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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

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

[The diagnostic value of the epidermal pigmentation in nevus-cell nevi (author's transl)].

The pigmentation of epidermis in areas of nevus-cell nevi is frequently not specially noted, but may be of considerable diagnostic value in particular cases. Two cases are demonstrated. Multiple tumors with the aspect of nevus Spitz in a 35-year old woman were histologically diagnosed by means of melanin impregnation methods as "amelanotic nevus-cell nevi with overlying vitiligo". A flat papillomatous nevus-cell nevus of the abdominal skin of a 38-year old man showed clinical signs of activity, but histologically only a total depigmentation of the epidermis and of the peripheral parts of the nevus was observed.

Adult

Clinical and histopathological studies on spotted grouped pigmented nevi with special reference to eccrine-centered nevus.

Clinical and histopathological studies on 16 cases of spotted grouped pigmented nevus resulted in this disease being classified into at least three types. Characteristic findings of each type are described in this paper, in particular that nevus cell proliferation, a finding common to each type, is closely related pathogenetically to skin appendages, especially with eccrine sweat ducts. The eccrine-centered nevus characterized by Mishima corresponds to the third type, according to the present author's classification. The similar characteristic histopathological finding was also observed in a specimen taken from a black papule in a case of giant pigmented nevus with disseminated pigment cell nevi. We would like to consider that the cells of eccrine-centered nevus are derived from nevoblasts present in the walls of sweat ducts.

Adolescent

Studies on café au lait spots in neurofibromatosis and pigmented macules of nevus spilus.

Café au lait spots from 14 Japanese patients with neurofibromatosis and nevus spilus from 9 Japanese patients were subjected to the studies on the differences in nature of their melanocytes. When the number of melanocytes of the pigmented lesions was compared with that of the surrounding normal skin, the former was always increased and that of café au lait spot was higher than that of nevus spilus. Giant pigment granules were recognized only in 6 patients out of 14 with neurofibromatosis but not in nevus spilus examined. 2 days after UV irradiation at 4 MED, the number of melanocytes was increased in both surrounding normal skin and pigmented lesion, and the rates of increase were lower in the pigmented lesion. Under the electron microscope, melanocytes in café au lait spots which received an ultraviolet light irradiation showed various changes in their cytoplasm; a development of dendrites containing many mature melanosomes, an increased number of cytoplasmic vacuoles and mitochondria, a development of Golgi apparatus in their cytoplasm, appearances of some dense-bodies and of autophagosomal melanosome-complexes. In nevus spilus, the same kind of changes occurred, but they were moderate compared with those developed in café au lait spots. Melanosomes in the keratinocytes of café au lait spots tended to come together around the nucleus and to form melanosome-complexes; while, melanosomes in the keratinocytes of nevus spilus seemed to be single-dispersed after irradiation. The causative factors of the hyperpigmentation and the different reactivity of melanocytes against UV irradiation in these two pigmented macules were discussed.

Adult

Junctional nevus of the oral mucosa. Light and electron microscopic observations.

Junctional nevi of the oral mucosa are rare and may be precancerous. A patient who had an enlarging junctional nevus of the labial mucosa with an adjacent lentigo simplex was studied by light and electron microscopy. On the basis of morphologic similarities--dentritic appearance, lack of desmonsomes, proliferative melanin production, and lack of cytoplasmic fibrils--it appears that the nevus cell most likely develops from melanocytes. The reason for the transformation from melanocyte to nevus cell or junctional nevus cell hyperplasia is unknown.

Acid Phosphatase