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Oculocutaneous albinism (OCA) increases predisposition to skin malignancies. Nevertheless, the differential diagnosis between melanoma and naevi in patients with OCA is still challenging, because pigmentary lesions have rarely been described in this population. We aimed to describe the dermoscopic patterns of naevi in patients with OCA. We prospectively evaluated 83 naevi from 37 patients with OCA in a single centre in Brazil. Lesions were analysed by eye and by dermoscopy and were grouped by dermoscopic pattern. Eight main patterns were identified: homogeneous structureless pattern (n = 28; 33.7%), globular pattern (n = 27; 32.5%), reticular pattern (n = 8; 9.6%), peripheral reticular pattern with central hypopigmentation (n = 8; 9.6%), peripheral globules (n = 8; 9.6%), irregular brown globules with pink background (n = 2; 2.4%), reticular globular disorganized pattern (n = 1; 1.2%) and peripheral reticular globular with central hypopigmentation (n = 1; 1.2%). We found previously undescribed dermoscopic patterns in patients with OCA, in addition to confirming previously described patterns. These descriptions may help the understanding of pigmented naevi in patients with OCA.

SummaryBackground The most important risk factors for malignant melanoma are skin type I or II, large number of atypical naevi and a history of sunburn in childhood and adolescence. Methods A cross-sectional study was performed to assess skin type, number of pigmented lesions and sun protection behaviour in 1157 12- to 19-year-old Hungarian students at 20 primary and secondary schools in Debrecen, Hungary. After receiving dermatological training, 18 school doctors examined the students' skin. A questionnaire was completed by the students with the assistance of their parents about sun protection, sunburns and the use of sunbed. Data from 612 questionnaires were evaluated. Results Based on the doctors' evaluation, most of the pupils were classified as having skin type II and majority of them had 5–20 naevi, particularly on the trunk. Based on the student's response, 5.2% purposely sunbathed daily, 10.1% did not use any form of sun protection, 32.2% wore sun-protective clothing and 65.7% applied sunscreen generally. 6.9% used sunbed, and 74.0% previously experienced serious sunburn at least once. Indoor tanning statistically correlated with the number of melanocytic naevi. Conclusion A high prevalence of sunburn was reported by the students and some of them did not apply any sun protection methods but used sunbed at a critical age for developing melanoma at a later time. These data highlight the importance of educating children and parents about appropriate sun protection.

Phakomatosis pigmentovascularis (PPV) is a rare cutaneous disorder characterized by combination of capillary malformation and other pigmented naevi. Four types and two subtypes have been described where subtype 'a' present only with cutaneous form and subtype 'b' also with systemic association like in Sturge-Weber syndrome or Klippel-Trenaunay syndrome. Hereby, we report a case where our patient presented with port-wine stain, Nevus of Ota, Sturge-Weber syndrome, and Klippel-Trenaunay syndrome; which has made it a rare combination.

The Proteus-syndrome is a recently described congenital hamartomatosis consisting of numerous clinical features of great variety. Mainly affected are the musculo-skeletal system, primarily by hemihypertrophy, macrodactyly and exostoses, and the skin and the subcutaneous tissue, primarily by pigmented naevi and subcutaneous tumors. The differential diagnosis includes other malformation syndromes, e. g. Klippel-Trenaunay-Weber syndrome and other hamartomatosis. Surgical intervention and treatment is difficult because of a frequency of complications and recurrences. This article describes clinical manifestations of Proteus syndrome, differential diagnosis and therapeutic strategies.

An 8-year-old child with a ring chromosome 7 is presented, the first female and the fourth such individual to be described. The associated anomalies were rather benign: she presented with short stature, minor skeletal alterations, and normal intelligence. The only truly striking feature was the presence of multiple large, pigmented naevi, suggestive of a hamartomatous origin, but unlike those typical of any particular syndrome. Though other ring 7 patients have had naevus flammeus, and one had café-au-lait spots, our proband is the first with an anomaly of chromosome 7 to have such extensive lesions. These four cases of ring 7, which show great phenotypic variation, are reviewed, and the clinical presentation of the proband is also compared with that of patients suffering from terminal, interstitial and translocation-derived 7p and 7q deletions. The formation and behavior of ring chromosomes are discussed, as are the cytogenetic factors which may influence their phenotypic expression.

