[Amelanotic spitzoid melanoma of the digit: a case report]

Melanomas of digit is rare, accounting for 1% of all cutaneous melanomas. We report a new case. Our purpose is to discuss the clinicopathological characteristics and the difficulties encountred in establishing diagnosis of this rare tumor. We report the case of a 25 years old woman, who consulted for nodular and ulcerated lesion of the right index, located in the external face of the metacrapo-phalangial joint. The nodule was biopsied and histopathologic exam concluded to spitzoid melanoma. The surgical margins were involved. The patient refused surgical recovery. She consulted 3 years later with axillary lymph nodes. A wide excision of the tumor with lymph node biopsy were made. Histological study concluded to a tumoral residu incompletely excided with lymph node metastases. Amputation of the second digit with dissection of the axillary lymph nodes was made. The surgical margins were tumor free. Lung metastases appeared with a follow up of two months. The patient died early after starting chemotherapy with Deticen. In our report, clinical presentation was misleading causing a diagnosis and therapeutic delay. Pathologically, all the histological types of melanoma were described in the digit except spitzoid melanoma

[Unusual thyroid lesion]

The incidence of aspergillosis has increased during the past decade with the widespread use of aggressive chemotherapy and immunosuppressive agents. The lungs are the most common primary site of infection. Thyroid involvement is rare, usually asymptomatic, and difficult to make an ante-mortem diagnosis. Our aim is to discuss the epidemiological characteristics, the diagnosis and the histogenesis of this unusual thyroid lesion. Case report A 13-year-old girl, with a past history of lung disease was hospitalised for cervical pseudo-tumoral lesion. Cervical scintigraphy was taken and showed abnormal uptake in the thyroid. A resected specimen of the thyroid showed an extensive abscess with diffuse aspergillus hyphae infiltration. Treatment with amphotericine B was initiated and the evolution was unfavorable. Aspergillus infection frequently disseminates to a variety of organs, thyroid involvement is rare and its prognosis depends on early diagnosis and prompt initiation of therapeutic measures

progressive family intrahepatic cholestasis difficulty of diagnosis and taking charge in a developing country

The progressive family intrahepatic cholestasis or Byler syndrome is a group of recessive autosomic illness. It is responsible of an intra-hepatic cholestate which is secondary to an anomaly of the metabolism and the excretion of biliareses acid .In spite of progress achieved over the last few years in the understanding of the physio-pathological mechanisms and the genetics of this group of cholestasis, the hold in charge remains difficult in a developing country like Tunisia. Patient and methods: it is about a retrospective survey concerning 12 children treated in the paediatric service during a period of 18 years [1986-2003] for progressive family intrahepatic cholestate confirmed by clinical, biological and histological data. our set includes 12 children who have presented a cholestatic jaundice with dark urine and discoloured waste beginning between the ages of 3 days and 18 months with an average age of 4 months, the parental inbreeding has been noted in 10 patients, the domestic antecedents of jaundice of the infant were found in 5 cases the jaundice was in irregular all cases and partner to a prurits in 8 cases. The hyperbilirubinemy and the increase of the alkaline phosphatases were present with all patients. The rate of cholesterol was normal with all patients. The gamma GT was normal in 10 cases and increased in 2 cases, biliary acids have been measured in 10 patients, they were raised in all cases. The hepatic biopsy has been achieved in 10 cases; it showed signs of cholestase and fibrosis in all cases. Eight patients received a treatment associating acidic ursodesoxycolic [AUDC] and Rifampicin, whereas 4 patients have been treated with cholesteramin in the absence of hold social. The K vitamin by way parenterale has been prescribed for all children, whereas vitamins A and E, not available in Tunisia are taken only by 2 patients. The evolution has been made toward the death in a graph of decomposed cirrhosis in 3 patient [age ranging from 1 month to 4 years]. The other patients are treated again with thrusts of jaundice and pruritus but with no sign of serious hepato-cellular insufficiency. A lot of difficulties persist at the level the diagnosis of the PFIC in Tunisia [biliary acid dosage abroad] and especially at the therapeutic level: unavailability of fat-soluble vitamin and absence of a hepatic transplantation program