18F-DOPA PET/CT and MRI Findings in a Patient With Multiple Meningiomas.

A 56-year-old man was referred to our Department for a 2-year story of upper limb tremor, severe headache, and episodes of confusion. Brain F-DOPA PET/CT showed multiple areas of tracer uptake in the two hemispheres of the brain. Subsequent MRI displayed demyelinating foci with high contrast enhancement. Histological specimen confirmed the diagnosis of multiple meningiomas.

Folliculotropic mycosis fungoides: clinicopathological analysis of 17 patients.

BACKGROUND: Folliculotropic mycosis fungoides (FMF) represents a variant of MF characterized by hair follicle invasion of mature, CD4-positive small lymphoid cells with cerebriform nuclei. The disease displays resistance to standard treatment modalities and has an unfavourable course. OBJECTIVE: Clinical analysis of 17 patients with FMF collected between 2005 and 2012, investigation of tumour cells and involved hair follicle. METHODS: Re-evaluation of clinical data, wide panel immunohistochemistry investigation on paraffin-embedded biopsy material, T-cell receptor gene rearrangement analysis of the samples. RESULTS: Male and older age group predominance, frequent head-neck involvement, acneiform lesions, keratotic plugs, cysts, nodules, follicular papules, alopecia and classic mycosis fungoides-like plaques represented the main clinical characteristics. Treatment response showed a wide range from transient complete response to therapy resistance and death due to the disease. The pathological alterations: folliculotropism, mild epidermotropism, follicular plugging, mucinous degeneration of hair follicle, basaloid hyperplasia, syringotropism were similar to those observed previously. The first case of a CD8-positive folliculotropic mycosis fungoides - with unusual clinical presentation - is reported here. Nestin overexpression of mesenchymal cells of the isthmic and suprabulbar regions of hair follicle and the reappearance of dermal nestin-expressing cells were observed in association with immature dendritic cell hyperplasia. Altered CK19 expression was detected suggesting a potential role of follicular keratinocytes in the disease process. It was found that a proportion of neoplastic T cells constantly express programmed death-1 receptor in our patients contrary to classic mycosis fungoides. CONCLUSION: The spectrum of the clinical manifestation and the course of folliculotropic mycosis fungoides are broad and differ from classic mycosis fungoides. Folliculotropic neoplastic T-cell proliferation is associated with activation of inflammatory reactive T- and B-lymphoid cells, mesenchymal cells and changes in the hair follicle.

[Dermatologic aspects of SAPHO-syndrome]. / A SAPHO-syndroma bórgyógyászati vonatkozásai.

SAPHO syndrome (synovitis, acne, pustulosis, hyperostosis, osteitis) as a new disease entity was first described in 1987. The syndrome is characterized by the presence of pustular dermatoses together with aseptic osteoarticular lesions. The bone involvement includes hyperostosis, aseptic osteomyelitis or arthritis of the anterior chest wall, sacroiliac joints or long bones. Skin diseases include acne conglobata or acne fulminans, palmoplantar pustulosis and hidradenitis suppurativa. Authors describe the dermatological relationship of SAPHO syndrome reporting their 7 cases (3 acne fulminans, 4 palmoplantar pustulosis). Authors draw attention to the isotretinoin therapy as a possible provoking factor of the articular symptoms, and they emphasize the diagnostic role of bone scintigraphy.

[New aspects in the classification of cutaneous lymphomas]. / Szemléletváltozás a kután lymphomák klasszifikációjában.

Authors discuss the classification of primary cutaneous lymphomas created by the Cutaneous Lymphoma Study Group of the European Organization for Research and Treatment of Cancer (EORTC) in 1996, which is based on the clinical, histological, immunohistochemical and genetic features of cutaneous lymphomas. Unlike the previous histologic classifications it contains well-defined disease entities characterized by their clinical and histological picture, clinical outcome, behaviour and therapeutic response. This classification does not use the term of low grade or high grade lymphoma, but introduces the indolent, aggressive and provisional subgroups in the T-cell lymphomas, and indolent, intermediate and provisional subgroups in the B-cell group. Authors demonstrate the EORTC classification by their own cases calling the attention to the clinical and therapeutic difference between nodal and extranodal lymphomas, and discuss the up-to-date therapeutic possibilities.

[Angioneurotic edema induced by angiotensin converting enzyme inhibitors]. / Angiotenzinkonvertáló enzim inhibitorok okozta Quincke-oedema.