Background Large, asymmetrical and irregularly pigmented naevi in patients with epidermolysis bullosa (EB) have been reported often to mimic cutaneous melanoma clinically. Objectives As the biological course of these peculiar moles is benign, we assessed EB naevi with a dermatoscope to determine whether they could be reliably differentiated from cutaneous melanoma. Methods We evaluated digital dermoscopic images of 23 EB naevi from 11 patients with EB and analysed these pigmented lesions according to pattern analysis, ABCD rule of dermoscopy and the seven-point checklist. Results Melanoma-associated dermoscopic criteria such as multicomponent pattern (20 of 23), atypical pigment network (17 of 23), irregular dots/globules (16 of 23), irregular pigmentation (22 of 23) and an atypical vascular pattern (seven of 23) were frequently seen in EB naevi. In contrast, other criteria frequently associated with melanoma progression, such as irregular streaks, blue-whitish veil, regression structures (blue-whitish areas) or black dots, were rarely seen. Most lesions gave false-positive results when the scores of the dermoscopic diagnostic algorithms were calculated. Conclusions Recurring dermoscopic structures in EB naevi reveal a distinctive dermoscopic pattern of this recently defined entity. Although EB naevi represent an exception to dermoscopic diagnostic algorithms, their dermoscopic evaluation most often allows us to estimate their benign nature. Nevertheless, as an unequivocal discrimination from malignant melanoma in vivo is sometimes not possible, regular clinical follow up of EB naevi with histopathological evaluation of highly suspicious lesions is mandatory.

Pigmented lesions are commonly found in children and adolescents. It is essential that physicians are aware of the clinical features of congenital and acquired pigmented lesions and their natural history. An accurate diagnosis is critical for appropriate management. In particular, atypical naevi and melanoma recognition would lead to an early, effective treatment and a better prognosis. We discuss the various types of pigmented naevi that can present in childhood, and concentrate on the appropriate treatment for each type of lesion.

Summary We report two patients with an uncommon form of pigmented naevus consisting of grouped follicular papules. A biopsy taken from the lesions showed multiple naevus cells, predominantly around the hair follicles, with sparing of the eccrine glands. The clinicohistopathological term given for this condition is ‘spotted grouped pigmented naevi type I’, and has rarely been reported. We discuss the unusual morphology and differential diagnosis of this condition, and suggest that the term ‘congenital follicular melanocytic naevi’ is more appropriate for this presentation.

We report four patients presenting with rare synchronous upper- and lower-eyelid naevi. The distributions and appearances of these naevi resemble the distinctive periorbital pigmentation of the panda. The possible embryological origin of this naevus and an approach to management are discussed.

Giant congenital naevi (GCN) are disfiguring, potentially malignant pigmented naevi present at birth. The naevus cells in GCN are found throughout the dermis and sometimes penetrate the subcutaneous septa. It is claimed that superficial, more heavily pigmented and biologically different naevus cells reside in the upper dermis. Partial removal of these superficial naevus cells by dermabrasion, laser therapy, curettage or shave excision is less traumatic than excision surgery and produces an acceptable cosmetic result. However, none of these techniques or excision of GCN to superficial fat completely removes the risk of malignant transformation.

Congenital pigmented naevi are lesions that are usually confined to skin. We report a giant congenital naevus of the scalp which involved skin, galea, full thickness cranial bone, dura and the intracranial venous sinuses. The literature on giant congenital naevi with direct involvement of underlying bone is reviewed.

