Tuberous sclerosis is a phacomatosis, namely an inherited disorder involving the skin and the nervous aystem. Besides the classic forms with typical skin lesions (congenital hypomelanic macules, angiofibromas, Koenen tumors, shagreen patches) and characteristic neurological lesions (seizures), do exist minimal forms, which can present only cutaneous lesions, mainly angiofibromas. In the past, the latter were called sebaceous adenomas, mainly due to their distribution around the nose, which is a region particularly rich in sebaceous glands. In some cases angiofibromas of tuberous sclerosis should be differentiated from inflammatory acne.
Histiocytosis is the most frequent infantile proliferation after mastocytosis. Histiocytosis is due to the proliferation of the histiocyte. From the undifferentiated histiocyte derive two more differentiated cells, namely the monocyte-macrophage and the Langerhans cell. This is why there are two types of histiocytosis, namely non-Langerhans histiocytosis, which is due to the proliferation of monocytes-macrophages, and Langerhans cell histiocytosis. Similarly to mastocytosis and all the proliferations of the first months of life, the clinical spectrum and prognosis of histiocytosis is very wide, ranging from an isolated lesion of the skin or other organs to the generalized involvement of the skin, till the multisystem involvement of the organism. The prognosis is also extremely variable, ranging from the spontaneous regression within months or years to the death in a few weeks. The biological significance of histiocytosis is under discussion. According to some authors, histiocytosis is a self-healing, reactive, hyperplastic proliferation, whereas other authors believe that it is a malignant, potentially lethal neoplastic process. We are treating only the most frequent forms in childhood of this extremely complex chapter, namely juvenile xanthogranuloma and cephalic histiocytosis among the non Langerhans histiocytoses and finally Langerhans cell histiocytosis.
Here is reported a strange case of hyperkeratosis of the nail, arisen immediately after birth according to the mother, in a 5-year-old boy. The differential diagnosis, the recent classification of congenital pachyonichia and the new diagnostic criteria required for its diagnosis are discussed. However, the surprising end of the story, although happy for the child health, does not favor our diagnostic ability.
Lymphomas are malignant tumors deriving from the proliferation of cells setting up the immune system. As these cells normally circulate with the blood in the whole organism, lymphomas are generalized tumors from their appearance. However, exceptions do exist leading the Authors of the European Organization for Research and Treatment of Cancer (EORTC) to say that morphologically identical lymphomas, when arising in different organs, are clonal proliferations of distinct lymphocyte subpopulations, which are in some way related to the organ where the lymphoma arose (45). Lymphomas are much less frequent in children as compared with adults. However, some Authors (5) established that 1/6,000 children under the age of 15 develops lymphoma. According to these Authors, the frequency of lymphomas follows only that of leukemias and brain tumors. (...)
During the Congress of the European Academy of Dermatology and Vene-reology, which was held in Geneva, from 11 to 15 October, 2000, many communications and posters were devoted to antimycotic treatment. Here these reports are summarized, giving particular emphasis to the problems of the pediatric age. We will essentially talk of the treatment of dermatophytoses, namely of the mycoses caused by the genus Dermatophytes. Then we well mention the treatment of pityriasis versicolor, candidiasis and sporotrichosis. Anyway, we will start with a review of the main systemic and topical therapeutical agents.
The number and distribution of melanocytic nevi at the age of ten were investigated in 244 students. The latter were chosen at random in 15 classes of the primary school of Bari (Italy). Besides the age and sex, in these subjects were investigated the presence of ephelides, nonmelanocytic nevi, the total number of melanocytic nevi, the number of such lesions for each site -head, trunk, upper and lower limbs- and the site of the largest nevus. Ephelides were found in 49 subjects (19%). Among the nonmelanocytic nevi, hyperpigmented nevus was the most frequent (19 cases -8%-). 66% of students showed a total number of melanocytic nevi higher than 10. This percentage was 72 in subjects with ephelides. The highest concentration of melanocytic nevi was on the head, whereas the lowest was on the lower limbs. No difference was shown between sexes with regard to the number and distribution of melanocytic nevi.
Vitiligo and hypopigmented nevus are responsible for white cutaneous patches. The latter can appear in the first years of life. Vitiligo is an acquired disease. It is characterized by an autoimmune disorder leading to the destruction of the melanocytes. On the other hand, hypopigmented nevus is a malformation disorder occurring during the fetal life. It probably consists of a defective transport of melanin inside the keratinocyte. Hypopigmented nevus often affects only a cutaneous segment. Hypopigmented nevus should be differentiated from segmental vitiligo, which is a clinical variant of vitiligo, more frequent in children than in adults. Due to significantly different clinical course, the differential diagnosis between the two disorders is important. Hypopigmented nevus is less white and thus less visible of vitiligo. Moreover, it persists unchanged with time. On the other hand, segmental vitiligo is an evolutive disease and thus may worsen or, more often in children, improve and almost completely regress. The differential diagnosis between the two disorders also leads to a different therapeutical approach.
Leukemia is the most frequent malignancy of children, being responsible for 30% of all malignant tumors at this age (35). Although rarely, the skin can be affected in case of leukemia. The cutaneous lesions are usally not specific, but sometimes specific, namely due to leukemic cells infiltrating the dermis and subcutaneous fat. Neuroblastoma (NB) arises from primary multipotent sympathetic cells deriving from the neural crests. Therefore, NB is able to secrete hormones. NB is the most common extracranial solid tumor in children, being fourth in frequency after leukemia, lymphomas and tumors of the central nervous system. It accounts for about 9% of all childhood cancers and its prevalence is about 1 case per 8000 live births (45). NB is rare after the first decade of age and in 50% of cases is diagnosed before the age of two. Therefore, NB is a typical tumor of childhood. NB is here treated because cutaneous metastases, which are not always associated with a severe prognosis, can be the presenting sign of the disease. This is why pediatric dermatologists should be aware of these lesions.
Pediatric dermatology is prevalently a medical discipline. Therefore, with regard to the pain, the most frequent problem is how visiting the child without causing a trauma to him/her, to his/her family, to the other children, who stay in the waiting room, and, finally to the physician. In this case we are dealing with a psychological problem, which can be overcome in most cases, if the physician knows the child world and its fears, namely if the physician works daily with children. However, in some situations physicians should face the problem of the pain, namely of that unpleasant sensation, connected with actual or potential tissue damage and influenced by personal experiences of the first years of life (21). This subjective component is different in the various subjects and thus is responsible for a different, visible or audible, behavior reaction, to the tissue damage. The latter in Paediatric Dermatology is usually related to the skin laceration and to the stimulation of its nervous pain receptors. (...).
In a study aimed at monitoring the cutaneous lesions of subjects with epidermolysis bullosa (EB), in order to precociously diagnose skin cancer, we investigated the distribution of melanocytic nevi in 13 subjects with epidermolysis bullosa dystrophicans recessiva (EBDR). Six subjects with EBDR presented peculiar melanocytic nevi. The latter were acquired, but large sized, eruptive, punctiform and localized on previous blisters. These nevi, which have been reported in other bullous disorders, are not expression of an increased trend to produce nevi in subjects with EBDR. In fact, the latter present a decreased number of melanocytic nevi as compared with normal controls.