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[Segmental pigment anomaly with neurologic disorders].

A 28-year-old female patient who developed an asymmetrically localized depigmentation on the right arm and on the right side of the trunk shortly after birth is presented. The depigmented areas with hyperpigmented macules within them had not changed in shape or extent since their development. At the age of 18 years, neurological alterations (hyporeflexy, sensory deficiencies, muscle atrophy and paralysis) were also observed. Ultrastructural investigations revealing the lack of melanocytes in the depigmented areas and the presence of atypical melanosomes in the hyperpigmented macules indicate a rare pigmentation disorder.

Adult↗

[FACIAL MELANOSIS].

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Facial Dermatoses↗

Skeletal disorders associated with skin pigmentation: a role of melatonin?

Although frequently encountered, no available consensus about the association between skeletal abnormalities and skin pigmentation. Several syndromes are characterized by the presence of skin pigmentation in association with skeletal disorders like neurofibroamtosis 1, McCune-Albright Syndrome, Jaffe-Campanacci Syndrome and Jaffe-Lichtenstein Syndrome. Even in the absence of these syndromes, skeletal abnormalities were detected in all radiologically examined patients having patterned skin pigmentation. Although skin pigmentation is controlled by several factors, melatonin is the most reliable factor to have relation to development of skeletal abnormalities. Recent research works support that melatonin might play a role in bone development and several hypotheses link melatonin with some bone diseases associated with skin pigmentation. It seems that melatonin deficiency is a probable operating co-factor in a lot of clinical situations characterized by skin pigmentation and skeletal disorders. This would explain some of the un-explained observations related to these syndromes and research works along these lines might lead to the development of efficient treatment for these diseases.

Bone Diseases↗

[Chronic gait disorder caused by pigmented villonodular synovitis of the sole of the foot].

A 13 year old girl showed increasingly over one and a half year a gait disturbance suspected to be a progressive neurologic disorder. Laboratory tests and routine x-rays were normal. Soft tissue radiography finally demonstrated a dense area on the sole of the left foot which was excised and identified as a pigmented villonodular synovitis (PVNS). The girl is well now. Because PVNS is a rare disorder in childhood the diagnosis is seldom suspected and difficult; however, it should be considered as a cause for localised pain and its consequences.

Adipose Tissue↗