Patients frequently request removal of benign papular naevi for cosmetic or functional reasons. Shave excision plus electrocautery is probably the most widely used method of removal, but this method is said to result in retained hair or pigment if deeply pigmented or hairy naevi are treated. In a prospective study, 82 benign papular naevi of all types were treated by shave excision using hot-wire electrocautery for haemostasis. Details of the naevi were accurately recorded before treatment and reassessment of shave sites carried out at 6–8 months. At review, a scar was visible at only 63% (52/82) of shave sites and all of these were cosmetically acceptable. Only 27% (15/55) of the initially pigmented naevi retained pigment and only 24% (5/21) of the initially hairy naevi regrew hair. Shave excision and electrocautery of benign naevi, including hairy and deeply pigmented ones, produce excellent cosmetic results. The patient must be warned that there is a potential risk of a scar or pigment remaining after shave excision of any naevus and for hair regrowth after shave excision of hairy naevi.

To assess whether the increase in malignant melanoma incidence could be due, at least in part, to changes in histological criteria of malignancy, pathologists in Australia, France, Italy, New Zealand, Norway, Sweden, the United Kingdom, the United States and the USSR reviewed diagnoses of 50 consecutive pigmented naevi (40 junctional and compound; 10 intradermal) and 20 consecutive malignant melanomas made in each participating centre around 1930, around 1955 and around 1980. Collaborating pathologists re-read the material, 2,665 cases in all, either from the original slide (82%) or from a recut block (17%), gave their diagnosis and indicated whether the lesion was benign (B), dubious benign (DB), dubious malignant (DM) or malignant (M). As the distribution of review diagnoses was much the same whether the original slide or one made from a recut block was read, the material was pooled. Overall, 2.8% of cases originally reported as B/DB were reviewed as DM/M, while 4.4% of the DM/M diagnoses were held to be B/DB. The shifts between categories were greatest around 1955 and least around 1980, suggesting increasing uniformity of interpretation. All available blocks were recut and sections sent to IARC for review: 1.7% (22) of 1293 B/DB diagnoses were considered to be DM/M and 3.3% (18) of 551 DM/M diagnoses were considered to have been B/DB. The consistently low frequency of shift in diagnostic category, whether the material was reviewed in the collaborating laboratories or by one pathologist at IARC, in a study designed to give maximum attention to those lesions--the junctional and compound naevi--in which a change in opinion as to malignancy would be most likely to arise, suggests that pathologists, irrespective of geographical location, are using common criteria. These findings argue against changes in histological interpretation as being responsible for more than a small portion of the continuous increase of some 3% to 8% per annum observed in malignant melanoma incidence. Other explanations, such as an increase in the frequency or potential for malignant transformation of precursor lesions, must be sought. The anatomical distribution of the malignant melanomas examined followed the usual site pattern by sex, and their thickness was observed to decrease over the period of the study in most centres.

There are two opposing schools of thought regarding the management of pigmented penile macules. Allan and Spitz, as well as Pack and Davis, are of the opinion that almost all pigmented naevi present on the palms, soles and genitalia are junctional naevi and have a higher incidence of malignant change and, therefore, should be removed. On the other hand, because most cases of penile and urethral melanomas arise de novo with only a few arising from previous long-standing naevi and as precursor lesions are not identified in most cases, Scott et al. and Stegmaire et al. consider prophylactic excision unnecessary. A more practical approach has been taken up by Barnhill et al. and their view is that management should be individualized with multiple biopsies to establish the diagnosis. The purpose of this paper is to review the literature regarding the natural history, risk of melanoma development on the penis and the management of cases presenting with pigmented penile macules.

Giant congenital pigmented naevi and neurofibromatosis (NF-1) may rarely occur together. We report an unusual case where extensive congenital melanocytic naevi were associated with neurofibroma-like lesions that were clinically and histologically confused with neurofibromatosis. The development of malignant melanomas within the pigmented and pendulous lesions representing multiple congenital melanocytic naevi highlights the importance of an accurate diagnosis and a close follow-up of such patients.

