Congenital Diffuse Mottling of the Skin (original) (raw)

Dermatologic Diseases Presenting with Pigmentation Disorders in Children: A Single Center Experience

Journal of Pediatric Sciences (ISSN:1309-1247), 2014

Background: To determine the incidence and demographic characteristics of skin diseases presenting with hyperpigmentation in children applying to the dermatology department. Methods: A total of 2815 children between the ages of 0 to 16 who applied to the dermatology clinic with the complaints of hyperpigmentation disorders were evaluated. The age, gender, socioeconomic status, place of residence and demographic characteristics of children with abnormal pigmentation skin lesions were investigated. Results: A total of 2815 children applied to the dermatology clinic during the study period. Of these patients 1491 were female (53%) and 1324 were male (47%). Of these 266 (9.4%) were diagnosed with skin disorders presenting with hyperpigmention. The causes of hyperpigmentation in these patients were was follows; pityriasis alba (2.6%), melanocytic nevus (2.1%), vitiligo (1.8%) postinflammatory hyperpigmentation (0.5%), and postinflammatory hypopigmentation (0.5%). According to the gender, p.alba, vitiligo, Becker nevus, acanthosis nigricans, tuberosclerosis and albinism were seen more in boys and nevus depigmentosus ephelis, postinflammatory hyperpigmentation/hypopigmentation and melanocytic nevus were seen more in the girls. Pitriyazis versicolor was seen equally in both genders. According to age groups, melanocytis nevus were found to be more frequent between the ages of 0-2 and 12-16, whereas pityrsasis versicolor was more frequent in ages 12-16 and P. alba in the 3-11 age group. Conclusions: There are quite a substantial number of pigmentation diseases occurring in children. Early diagnosis and treatment are important because although these diseases mainly cause cosmetic problems, they can decrease the quality of life. Here, we attempted to define the demographic characteristics of diseases presenting with disorders in pigmentation in children.

Progressive Hyperpigmentation and Generalized Lentiginosis without Associated Systemic Symptoms: a Rare Hereditary Pigmentation Disorder in South-East Germany

Acta Dermato-Venereologica, 2003

Familial progressive hyperpigmentation is rarely described in the literature. We report on five patients from three different families presenting with a peculiar progressive pigmentary disorder. The patients show a progressive diffuse, partly blotchy, hyperpigmentation, intermixed with scattered small hypopigmented macules, a few large hypopigmented areas, occasional café-au-lait spots and, most remarkably, a generalized lentiginosis. Histology revealed different degrees of basal layer hyperpigmentation and pigment incontinence, also in the spots appearing hypopigmented. Ultrastructural analysis showed a normal mode of Caucasian-like melanogenesis with varying content of regular melanosome complexes within the keratinocytes. All families are clustered in a small area around the town of Teublitz in south-east Germany with about 20,000 inhabitants, suggesting a genetic founder effect. Pedigree analysis is compatible with an autosomal dominant mode of inheritance with variable penetrance. Only a few similar, but not identical, cases have been reported in the past. This cluster of cases may therefore represent a rare and perhaps novel variant of a familial progressive disorder of hyperpigmentation.

Epidemiology of congenital pigmented naevi: I. Incidence rates and relative frequencies

British Journal of Dermatology, 1981

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 (02%) non-malformed infants, while PN associated with other congenital anomalies (APN) were observed in ninety-four (i'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.