Persistent hepatic venous plexus in a patient with Sturge-Weber syndrome.

A patient with extensive facial and intracranial features of Sturge-Weber syndrome was found to have a persistent primitive hepatic venous plexus, characterized by three additional vessels arising from the inferior vena cava, circulating between liver segments, which formed a common trunk in the supra-hepatic region that flowed into the right atrium. To the best of our knowledge, this hepatic finding has not been previously described in association with Sturge-Weber syndrome.

[Skin diseases and hepatitis virus C infection]. / Oboljenja koze i infekcija virusom hepatitisa C.

INTRODUCTION: Using a creative molecularbiologic technique in 1989 Choo and co-workers isolated a new virus of hepatitis C, an agent responsible for numerous cases of parenterally transmissible viral hepatitis. Hepatitis C virus is a RNA virus with unique genomic organisation. Six genotypes of hepatitis C virus have been identified, which differ in geographical distribution, tendency towards chronicity and sensitivity to antiviral therapy. Transmission occurs due to apparent and inapparent parenteral procedures (after transfusion, transplantation, transplacentally, during lactation, sexually or after accidental injury of medical staff). Mode of transmission through intact skin or mucosa has not been proved yet. Due to development of laboratory methods for detection of anti-hepatitis C virus antibodies and obligation for routine testing of blood donors for hepatitis C in majority of countries in the world, risk of post-transfusion hepatitis C is minimised to less than 1%. In 70% of patients the infection runs a chronic course, affecting numerous extrahepatic organ systems, including skin. VASCULITIS ASSOCIATED WITH MIXED ESSENTIAL CRYOGLOBULINEMIA: Mixed essential cryoglobulinemia is a disorder with deposition of circulating immune complexes in small and medium blood vessels. Clinical characteristics comprise palpable purpura on lower extremities, arthralgias and weakness. It might occur during autoimmune disorders, liver diseases and viral infections, among which hepatitis C infection has a central part. Mixed cryoglobulins can be detected in 35-54% of patients with hepatitis C and symptomatic vasculitis associated cryoglobulinemia, decreased C4 component of complement, positive rheumatoid factor and elevation of hepatic enzymes occurs in 10-21% of patients. Findings of anti-hepatic C virus antibodies and/or viral RNA in 96% of patients with mixed cryoglobulinemia can be considered as a definitive proof of etiopathogenetic association between hepatitis C infection and mixed cryoglobulinemia. Interferon alpha therapy is a first-choice therapy, although transient responses are frequent. PORPHYRIA CUTANEA TARDA: Hepatitis C infection has recently been recognised as an important precipitating factor of clinical porphyria cutanea tarda sympotomatology. Apart from high level of seropositivity among porphyria cutanea tarda patients (62-100%), association between these two entities hasn't been clearly revealed yet. The question whether hepatitis C infection is enough to be the only precipitating factor, or other hepatotoxic cofactors are necessary, still exits. Interferon therapy has been a meter of several studies, but no definite recommendations had been given about its administration in these cases. LICHEN PLANUS: About possible association between lichen planus and chronic liver diseases, and hepatitis C infection as well, suggest 35% prevalence of hepatic disorders in patients with lichen planus, and 9.8-23% of hepatitis C virus seropositivity. The clinical picture of lichen planus in hepatitis C virus infection is characterised by generalised skin lesions, with erosive involvement of oral mucous membrane, and by chronic course. Therapeutical efficiency of interferon is unpredictable, with possible improvement, cure or deterioration of lichen planus. OTHER DERMATOLOGICAL DISORDERS: Other dermatological disorders (erythema nodosum, erythema multiforme, urticaria) may be direct consequences of hepatitis C infection, of other extrahepaic non-dermatological manifestations, or fortuitous reports. CONCLUSION: Considering big pathogenetic potentials of hepatitis C virus with possible skin involvement, and proved association between cutaneous necrotising vasculitis with mixed essential cryoglobulinemia, porphyria cutanea tarda, lichen planus and chronic hepatitis C infection, all patients with these disorders should be tested for hepatitis C and all cases of hepatitis C should be searched for signs and symptoms of these skin diseases.

[Persistent dyschromic erythema]. / Perzistentni dishromicni eritem.

INTRODUCTION: Erythema dyschromicum perstans (ashy dermatosis) is a very rare skin disease included in the group of acquired, idiopathic hypermelanosis, with development of blue-gray macules. This disease appears more frequently in dark coloured persons, especially women in the first and second life decade. CASE REPORT: A male patient, 42 years of age, was admitted to Clinic of Dermatovenereology in Novi Sad due to appearance of slightly pruriginous, brown-reddish macules on the trunk, upper and lower extremities, without affecting the skin of the face, scalp, palm soles and visible mucous membranes. Later, the color of the macules changed into blue-gray and new lesions appeared in axilla and flexor side of the big joints, with active, erythematous and thin raised borders. Laboratory findings showed no abnormalities; antinuclear antibodies were negative. Histopathological examination of the skin specimens (which were taken from two different places) showed vacuolar degeneration of the basal cell layer, numerous pigmentophages in papillary dermis and presence of lymphohistiocytic infiltrate in dermis. No history of drug intake or exposure to UV light was established. DISCUSSION: Ashy dermatosis is included in the group of hypermelanosis of unknown origin. As possible etiological factors we can mention ingestion of ammonium nitrate, environmental pollution, hypersensitivity to cobalt chloride and postinflammatory pigmentation. Clinical characteristics: occurrence of blue-gray and gray macules on the trunk, face, neck and extremities (absence on the palms, soles, visible mucous membranes, scalp and nails). In the active phase of the disease, these macules are surrounded by erythematous and thin, raised borders. The lesions are mostly permanent. Due to clinical, histopathological, immunofluorescent and electron microscopy established similarities with lichen planus, it is considered that ashy dermatosis is a variant of lichen planus. Absence of previous drug intake, exposure to UV light, absence of the antinuclear antibodies, clinical picture and histological findings confirm the diagnosis of erythema dyschromicum perstans. CONCLUSION: This case of ashy dermatosis shows that there is a need for differential diagnosis of acquired skin pigmentations, because this dermatosis must also be taken into consideration.

Surgical treatment of craniopharyngiomas in children.

This paper sets out the surgical difficulties and the results of treatment in 50 cases of craniopharyngiomas in children up to the age of 13. During surgery, 10 patients were in a sub-comatose state, while 4 had respiratory arrest. Two-thirds of the children under 10 were given an A-V shunt implant. Radical excisions were attempted in 20 cases but were executed in only 13. Three of these died. Separation of the upper and posterior part of the capsule from the hypothalamus had to be interrupted in seven cases. The object of the operation was the radical removal of the capsule. We failed in one-third of our cases. Tumour excision is always attempted unilaterally. When this proves impossible, a second operation is undertaken on the opposite side when the hypothalamic disturbance subsides. If repeated surgery is indicated, it is always performed on the opposite side of the primary operation.

Surgical treatment of craniopharyngiomas in adults.

Thirty-eight patients over the age of 21 were found to be suffering from craniopharyngiomas. According to the clinical symptoms, 8 tumours were of the juvenile type and 24 of the adult type. Thirty-four patients underwent direct surgery of the tumour. Ten were operated on unilaterally and then contra-laterally, as we were unable to remove the tumour in one go. Fifteen of the 24 adult type tumours were operated on radically, and five subtotally. Four patients were given an A-V shunt implant. The best results were obtained with subtotal excisions of adult suprasellar craniopharyngiomas.