Asymmetry of pigmented skin lesions is an important indicator of possible malignant melanoma and contributes substantially to the diagnosis of melanoma in the dermatoscopic ABCD rule for melanocytic lesions and other algorithms. However, it may be observer dependent. Dermatoscopic asymmetry cannot be assessed objectively and no golden standard of asymmetry diagnosis exists. The aim of this study was to assess the sensitivity of axis (a-) symmetry using latent class analysis. We analysed ratings from four experts in dermatoscopy of 232 pigmented lesions by latent class analysis (LCA). Possible ratings were 'no asymmetry', 'asymmetry in one axis' and 'asymmetry in two axes'. A subset of melanocytic lesions (blue naevi excluded) was analysed. Based on LCA, the asymmetry of the singular lesion was determined. The sensitivity of 'no asymmetry' was 40-77%, 40-70% for one-axis asymmetry, and 77-92% for two-axes asymmetry. Overestimation of asymmetry was more common than underestimation. Melanomas were significantly more asymmetric than pigmented naevi, atypical naevi and papillomas, but not basal cell cancers. Analysis of the melanocytic subset gave similar results. The median asymmetry of malignant melanomas (1.67, interquartile range 1.81-1.99) was higher than for melanocytic naevi. In conclusion, asymmetry and symmetry are important criteria for diagnosing or excluding malignant melanoma using the dermatoscopic ABCD rule, risk stratification and other diagnostic rules. Using LCA, we minimized observer dependence in the assessment of axis (a-) symmetry. LCA, besides conceptualizing the diagnostic process, enables the assignment of lesions to their true diagnostic class.

A ring chromosome 7 was found in an 8-year-old boy investigated on account of short stature and facial dysmorphism. He had a fair skin, almost white hair, cafe-au lait spots and many black atypical pigmented naevi. Electron microscopy of his skin revealed structural changes in melanocytes and keratinocytes, suggesting disturbed cell growth and differentiation.

A variety of benign epidermal lesions may be encountered, including epidermal and sebaceous cysts, seborrhoeic keratoses, squamous papillomata and pigmented naevi.

Summary The majority of deaths from malignant melanoma could be prevented by regular screening of at-risk individuals. However, the feasibility of introducing active screening programmes depends on the reliability of criteria for identifying those individuals who are at increased risk. Several case-control studies indicate that one of the most important risk Factors is the number of benign pigmented naevi. This criterion might constitute a practical means of identifying individuals suitable for inclusion in an active screening programme. The aim of this study was to assess the reliability of using naevus counts as an indicator of risk. Two possible methods of assessment were compared: assessment by trained interviewers, and assessment by experienced dermatologists. The results suggest that this criterion may not be as practical or reliable as originally anticipated. Interviewer naevus counts correlated poorly with dermatologists' assessments (kappa =0·19). Counts by dermatologists of the number of naevi on the left arm differed by up to nine naevi, although most counts were within four naevi of each other. There was reasonable correlation between naevus counts on the left arm and those on the whole body (r= 0·71). However, the relationship was not perfect. Selection of individuals for screening solely based on the number of naevi on the left arm may exclude many people with high total body counts but relatively low left arm counts.

Summary Two cases of presumed Spitz naevus, whose diagnosis on clinical and histological grounds was uncertain, were examined for cellular DNA content using the technique of DAPI-DNA microfluoromety. They were compared with 20 cases, respectively, of clinically and histologically confirmed, Spitz, naevus, malignant melanoma and acquired pigmented naevus. The two Spitz naevi showed a diploid pattern in a distribution histogram of cellular DNA content. The pattern was similar to that of confirmed Spitz naevi and of acquired pigmented naevi but different from the aneuploid pattern of malignant melanomas. DNA index values of the two cases were within the range of confirmed Spitz naevi and different from those of malignant melanomas. The DAPI-DNA microfluorometric method thus provided confirmatory evidence for the diagnosis of Spitz naevus. The method appears to reflect sensitively the biological behaviour of tumour cells, and is a useful aid to the diagnosis of uncertain Spitz naevi.

