Cases reported "Hypopigmentation"

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1/6. Reticulate acropigmentation of Dohi: a case report of autosomal recessive inheritance.

    Reticulate acropigmentation of Dohi is a rare dyschromic disorder that generally has an autosomal dominant pattern of inheritance. Most of the cases have primarily been described from japan. Only a few similar cases have been described elsewhere. We describe 3 black siblings, one boy and two girls, who had progressive reticulate hyperpigmented and hypopigmented macules over the dorsa of hands and feet, which began in early childhood. There were no palmar pits or breaks of the epidermal rete ridge pattern nor was there a family history of any pigmentary skin diseases. Three skin biopsies were performed on one patient; a biopsy specimen from a hyperpigmented macule showed increased melanin in all epidermal levels tapering towards the surface, a second biopsy specimen from a hypopigmented macule showed much less melanin, but it had a similar distribution. A third specimen from a hyperpigmented macule for electron microscopy showed a moderate number of stage III and IV melanosomes in the cytoplasm of the melanocytes. To our knowledge, these patients are the first cases reported from the middle east with an autosomal recessive pattern of inheritance, confirming previous reports.
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2/6. Treating REM syndrome with the pulsed dye laser.

    BACKGROUND AND OBJECTIVE: REM syndrome (reticular erythematous mucinosis) is a benign but bothersome skin disease that common occurs in middle age and among women. Local and systemic treatment measures are often associated with a high rate of side effects and relapses are common. We evaluated the pulsed dye laser as an alternative method because of its good efficacy in vascular skin diseases. STUDY DESIGN/MATERIALS AND methods: We treated two female patients with REM syndrome using the pulsed dye laser. RESULTS: In both patients the erythematous skin changes were almost completely removed after five and three laser sessions, respectively. Other than transient hypopigmentation, no side effects occurred. In one case there is still no evidence of recurrence 6 years after a trial treatment was conducted. In the same patient, clinical success was histologically confirmed. CONCLUSIONS: This is the first report on the successful treatment of REM syndrome of two female patients with the pulsed dye laser.
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3/6. Cole disease: hypopigmentation with punctate keratosis of the palms and soles.

    Cole disease is an uncommon disorder characterized by distinctive cutaneous hypopigmentation and punctate keratosis of the palms and soles. It is a congenital skin disease with an autosomal dominant inheritance pattern. We report two patients from a family with 15 members, 5 of whom were affected. One of the patients had both types of lesions since birth, while in the other they arose in the first months of life. We studied the pedigree, histopathology, immunohistochemistry, and electron microscopy findings of the hypopigmented macules with the patients' normal skin used as a control. The pedigree showed involvement of both genders, with a Mendelian autosomal dominant inheritance pattern with phenotypic variability in the family. immunohistochemistry showed a reduction in the melanin pigment in the keratinocytes and normal pigmentation in the melanocytes. Ultrastructural studies showed a strong contrast between the large number of melanosomes in the body and dendrites of the melanocytes, in contrast with the small number of these organelles in the neighboring keratinocytes. These findings suggest that this disease is a primary congenital disorder of the transfer mechanisms of the melanosomes from melanocytes to keratinocytes in hypopigmented lesions, associated with abnormal epidermopoiesis in the punctate hyperkeratosis.
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4/6. Streptococcal exanthem in a blaschkolinear pattern: clinical evidence for genetic mosaicism in hypomelanosis of ito.

    Due to the presence of two different clones of cells in early embryogenesis, numerous congenital and acquired dermatoses have a linear distribution following the lines of Blaschko. Acquired inflammatory skin diseases are rarely observed in linear patterns. Our patient was born with macrocephaly, left eye glaucoma, and a left facial and contralateral corporal hemihypertrophy, cerebral dysgenesis, and skeletal abnormalities. Hypopigmented S-shaped linear macules on the trunk and linear streaks on the arms and legs were compatible with hypomelanosis of Ito. At 5 years of age the patient presented with an erythematous follicular exanthem compatible with scarlet fever exclusively in the lines of Blaschko. This fact suggests a genetic mosaicism.
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5/6. Idiopathic guttate hypomelanosis: idiopathic or ultraviolet induced?

    Idiopathic guttate hypomelanosis (IGH) is a common but often unrecognized skin disease with obscure etiopathogenesis. As the lesions mostly develop on the exposed parts of the extremities, ultraviolet has been hypothesized to induce these lesions. However, previous statistical studies did not confirm any association between ultraviolet and IGH. We describe a 72-year-old woman with mycosis fungoides in whom widespread IGH occurred during narrow band ultraviolet B (UVB) therapy. The lesion development in the trunk following narrow band UVB therapy suggests that these lesions may not be idiopathic and they may be associated with UV exposure although controversy exist in the literature. This case is particular as it reveals strong evidence on this relationship.
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6/6. hypopigmentation in hemodialysis. Acquired hair and skin fairness in a uremic patient undergoing maintenance hemodialysis: case report and review of the literature.

    With recent advances in medicine, uremic patients are living longer with an improving quality of life. Several skin diseases have been reported in patients with chronic renal failure, and the opportunity has been offered to elucidate newer cutaneous abnormalities among patients undergoing long-term hemodialysis. hyperpigmentation was the most prevalent cutaneous abnormality observed in these patients, but hypopigmentation remains an exceptional event. We report here a case of a maintenance hemodialysis patient with an acquired hair and skin fairness. Although the true mechanism involved in this entity remains obscure, it can be correlated with a disturbance of phenylalanine metabolism on the basis of the current knowledge.
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