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世界核心医学期刊文摘（皮肤病学分册） 2006年第3期2卷 58-59页

作者： El-Darouti m.A. Fawzi S.A. marzook S.A. 罗素菊 Department of Dermatology Faculty of Medicine Cairo University 47 El-Falaki Street Bab El-Louk Cairo 11471 Egypt Dr

Background: Familial gigantic melanocytosis (FGm) is a rare disorder first described in 1984 and termed "familial melanopathy with gigantic melanocytes". The cause of the disorder is still unknown. melanocytes in both hyper- and hypopigmented skin seem to be unable to deliver melanin to the surrounding keratinocytes. Objective: In this study,we report four newcases of FGm. Electronmicroscopic examination was performed in a trial to shed more light on the underlying defect in this disorder. Patients and methods: Patients were examined clinically and biopsies were taken from both hyperpigmented and hypopigmented areas, and divided into two parts; one part was processed for routine microscopic examination with hematoxylin and eosin and masson Fontana stains. The other portion of the biopsy was fixed in glutraldhyde 3% and processed for electron microscopic (Em) examination. Results: By light microscopy, the patients skin showed areas of hyperpigmented basal cells alternating with poorly pigmented areas. Hair follicles in the scalp biopsies showed the same pathology. By Em, pigmented areas showed gigantic melanocytes and heavily pigmented keratinocytes. Nonpigmented areas showed poorly pigmented keratinocytes and fewer, but also gigantic melanocytes. Conclusions: The raindrop-like hypopigmentation in this disorder can be explained by a failure of melanocytes to deliver melanin to their surrounding keratinocytes. The cause of the presence of heavily pigmented keratinocytes in the hyperpigmented zones could not be determined. There is a strong possibility of a more widespread abnormality affecting not just the melanocytes.

关键词：黑色素细胞细胞增生症家族性巨大角质形成细胞活检标本显微镜检查苏木素伊红罕见疾病电镜检查

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基底细胞样毛囊错构瘤

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世界核心医学期刊文摘（皮肤病学分册） 2005年第9期1卷 11-11页

作者： El-Darouti m.A. marzouk S.A. Adbel-Halim m.R.E. 刘艳 47 Bab El-louk St. Cairo 11 471 Egypt

Basaloid follicular hamartoma (BFH) is a unique benign follicular hamartoma characterized by variable clinical presentations, identical histologic features and possible associations with numerous disorders. Basaloid follicular hamartoma may be hereditary or acquired. Hereditary cases may be either generalized or unilateral nevoid. Although the generalized forms are usually associated with systemic manifestations, such as myasthenia gravis, it may occasionally present without internal disorders. On the other hand, the acquired forms of BFHmay present in the form of localized or solitary forms. Herein we present four cases of BFH, one of them (first case) represents a unique form of the generalized variant of BFH, showing no associated internal disorders.

关键词：基底细胞错构瘤单侧痣内脏疾病重症肌无力泛发性遗传型多型性

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