We investigated a 4-year-old Japanese boy with oculocutaneous albinism who had a solitary pigmented mole measuring 5 mm in diameter on his back. An electron microscopic tyrosine incubation test and a DOPA reaction test clearly demonstrated the presence of tyrosinase activity in the patient's hypopigmented skin. The presence of tyrosinase activity was confirmed by tests on hair bulb samples. Histopathological evidence showed that the mole was a typical compound cellular naevus with melanin pigmentation. Although no reports to date have focused on the relationship between pigmented naevi in albinism and tyrosinase activity, our findings suggest that the occurrence of pigmented naevi in an albino may indicate the presence of tyrosinase activity.

A 13-year-old boy with a 46,XY,r(7) karyotype presented with growth failure, microcephaly, achromic spots and multiple pigmented naevi. Psychomotor development was normal and no major malformations were present. Comparison with four previously reported patients with ring chromosome 7 shows that the most frequent findings in these subjects were short stature, microcephaly and dermatological abnormalities.

A series of 577 pigmented naevi from an equal number of patients has been studied histopathologically without access to clinical information. Later the histological findings have been compared with clinical information obtained by sending a questionnaire to the patients. The correlations between the many histological and clinical variables have been studied. Patients with a red or fair hair colour, a freckled, easily sunburnt skin type and/or a poor suntanning ability have the tendency to develop irregular and atypical naevi. Histological variables like nuclear atypia, mitoses, lymphocyte reaction, fibrosis and "shoulder"-phenomenon regarding the growth pattern of naevi are correlated to this delicate skin type. These findings support til theory that irregular and atypical naevi may be potential precursors to malignant melanomas as patients with this skin type belong to the melanoma risk group.

Nine benign pigmented naevi and 16 melanocarcinomas (malignant melanomas) were studied by light and transmission electron microscopy and the fine structure of benign and malignant melanocytes is described. It was found that malignant melanocytes generally exhibit a range of abnormalities of structure of nuclei and cytoplasmic organelles which distinguish them clearly from their benign counterparts. Of the abnormalities, those affecting the specific organelle of the melanocyte, the melanosome, were the most constantly present. No firm evidence was found for the existence of two types of melanosome or two types of melanocarcinoma. Although electron microscopy is capable of showing in great detail within individual melanocytes many changes which are clearly associated with malignancy, the technique does not appear to offer significant advantages over light microscopy in determining the diagnosis and prognosis in this group of conditions.

The term "dysplastic" melanocytic naevus has recently been used to describe pigmented naevi with unusual histological and clinical features. There is currently no clear clinical or pathological definition of the term, and this has led to a lack of comparability of material described in reports on these lesions. As a result of careful histological study and a clinicopathological correlation of 100 naevi, we suggest that three distinct groups of histopathological features distinguish so called dysplastic naevi from banal melanocytic naevi. These are architectural atypia, cytological atypia, and a host response. Description of each of these features in routine reports and in published series in place of the loose use of the term "dysplastic" would enable comparisons to be made between series of melanocytic lesions reported from different centres. In the course of this study we observed a considerably increased incidence of naevus type giant cells in the dermal portion of the atypical naevi. These giant cells should not be confused with possibly premalignant cytological atypia.

SUMMARY A case of pigmented naevi arising in an albino is described. The histological appearance of a naevus described and the nature of the pigment discussed.

Summary Congenital pigmented naevi (C.P.N.) are discussed from the viewpoints of incidence, clinical and histopathological features, and development of malignancy. The management of four such naevi involving the eyelids is discussed.

We report the progress up to the age of seven years of a small for dates baby whose face and neck are strikingly devoid of subcutaneous tissue and who has, in addition, multiple pigmented naevi.

Using silver (Ag) staining to demonstrate nucleolar organizer region-associated proteins (AgNORs), pigmented naevi exhibiting features of melanocytic dysplasia have been examined and compared with benign intradermal and compound naevi and with malignant melanomas. A highly significant difference was found between the numbers of AgNORs demonstrated in benign naevus cells and atypical melanocytes and in malignant melanocytes, suggesting that this technique may have a role in differentiating between difficult melanocytic lesions.

