in three siblings of an inbred amish family. Since then, seven other patients, three sporadic and four with familial recurrence, have been reported in the literature, confirming the autosomal recessive inheritance. The clinical spectrum of the syndrome has been expanded to include true developmental defects of the CNS such as cystic malformation of the posterior fossa of the Dandy-Walker type. We report a new case of Cross syndrome.- - - - - - - - - - ranking = 5keywords = hypopigmentation (Clic here for more details about this article) |
14/54. 'Dyschromia in confetti' as a side effect of topical immunotherapy with diphenylcyclopropenone."Dyschromia in confetti" is a pigmentary disturbance that so far has not been reported to have occurred during topical immunotherapy. In a group of 243 patients treated with diphenylcyclopropenone for alopecia areata, four patients showed a similar pattern of hyperpigmentation and hypopigmentation. In one patient the pigmentary disturbance was confined to the site of diphenylcyclopropenone application on the scalp. Besides dyschromic skin changes on her scalp, one patient had development of a hyperpigmentation on her forearms. In the remaining two patients, dyschromia in confetti became manifest exclusively in areas other than the scalp. This type of pigmentary disturbance appeared 4 to 14 months after the start of treatment, and it has remained unchanged in three patients during a follow-up period of 3 to 10 months. In one patient, the pigmentary changes disappeared when therapy was discontinued but recurred, albeit to a lesser extent, after treatment had been resumed. We conclude that dyschromia in confetti should be regarded as a possible, but probably rare, side effect of topical immunotherapy with diphenylcyclopropenone, especially in individuals with a rather dark complexion.- - - - - - - - - - ranking = 1keywords = hypopigmentation (Clic here for more details about this article) |
15/54. Pigmentary mosaicism following the lines of Blaschko in a girl with a double aneuploidy mosaicism: (47,XX, 7/45,X).We report on a 6-year-old girl with linear streaks of apparent hypopigmentation and hyperpigmentation following the Blaschko lines, growth retardation, bupthalmos of the left eye, and mild mental retardation. She had a 45,X karyotype in lymphocytes. In cultured fibroblasts a double aneuploidy mosaicism was detected, consisting of a cell line with trisomy for chromosome 7 and a cell line with monosomy for the X-chromosome and no cell line with a normal karyotype. Cutis tricolor or three levels of pigmentation in different skin areas suggested presence of a third, probably normal cell line. Double aneuploidy mosaicism of a cell line with monosomy X and a cell line with trisomy of an autosome is a rare finding. The combination of monosomy X with trisomy of chromosomes 8, 10, 13, 18, and 21 has been reported, but not the combination with trisomy 7. In the 45,X cell line, microsatellite analysis showed loss of the maternal X-chromosome, and presence of a maternal and paternal chromosome 7. The 47,XX, 7 cell line showed a paternal and a maternal X-chromosome, and a paternal and two identical maternal chromosomes 7. Mechanisms that might explain this double aneuploidy mosaicism are discussed.- - - - - - - - - - ranking = 1keywords = hypopigmentation (Clic here for more details about this article) |
16/54. incontinentia pigmenti achromians of Ito: an ultrastructural study.A clinico-pathological and EM study of a Thai boy with hypomelanosis of Ito, one of the neurocutaneous syndromes, is reported. At birth, typical skin hypopigmentation on the trunk and a hypopigmented streak on the left lower extremity were noted Associated cutaneous pathology shows a decrease of melanin granules within basal and malpighian keratinocytes. Ultrastructural studies highlight a normal appearance for basal and malpighian keratinocytes, but a lack of melanosomes in the malpighian cells. melanosomes are also dramatically reduced in the basal keratinocytes, which appear small, single or clustered and surrounded by a membrane. Melanocytic degeneration has been observed and dendritic melanocytes contained various stages of nonmelanised (stage II), partially melanised premelanosome (stage III) and rarely stage 4 melanosomes. The authors observed an increase in the number of Langerhans cell which have not previously been described. There were unmyelinated axon of nerve containing melanosomes at the dermoepidermal junction. The significance of these findings will be worthwhile to note that abnormal nerve termination in close relationship with basal keratinocyte, degenerated melanocyte, premelanosomes and langerhans cell are important in explaining the pathogenesis of Hypomelanosis of Ito.- - - - - - - - - - ranking = 1keywords = hypopigmentation (Clic here for more details about this article) |
17/54. Aquagenic syringeal acrokeratoderma: report of a case with histologic findings.Aquagenic syringeal acrokeratoderma is a rare acquired condition characterized by painful symmetric swelling and hypopigmentation of the palms and lateral fingers, which develops after brief exposure to water. Histopathologic examination suggests that an aberration in the eccrine sweat gland apparatus may be the underlying cause of this condition. The "hand-in-the-bucket sign," in which patients arrive in their physician's office with their hand in a bucket of water to more readily demonstrate their lesions, is such a common presentation that it almost can be regarded as pathognomonic. All 12 cases reported to date have been in young females. We report a case of aquagenic syringeal acrokeratoderma in a male with unique histologic findings.- - - - - - - - - - ranking = 1keywords = hypopigmentation (Clic here for more details about this article) |
18/54. Hypomelanosis of Ito and hemimegalencephaly.Hypomelanosis of Ito is a congenital neurocutaneous syndrome with a particular pattern of swirling hypopigmentation. Multiple extracutaneous abnormalities involving the central nervous system, the eyes, and musculoskeletal structures occur in over two-thirds of the cases. This report describes two patients with typical unilateral cutaneous lesions associated with extracutaneous features, including hypertrophy of the cerebral hemisphere contralateral to the cutaneous hypopigmentation. magnetic resonance imaging and EEG findings support the diagnosis of hemimegalencephaly, as has recently been reported in other isolated cases of this rare phakomatosis.- - - - - - - - - - ranking = 2keywords = hypopigmentation (Clic here for more details about this article) |
19/54. The prevention and management of postdermabrasion complications.The complications of keloids, pigment changes, loss of skin texture, and enlarged facial pores remain a problem with dermabrasion patients. Their occurrence can be reduced by proper patient selection, proper dermabrasion technique, proper wound management, and prompt treatment. Keloids are rapidly resolved with the use of flurandrenolide tape covered with positive-pressure chin-strap dressings. Streaks of hyperpigmentation are lightened with a combination of sunscreen, opaque makeup, tretinoin, and hydroquinone lotions. The loss of skin texture can be prevented by not abrading too deeply and avoiding subsequent bacterial contamination during wound healing. However, enlarged skin pores in the central portion of the face and hypopigmentation can be permanent complications.- - - - - - - - - - ranking = 1keywords = hypopigmentation (Clic here for more details about this article) |
20/54. Hypomelanosis of Ito (incontinentia pigmenti achromians). Ophthalmological evidence for somatic mosaicism.The authors report on a ten-year-old boy with hypomelanosis of Ito. He suffered from epileptic seizures and exhibited typical generalized partial skin hypomelanosis in whorl-like and striated pattern following Blaschko's lines. The fundi showed patchy, mottled hypopigmentations becoming increasingly striated in the periphery with a general orientation to the optic nerve head. This pattern of affection reminds of the retinal findings in carrier women for X-linked ocular albinism. magnetic resonance imaging revealed multiple small areas of increased relaxation time scattered in the white matter of the brain, which are interpreted as porencephalic cysts. These clinical findings suggest somatic cell mosaicism even though the cytogenetic study was not conclusive.- - - - - - - - - - ranking = 1keywords = hypopigmentation (Clic here for more details about this article) |
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