Christian Sigg

Frequency of congenital nevi, nevi spili and café-au-lait spots and their relation to nevus count and skin complexion in 939 children

To determine the prevalence of congenital melanoevocytic nevi (CMN), of so-called congenital nevus-like nevi (CNLN), nevi spili (NS) and café-au-lait spots (CLS) in childhood, a series of 939 children aged 8-16 years had total skin examination. CMN/CNLN were observed in a frequency of 5.9%, NS in 2.1% and CLS even in 32.7%. Except CLS (which occurred more frequently in boys), all of these lesions were equally represented in both sexes. According to the classification of Kopf and coworkers, 44/55 CNM/CNLN were small and 11/55 medium-sized. CMN/CNLN affected preferentially trunk and upper limbs while head and neck were spared. Such nevi occurred more frequently in dark types of skin complexion and showed a tendency to be more frequent in groups of patients with increased average number of acquired melanonevocytic nevi. Remarkably, CLS were also found in 23/55 (41.7%) children with CMN/CNLN and in 12/20 (60%) children with NS (in a higher frequency than such combinations have to be expected). Compared with the data from other studies, both CMN/CNLN and CLS in our patients were observed in highest prevalence in the literature. Since the frequency of CLS in adults is much lower, it cannot be ruled out that some of the CLS disappear in adolescence.

Frequency of Acquired Melanonevocytic Nevi and Their Relationship to Skin Complexion in 939 Schoolchildren

In 939 schoolchildren aged 8-16 years skin complexion was determined by a system of seven categories Anamnestic data concerning tanning ability and history of sunburns, however, were not considered owing to their obvious unreliability in children. Mean nevus count was 17.97, was significantly higher in boys than in girls and increased with age. There was a clear relation of mean nevus number and skin complexion: in fair skin type nevi are most frequent, decreasing in number by increasing of pigment content. Additionally, children with freckles have higher mean nevus count in every category of skin complexion. All literature data critically reviewed as dealing with mean mole count are not comparable in several respects. In different races, however, such studies may serve as a basis for subsequent investigations concerning the questions of probably increasing number of melanonevocytic nevi.

‘Papillomatose papuleuse confluente et réticulée Gougerot- Carteaud’ – A Further Form of Skin Amyloidosis?

In 2 cases with the classical clinical features described in connection with papillomatose papuleuse confluente et reticulee amyloid deposits could be demonstrated by histochemical (alkaline Congo red and thioflavin T reaction) and electron microscopical means. These results allow to discuss the nosological position of this disease.

Dysplastic Nevi and Germ Cell Tumors of the Testis – a Possible Further Tumor in the Spectrum of Associated Malignancies in Dysplastic Nevus Syndrome

Even though the exact spectrum of tumors is not yet defined in the dysplastic nevus syndrome (DNS), the increasing frequency of associated cancers is remarkable. The observation of 4 patients with sporadic form of DNS suffering from seminoma or embryonal carcinoma of the testicle suggests that germ cell tumors of the testis represent another form of associated cancer in DNS.

New Histochemical and Ultrastructural Findings in Three Cases of ‘Papillomatose Papuleuse Confluente et Réticulée (Gougerot-Carteaud)’

After we could demonstrate amyloid deposits both histochemically and ultrastructurally in the papillary body of typical skin lesions in 1 patient with ‘papillomatose papuleuse confluente et reticulee’

Globozoospermia (Round-Headed Human Spermatozoa)

Round-headed human spermatozoa (also called globozoospermia) are characterized by the absence of acrosomes and postacrosomal structures of the spermatozoa causing an atypical roundish shape of the head. A typical case of round-headed human spermatozoa (total globozoospermia) is presented and the literature concerning the morphological and biochemical data known about this rare andrological syndrome is reviewed.

Glucagonoma Syndrome with Necrolytic Migratory Erythema

Two cases of necrolytic migratory erythema caused by a metastatic pancreatic islet cell carcinoma are described. The absence of overt diabetes and complete disappearance of skin lesions during treatment with somatostatin are remarkable. Histological and ultrastructural findings showed the typical features of necrolytic migratory erythema. These two cases demonstrate that skin eruptions may represent the first symptom of glucagonoma syndrome and thus dermatologists may give important diagnostic hints.