The prevalence of dysplastic naevi was studied in a population-based survey of 380 30 to 39-year-old and 50 to 59-year-old non-Maoris in Milton, a small town in the south of New Zealand. Photographs of all pigmented naevi greater than or equal to 4 mm in diameter were taken during the survey and subsequently evaluated by two experienced reviewers. Nine percent of the group (95% CI, 6%-13%) were judged to have one or more dysplastic naevi. Interobserver agreement over the diagnosis of dysplastic naevi was 85% (kappa = 0.55). Dysplastic naevi were associated with higher counts of moles greater than or equal to 2 mm in diameter; 50 or more such moles increased the likelihood of having three or more dysplastic naevi about 40-fold. Naevi judged to be dysplastic were, therefore, relatively common and were associated with increased numbers of common moles.

Aufgrund der vorliegenden cytophotometrischen Untersuchungen wird uber den Gesamtproteingehalt (Naphthol-Yellow-Farbung) in Zellkernen ruhender Pigmentnaevi unterschiedlicher histoarchitektonischer Ausgestaltung berichtet. Ergebnisse des DNS-, Histonprotein- und Arginingehaltes am gleichen Gewebsmaterial wurden bereits fruher mitgeteilt.

The significance of the cafe-au-lait spot in the diagnosis of certain disorders of childhood is still not as widely appreciated as it should be, partly due to lack of information as to the normal incidence of such pigmentation, and partly to confusion over its relation to other forms of congenital pigmentation. The varieties of congenital melanin pigmentation of the skin consist of a number of separate entities superimposed upon the basic normal colour of the skin, depending on the racial and genetic endowment of the individual. Although these entities are clear cut and separate in form and histology, and irrespective of the basic colour of the skin, a small number may give rise to difficulty in differentiation from each other. These accessory pigmented areas include the various types of moles or melanotic naevi, mongolian spots, and caf&-au-lait spots. The various types of vitiligo also merit inclusion in this group, because there is often a distinct suggestion of hyperpigmentation at the border of the depigmented areas. Although these pigmentary changes are so universally present as to be regarded as normal, under certain circumstances they may indicate the coexistence of an underlying disease. This is particularly so with respect to the cafe-au-lait spot and, to a lesser degree, to the reverse phenomenon, vitiligo. Even in a very recent and comprehensive review of the pigment cell (Riley and Fortner, 1963), the cafe-au-lait spot has been largely ignored. Early recognition of the importance of the cafe-au-lait mark was probably obscured by the nineteenth century grouping of all congenital pigmented birth marks as 'pigmented naevi', but Marie and Bernard (1896) and Chauffard (1896) independently drew attention to the association of certain types of pigmentation of the skin with generalized neurofibromatosis of the type previously described in 1882 by von Recklinghausen. Since that time the significance of these observations has been amply confirmed and extended, but the condi

46 individuals, ascertained due to gonadal dysgenesis symptoms, were studied. 16 of them were 45, X and showed characteristics of Turner's syndrome. 15 proved to be chromosome mosaics and presented Turner's syndrome (12 cases), mixed gonadal dysgenesis (2) and gonadoblastoma (1). There were also 2 cases of pure gonadal dysgenesis and 13 patients with normal karyotypes. The clinical and genealogical data obtained from these individuals and their families were compared with 26 other series reported in the literature. Common malformations besides those related to sexual development are: short stature, abnormal nails, low implantation of hair, pigmented naevi, shield chest, short neck and cubitus valgus. Persons with 45,X karyotypes generally presented a more severe clinical picture than mosaics. The prevalence of twins is higher than expected among the patients' sibs in the cases reported here and in 3 of the 5 other series for which data are available.

SUMMARY. The frequency and regional distribution of pigmented naevi has been studied in Ugandan Africans. An average of 11 naevi was present in each adult. The association between the distribution of naevi and that of malignant melanoma is discussed and evidence is presented that pigmented skin is able to prevent the malignant transformation of naevi and the spread of already established malignant melanoma.

One hundred cases of histologically proven corneal and conjunctival tumours were extracted from the records at the Princess Alexandra Hospital and from the senior author's private practice in the last ten years. The tumours were analysed according to Duke-Elder's classification. There were 78 epithelial tumours, 15 pigmented naevi, four melanomas, two dermolipomas and one limbal dermoid. Fourteen of the 41 epithelial carcinomas were followed for three years or more. None of the 14 recurred.

