1/15. Acrochordons as a presenting sign of nevoid basal cell carcinoma syndrome.BACKGROUND: Nevoid basal cell carcinoma syndrome (NBCCS) is a genodermatosis with autosomal dominant inheritance. In identified kindreds the diagnosis is relatively easy, but for the patients without family history of this syndrome a high clinical suspicion is necessary for diagnosis. OBJECTIVE: Acrochordons are distinctly uncommon in childhood. Our purpose was to evaluate skin tags that develop at an early age. methods: This is a retrospective series evaluation of 7 children who presented with pedunculated papules (acrochordon-like growths). A full history was then correlated with biopsy results in each patient. RESULTS: Clinically, lesions consisted of flesh-colored and pigmented pedunculated papules. Histopathologic examination of these papules showed basal cell carcinomas in each biopsy specimen. CONCLUSION: We consider that "skin tag"-like basal cell carcinomas in childhood may represent a marker for NBCCS. early diagnosis of this syndrome and early sun protection of the affected children could help decrease the number of lifetime tumors. biopsy should be performed on acrochordons in children because they may be the presenting sign of NBCCS. Because these tags may precede other stigmata of the NBCCS, recognition may facilitate early diagnosis and allow early treatment and sun protection.- - - - - - - - - - ranking = 1keywords = pigmented (Clic here for more details about this article) |
2/15. Multiple synchronous pigmented basal cell carcinomas following radiotherapy for Hodgkin's disease.BACKGROUND: Multiple basal cell carcinomas (BCCs) are infrequently seen in patients under 30 years of age. Their occurrence at a young age is often linked to some genodermatosis, including Nevoid Basal Cell carcinoma Syndrome (NBCCS). The exposure to ionizing radiation is also considered to be a predisposing factor in the development of BCCs. methods: We report the case of a 35-year-old patient who presented with seven synchronous, nodular, brownish-pigmented BCCs, confined within the radiation-treated cutaneous areas, 15 years after receiving cobalt-60 (60Co) irradiation for Hodgkin's disease. RESULTS: On the basis of clinical, radiological, and anamnestic data we excluded a NBCCS, thus proposing irradiation as the cause of the multiple synchronous pigmented BCCs. CONCLUSIONS: Previous therapeutic ionizing radiation leads to an increased risk of BCCs confined to the region of the body to which radiotherapy was delivered. We consider our patient's BCCs represents a late adverse effect of the treatment with cobalt-60.- - - - - - - - - - ranking = 6keywords = pigmented (Clic here for more details about this article) |
3/15. Nevoid basal cell carcinoma syndrome in a person with dark skin.The expression of basal cell carcinoma tumors of the skin is blunted in individuals with dark skin and nevoid basal cell carcinoma syndrome. The occurrence of multiple basal cell carcinomas in these patients is a relatively rare finding. We describe a 25-year-old man of partial African-American descent with constitutive Fitzpatrick type IV pigmented skin and the clinical stigmata of nevoid basal cell carcinoma syndrome including histopathologic evidence of 11 basal cell carcinomas.- - - - - - - - - - ranking = 1keywords = pigmented (Clic here for more details about this article) |
4/15. Early recognition of basal cell naevus syndrome.The basal cell naevus syndrome is an autosomal dominant syndrome characterised by major manifestations such as basal cell carcinomas, jaw cysts, palmar or plantar pits, and intracranial calcifications. Early recognition is important in order to reduce morbidity due to cutaneous and cerebral malignancy and oromaxillofacial deformation and destruction, although diagnosis in infancy is rare. We describe three unrelated children with basal cell naevus syndrome who appeared to be the first patient in each family. Conclusion: Our observations lead us to recommend looking for other manifestations of this disease in patients who present with cardiac fibroma, cleft lip/palate, polydactyly or macrocephaly. Bifid, fused or splayed ribs should be considered a major criterion of great help in establishing a diagnosis, particularly in young children.- - - - - - - - - - ranking = 8153.2862343517keywords = naevus (Clic here for more details about this article) |