Angioneurotic oedema is one of rare side effects of angiotensin converting enzyme inhibitors, its incidence is around 0.1-0.2%. Angio-oedema most commonly develops in the first 4 weeks of the treatment, but it can be observed later, after several months or even years. The association between the oedema and the drug intake can be difficult to recognize if the oedema is of delayed type and because the attacks can disappear spontaneously without discontinuation of the drug. The angioneurotic oedema is tend to be worsening during the treatment, and finally the obstruction of the upper respiratory tract can be fatal. The affected sites are the face, lips, tongue, upper respiratory tract, and the oedema can also develop in the gastrointestinal tract with abdominal pain and diarrhea, which can be misdiagnosed. The pathomechanism is thought to be rather biochemical than immunological. The pathogenetic factors are under investigation nowadays, but the increased level of bradykinin seems to be the most important factor. Authors treated 248 patients with angioneurotic oedema in the Department of Dermatology (Semmelweis Hospital, Miskolc) between January of 1997 and December of 2000, 44 patients took angiotensin converting enzyme inhibitors, and 16 patients were suspected as suffering from angio-oedema induced by this drug. All of the patients remained symptom-free after the adequate treatment and discontinuation of the suspected drug. Authors describe the clinical picture of the angio-oedema, the risk factors, and the contraindications of the angiotensin converting enzyme inhibitor treatment.

[Cutaneous and mucosal manifestations of inflammatory bowel diseases]. / Gyulladásos bélbetegségek bór- és nyálkahártyatünetei.

The rheumatological, ophthalmological- and dermatological complications are the most common ones among the extraintestinal manifestations of inflammatory bowel diseases (IBD). The incidence of skin manifestations is estimated to be 15-20% in case of Crohn's disease and 10% in case of ulcerative colitis. The so called specific lesions (perianal fissures, metastatic Crohn's disease), which are part of the skin symptoms associated with IBD, show a intimate connections with the bowel disease itself, as they histologically show granulomatous inflammation with epitheloid cells, similar to the ones seen in the intestines. The reactive lesion (erythema nodosum, pyoderma gangraenosum), that form the second main group of skin changes, can also be found is other systemic diseases, but they are more frequently associated with IBD than the average. Cutaneous manifestations may occur due to malabsorption or drug therapy. Finally, there are dermatoses (epidermolysis bullosa acquisitia, acne fulminans) which have a still questionable connection with IBD. Authors present an overview of the IBD's possible skin and mucosal symptoms and their prognostic significance and they demonstrate some rare common skin manifestations found among the IBD patients of Borsod Country (580 ulcerative colitis, 265 Crohn's disease) in the last 25 years.

[Cutaneous manifestations of strongyloidosis]. / A strongyloidiasis börtünetei.

Strongyloidiasis is a parasitic disease, caused by Strongyloides stercoralis, an intestinal nematode, which is mainly endemic in tropical and subtropical regions. It can be sporadically found in the temperate zone, especially in closed communities and among people living under bad social conditions. Gastrointestinal, pulmonary and cutaneous symptoms may arise during the migration of the larvae. The infections are chronic and poor in symptoms among immunocompetent patients. Sometimes the cutaneous manifestation is the only symptom of the disease besides the distinct eosinophilia. Intense itching, erythematosus papule and petechiae develop at the site of the skin infection. Rapidly progressing linear, serpiginous, urticarial streaks are the pathognomic cutaneous manifestations that are called larva currents. The appearance of erythematosus, linear stripes are due to the migrating larvae in the skin. The most common nonspecific symptoms are urticaria, maculopapular exanthema, localized or generalized pruritus and prurigo. The parasite is uniquely able to carry out its whole life cycle inside the human body, so in immunocompromised patients the disease can lead to a hyperinfection syndrome with high mortality, due to the accelerated endogenous autoinfection. Authors present all possible skin manifestations of the strongyloidiasis, based on the case history of three brothers and sisters and that of a female patient suffering from hyperinfection syndrome.

Successful treatment of transient acantholytic dermatosis with systemic steroids.

A sixty-year-old man, developed 2-5 mm sized, hyperemic, itchy papules, vesicles, erosions and crusts on hyperemic base on his chest, abdomen, back, gluteal region, and proximal sites of his upper and lower extremities. The direct and indirect immunoflurescence tests were negative. Histology revealed extensive acantholysis in the epidermis in the following forms: pemphigus vulgaris-like suprabasal acantholysis, Darier-like acantholytic dyskeratosis with corps ronds, Hailey-Hailey-like suprabasal clefts, and pemphigus foliaceus-like superficial acantholysis with spongiosis. Using systemic steroids, topical drying, and reepithelising therapy, the patient was cured. He was symptom-free the first, fourth, and thirteenth months after finishing steroid therapy. We review the literature and the new subdivision of the disease according to the histological and clinical features.