The relationship between common acquired naevi and malignant melanoma of the skin was assessed in a case‐control study. In 183 patients and 183 controls matched for sex, age and area of residence, there was a strong association between the presence of pigmented naevi on the arms and melanoma. In comparison with persons having no naevi on the arms, the crude relative risk of melanoma was 28.0 when naevi were present. After adjusting for hair colour, propensity to sunburn and lifetime sun exposure, the final risk estimate was 30. 1. Family history did not appear to be a determinant of disease independent of the above risk factors.

SUMMARY Pigmented naevi (PN) were clinically diagnosed in 1083 newborn infants from a series of 531,831 consecutive livebirths examined in fifty-nine hospitals in South America. Isolated PN (IPN) were seen in 989 (0.2%) non-malformed infants, while PN associated with other congenital anomalies (APN) were observed in ninety-four (1.0%) malformed babies. Incidence rates calculated from the fifty-nine hospitals were very variable due to under-reporting, which might have been as high as 80%. The PN were classified by their location, number, size, texture, and colour. Observed locations were compared with random expected distributions based on the surface proportions of the newborn. IPN were preferentially found on the chest and abdomen, with a low concentration on the head and upper limbs. No examples were observed on soles or palms. IPN were single in 90% of cases. Size was small (1–9 mm) in 29%, medium (10–;40 mm) in 63%, and large (over 40 mm) in 8% of cases. Abnormal texture, (rugose, raised, and/or hairy) was found in 7–11% of IPN. Colour was brown in 68%, black in 21%, and of other colours in 11%. The large IPN tended to be multiple in number and abnormal in texture. Small IPN tended to be black. IPN on the chest and abdomen were more frequently small, non-black, and of normal texture; those found on the back tended to be black and abnormal in texture; black IPN were also frequent on the upper limbs; large IPN frequently affected the lower limbs. The APN did not differ from the IPN in their number, size, texture, or colour. APN were less common than expected on the back and upper limbs. An association was observed between the PN and the following four congenital anomalies: ear deformities, preauricular appendages, angiomas, and other skin anomalies. The differences in distribution of PN in the newborn infant and that in the adult that have been previously published suggest that most of the PN found in the adult are not congenital.

The giant pigmented naevus is fortunately an uncommon congenital malformation, but when present it often brings tragedy to both the bearer and his family. In 1939, Conway recorded 40 cases, and in 1959 Russell and Reyes reviewed 53 documented cases in the world literature. In 1965, Greeley and his colleagues reported 56 cases treated over a 25-year period. In a 60-year period, 1915–1975, 51 patients were registered with the Danish Health System. In the 20- year period from 1958 to 1978, the author treated 26 cases of giant hairy pigmented naevi.

A family is reported in which multiple pigmented naevi are associated with late-onset sensorineural deafness. It is hypothesized that this association is an autosomal dominant syndrome.

SUMMARY The chance of malignancy developing in a giant pigmented naevus is often regarded as sufficiently high to justify prophylactic removal of all giant pigmented naevi as soon as possible, but most of the reports from which these figures are derived appear biased because of selection of patients. Because malignant melanoma seemed extremely rare in the Australian prepubertal child, attempts were made to document its frequency. Despite difficulty in obtaining complete figures, sufficient information was obtained to confirm the extreme rarity of malignant melanoma in general and especially arising in a giant pigmented naevus before puberty. It is therefore suggested that there is no general indication for prophylactic removal of giant pigmented naevi in Australian prepubertal children. Indications for removal on cosmetic grounds are discussed. It is speculated whether these conclusions might apply in other countries.

SUMMARY.— The pedigrees of 3 patients with giant pigmented hairy naevus (GPHN) were investigated. The occurrence of multiple small pigmented naevi in members of the patients' families is reported and it is suggested that at least some cases of GPHN may be determined by an autosomal dominant gene of variable expressivity.