5/15. Odontogenic keratocyst in an infant.Odontogenic keratocysts occur as solitary lesions or in association with the basal cell naevus syndrome (BCNS), and have been documented in the age range 5 to 83 years. A rare case of an odontogenic keratocyst in a one-year-old girl is presented. Its characteristic tendency to recur, and its association with BCNS present important clinical implications.- - - - - - - - - - ranking = 1358.8810390586keywords = naevus (Clic here for more details about this article) |
6/15. Nevoid basal cell carcinoma syndrome (Gorlin's syndrome): a case report.A case of nevoid basal cell carcinoma syndrome is presented and its varied clinical manifestations and multi-system involvement are emphasised. Our case presented with an early onset of symptoms but sought medical help later on for progressively increasing jaw swelling and pain. On further evaluation, multiple pigmented skin papules, palmar pits, multiple jaw cysts, skull bone osteoporosis, bifid ribs and kyphosis were present. Systemic involvement was minimal. There was no significant family history.- - - - - - - - - - ranking = 1keywords = pigmented (Clic here for more details about this article) |
7/15. Nevoid basal cell carcinoma syndrome. A review and case report of a patient with unilateral basal cell nevus syndrome.An exceptionally rare example of nevoid basal cell carcinoma syndrome occurring unilaterally is presented, along with a review of the syndrome. We ruled out the possible association of this case with the linear unilateral basal cell nevus with comedones because of several incongruities, including the absence of epidermal cysts and strialike atrophy and the presence of pigmented basal cell carcinomas. Linear unilateral basal cell nevus with comedones is not a variant of the nevoid basal cell carcinoma syndrome and the tumors follow a benign course. The aggressive, destructive behavior of numerous pigmented basal cell carcinomas that involve the eyelid and medial canthus of the eye and the head and neck area represents important evidence that our patient's condition was not an example of linear unilateral basal cell nevus with comedones.- - - - - - - - - - ranking = 2keywords = pigmented (Clic here for more details about this article) |
8/15. Ocular findings in linear sebaceous naevus syndrome.The case of a 5-month-old black female child with a linear sebaceous naevus syndrome and multiple congenital anomalies is presented. Ocular malformations consisted of colobomatous changes of the lid and retina, dermoid of the conjunctiva, chorioretinal changes, and peripapillary atrophy of the optic nerve. Systemic findings included midline cleft of the secondary palate with involvement by the naevus, bilateral hearing loss, asymmetrical skull bones, ventricular septal defect, epidermal inclusion cyst, and developmental delay without seizures.- - - - - - - - - - ranking = 8153.2862343517keywords = naevus (Clic here for more details about this article) |
9/15. Multiple metastases from basal cell naevus syndrome.A patient with basal cell naevus syndrome (Gorlin-Goltz Syndrome) is presented. Of note in the case was the extensive symmetrical tumour invasion of both external auditory canals requiring bilateral radical resection. The patient expired 14 months later, at which time the autopsy revealed widespread metastases to the pleura, diaphragm, pericardium, epicardium and myocardium. Although lung metastases have been reported in this syndrome, no cases have been reported of metastases to these sites.- - - - - - - - - - ranking = 6794.4051952931keywords = naevus (Clic here for more details about this article) |
10/15. adult form of basal cell naevus syndrome: a family study.A 32-year-old patient had marked reduction of visual acuity due to falciform folds of the retina and retinal detachment, and severe neurological abnormality: bilateral pyramidal involvement, fasciculation in all limbs and gait ataxia. skull radiographs showed internal frontal hyperostosis; CT scan showed calcification of the falx cerebri, and multiple arachnoid cysts were shown by myelography. A naevoid lesion had previously been removed from the left forearm. There was a history of ophthalmological symptoms in the mother and the daughter of the propositus. His son has "cafe au lait" spot on the abdomen and dentigerous cysts. The diagnosis of an adult form of basal cell naevus syndrome with an autosomal dominant mode of inheritance is discussed.- - - - - - - - - - ranking = 6794.4051952931keywords = naevus (Clic here for more details about this article) |
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