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[Jadassohn's nevus sebaceus and nevus unius lateris. Report of an observation with multiple dysplasia (author's transl)].

The case of a female patient with multiple ectodermal and mesodermal malformations present since birth is reported. The cutaneous lesions were of two types: Jadassohn's nevus sebaceus and nevus unius lateris. These entities have been described in the literature as congenital dermatologic alterations of nevoid character and organoid structure. They can be considered as congenital epidermal nevi. In many cases, including this one, there are various associated disorders especially of the nervous system, eyes, and skeleton. Both syndromes are cutaneous hamartomas which can be differentiated histologically but not by the anomalies accompanying them. Their dermatologic aspects are very similar. The histopathologic characteristics of the skin lesions of nevus unius lateris consist of hyperkeratosis, acanthosis, and epidermal papillomatosis. In Jadassohn's nevus sebaceus there are also alterations of the skin adnexa, namely the absence of hair follicles and the presence of numerous mature sebaceus and hyperplastic glands. In general, the presence of organoid nevus may be a sign of multiple ectodermal and mesodermal malformations. Both syndromes are often present in the same patient, as in the case described here, and their etiology is the same. It is based on an alteration in embryogenic development affecting primarily, though not exclusively, the formations of ectodermal origin. Thus Jadassohn's nevus sebaceus and nevus unius lateris are both forms of phacomatosis. Clinical cases have in common the cutaneous cited above, either in combination or singly. The other possible signs and symptoms are variable, depending on which stage of embryogenic development is affected. There may be defects in the structures of both ectodermal and mesodermal origin.

Adult

Nevus of large spindle and/or epithelioid cells (Spitz's nevus).

By now it is well recognized that there is a benign melanocytic nevus, common in the young and common enough in adults, that has histological features that are confusable with those of malignant melanoma. The anomaly is usually referred to as benign juvenile melanoma, sometimes as Spitz's nevus, and, by some histopathologists, as spindle and epithelioid cell nevus. All the histological subtleties and variations of the condition are still not fully appreciated and some of them are still being misinterpreted as those of malignant melanoma. We herewith present a study designed to clarify the issue and offer firm criteria for histological differentiation of the nevus in point from malignant melanoma. We also suggest a new name for it and supporting arguments therefor.

Adolescent

Divided nevus spilus and divided form of spotted grouped pigmented nevus.

Two cases of divided nevus spilus and a case of divided form of spotted grouped pigmented nevus are reported. In this group, flat melanotic macules around the eyes were located on the opposite parts of the upper and lower eyelids. The macules were divided by the palpebral fissure when the lids were open, and the two halves of the lesions formed a unit when the lids were closed. Case presentation as such may be intriguing in the clarification of the developmental relation between aberrantly differentiated melanin synthesizing cells and normal melanocytes.

Adult

Organoid nevus syndrome (linear nevus sebaceus of Jadassohn): clinical and radiological study of a case.

A 3 1/2-year-old boy with this syndrome is reported. The clinical features were: linear organoid nevus, characteristically involving the midline of the face with extension to one side (right side in this case), hemimacrocephaly, mental and motor retardation, epilepsy originating from a focus in the right hemisphere, a malformed hemisphere and ventricular system on the right side on pneumoencephalographic and angiographic study and a hamartoma-like malformation protruding into the frontal horn of the right lateral ventricle.

Cerebral Angiography

[The blue rubber bleb nevus or the cellular blue nevus or Bean syndrome. A rare case of iron-deficiency anemia].

Described by Bean in 1958 the Blue rubber bleb nevus syndrome is a viscero-cutaneous hemangiomatosis, a rare condition associating typical cutaneous and visceral hemangiomas of the cavernous type. We report the case of a four year old boy who presented a microcytic and hyperchromic anemia. The previous medical history of this boy was characterized by a large cavernous angioma on the right hand operated on the at the age of three months. The stools were guaiac-positive; examination of the gastrointestinal tract to clarify the etiology of gastrointestinal bleeding was negative. An oral iron therapy was prescribed for several years by the pediatrician. At age nine and half years Labeled erythrocytes demonstrated that gastrointestinal bleeding still continues. The indication for surgical operation was established: Small bowel enteroclysis revealed an oval filling defect in the lumen of ileum. Intraoperative finding: Protuberous hemangioma in the wall of small bowel; resection of the involved intestine and primary anastomosis were performed. On histological examination a cavernous hemangioma has been observed. Clinic and Pathology of this rare condition are discussed.

Anemia

Speckled lentiginous nevus.

Speckled lentiginous nevus is, we feel, a distinctive nevocytic disorder and a clinical variety of nevus-cell nevus. The speckled areas show varying histological patterns that range from nevus incipiens to junctional and compound nevi. The background shows histological features of lentigo simplex. It is our contention that speckled lentiginous nevus should be separated from nevus spilus and nevus spilus tardus (Becker's), which we consider to be variants of epidermal nevus.

Adolescent