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Incontinentia pigmenti achromian is a very rare cutaneous disorder of pigmentation which has been reported from Japan, the United States of America, the Caribbean countries, Mexico and India. A case with coarse hair and associated with atopic dermatitis in a West African girl is presented in this brief report. The case, to the authors' knowledge, is the first documented in Africa.
The author desires to attract attention to the disadvantages of drugs for external use which are more and more widely used. The two main reactions observed are contact dermatitis and photosensitisation, but practically all skin lesions may be produced. She then rapidly recapitulates the various responsible agents insisting on dermo-corticoids and the precautions which should be taken in their use.
Phytophotodermatitis is an acute skin reaction that may be easily confused with other causes of contact dermatitis. It is characterized by sunburn, blisters, and/or hyperpigmentation. The reaction takes place when certain plant substances known as psoralens, after being activated by ultraviolet light from the sun, come in contact with the skin. The condition is usually mild and self-limited but hyperpigmentation may persist for many weeks. Failure to recognize phytophotodermatitis in a child may lead to a mistaken diagnosis of child abuse. It is also important to be aware of perfumes and grocery products as causes of this phenomenon.
Melanosis Coli (MC) is a benign pigmentation disorder of the colon occasionally diagnosed at endoscopy. The authors report a case of M. coli in a patient with a gunshot to the abdomen, in whom the entity produced an interesting clinical picture, and a review of the literature on this condition.
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A 49-year-old male patient is presented, who developed hyperpigmented macules on the face. An exact classification of the disorder was not possible on the basis of anamnestic data, histology and electron microscopy. An attempt was made to differentiate it from other known dyschromias of the face.
The literature on the subject is reviewed, special attention being paid to the complex problems of aetiological and pathogenetic classification involved by such lesions in the oral cavity. Stress in salso laid on topographical and histological aspects of pigmented lesions of melanocytic origin.
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We report on a 42 year old male patient having been ill with pseudo-pobstruction and gastroinstestinal atonid for 24 years. He was thought to suffer from Crohn's disease and was treated conservatively. Since the therapy proved to be insufficient, explorative laparatomie was performed and parts of the small and large intestine which were thought to be inflamed, were removed. The histologic examination revealed the deposition of a significant amount of lipofuscin in the muscularis propria. This so-called brown bowel syndrome is known to be responsible for the development of gastrointestinal atonia. It is generally believed that this syndrome is caused by a vitamin E deficiency which may occur due to malabsorption. The brown bowel syndrome is treated sympomatically, and until now only one case achieving a remission of the disease has been reported.
The risk of adverse side effects of neuroleptic agents must be considered carefully in the treatment of acute and long-term psychotic states. Consequences should be assessed by analysis of dose-response relationships, risk factors, and central nervous system (especially extrapyramidal) side effects, as well as the influence of neuroleptics on neurotransmitter systems. Appropriate minimum dosages of neuroleptics must be further evaluated as an effective measure in reducing these side effects.
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Hypomelanosis of Ito (incontinentia pigmenti achromians) is a neurocutaneous syndrome consisting of bizarre, patterned, macular hypopigmentation over variable portions of the body surface. Associated defects in other systems occur, commonly in the central nervous system, in a significant percentage of affected individuals. Six children affected by hypomelanosis of Ito are presented. Similarities and differences between hypomelanosis and incontinentia pigmenti and systematized achromic nevus are discussed.
This article focuses on a few of the most common genetically inherited disorders of pigmentation: multiple lentigines syndromes, Peutz-Jeghers syndrome, dyskeratosis congenita, incontinentia pigmenti, tuberous sclerosis, neurofibromatosis, and Albright's polyostotic fibrous dysplasia.
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