[Leucocytoclastic eosinophilic vasculitis with Löffler syndrome]. / Leukozytoklastische eosinophile Vaskulitis bei eosinophilem Lungeninfiltrat "Löffler"

Histologically, leukocytoclastic vasculitis (LV) presents with neutrophilic granulocytes with leukocytoclasia and erythrocyte extravasation, associated with variable counts of lymphocytes, plasma cells and eosinophilic granulocytes. The association of a LV with eosinophilic granulocytes and eosinophilic pneumonia was first described by Chan et al. in 1982. Our case represents the second report in the literature of this rare disease: a 85 year old patient with LV and numerous eosinophilic granulocytes in association with intermittent blood eosinophilia and Löffler syndrome (eosinophilic pulmonary infiltrates). The recurrent episodes were treated successfully with oral corticosteroids.

Primary mucinous carcinoma of the scalp.

Primary cutaneous mucinous carcinoma (MC) is a rare epithelial neoplasm derived from the sweat glands. Herein, we report a case of MC located on the head. A 66-year-old woman underwent excision of a nodular tumor with a reddish brown surface on the left parietal region. Histopathology revealed a neoplasm extending from the reticular dermis into the subcutaneous fat. The tumor cell aggregates showed cribriform and solid lobules and were embedded in lakes of mucin, separated by thin, fibrous septae. Focally single neoplastic cells were arranged in an Indian-file pattern. The tumor cells displayed an eosinophilic cytoplasm, large basophilic nuclei and some discrete nuclear atypia. Vascular spaces, filled by densely packed erythrocytes between the septae, were also observed. We compared the mucinous component with the tumor cell and the stromal component by light microscopy. Analyzing the tumor by an image analysis system in Alcian-blue-stained serial sections, we found the averaged total tumor area measuring 99.7 mm(2). The area of the mucinous component measured 92.4 mm(2), that of the tumor cells 3.7 mm(2) and that of the stromal component 3.6 mm(2). The extensive checkup of the patient disclosed no evidence for a further malignant neoplasm. After excision of the tumor an adjuvant radiotherapy was performed. The patient was free of recurrence and metastatic spread of the mucinous carcinoma during a 4-year follow-up.

Development of a disease activity index for the assessment of reactive arthritis (DAREA).

OBJECTIVES: The objectives of this study were to investigate and validate individual variables and to develop a composite score for disease activity measurement in patients with reactive arthritis (REA). METHODS: In the first cross-sectional part, the clinical and laboratory evaluation of 45 patients was used to elaborate the most important individual disease activity measures. In the second prospective part, these variables as well as a composite score for disease activity measurement of REA were prospectively validated in 23 patients at two points in time. RESULTS: The following variables emerged as the most useful for the composite measure: number of swollen and tender joints, patient's pain and global assessment, and C-reactive protein. The score was calculated by simple addition of the individual figures. CONCLUSION: DAREA constitutes a reliable score which can easily be assessed on a day-to-day office work basis.

[Lichen striatus or Blaschkitis in the adults. Variations of the same entity?]. / Lichen striatus oder Blaschkitis des Erwachsenen. Variationen derselben Entität?

A 64-year-old woman presented with extensive erythematous papulosquamous lesions along the lines of Blaschko on the right half of the trunk and the right leg. The clinical and histological features led to the diagnosis of lichen striatus, a dermatosis which usually appears during childhood. Treatment with acitretin led to marked improvement. Lichen striatus and its differential diagnoses--particularly "blaschkitis of adulthood" as first described in 1990--are discussed.

Primary invasive signet-ring cell melanoma.

The histopathological variants of malignant melanoma include the common type (lentigo maligna, superficial spreading melanoma, nodular melanoma, acrolentiginous melanoma), spindle cell, desmoplastic, balloon cell, pleomorphic (fibrohistiocytic), myxoid, small cell melanoma and malignant blue nevus. Recently, signet-ring cell melanoma was introduced as an additional cytologic variant. We describe a 72-year-old patient with a primary signet-ring cell melanoma of the skin located on the upper arm. Histopathologic examination disclosed a melanocytic tumor extending from the epidermis to the deep reticular dermis. Numerous pleomorphic tumor cells showed large, intracellular vacuoles and oval to spindle-shaped nuclei at their periphery. Mitotic figures and multinucleated melanocytes were also observed. Some of the signet-ring cells exhibited cytoplasmatic periodic acid-Schiff (PAS)-positivity. Immunohistochemistry showed positive reaction of the tumor cells for S-100, HMB-45 protein and vimentin, confirming their melanocytic differentiation. Tumor cells were negative for cytokeratins, epithelial membrane antigen (EMA), and carcinoembryonic antigen (CEA). The signet-ring cell melanoma disclosed an invasion to Clark Level IV and tumor thickness of 2.2 mm. Signet-ring cell melanoma is a rare morphologic variant of melanoma. Its recognition is important for differentiation from other tumors featuring signet ring cells.

[Papular-purpuric gloves and socks syndrome]. / Papulopurpurisches Gloves-and-socks-Syndrom.

In 1990, we first described the papular-purpuric "gloves and socks" syndrome (PPGSS). It is characterized by an itching erythema and edema of the hands and feet together with oral mucosal lesions and fever. The skin lesions subsequently become purpuric and resolve spontaneously after a few days. In more than half of the patients, an acute parvovirus B 19 infection has been proven. In a few cases of PPGSS, other viral infections have been found, for example hepatitis B and measles. We report on a patient with PPGSS and review the literature.

Pseudoporphyria cutanea tarda induced by furosemide in a patient undergoing peritoneal dialysis.

We report on a 32-year-old female patient undergoing peritoneal dialysis for diabetic nephropathy who developed blisters and cutaneous fragility on the dorsa of the hands. Histopathology of an early lesion showed a subepidermal cleft under a regular epidermis with single necrotic keratinocytes and normal dermal structures. In an advanced lesion, a subepidermal bulla and caterpillar bodies were found in the epidermis. Dermal vessel walls were normal and surrounded by a discrete lymphocytic infiltrate. Direct immunofluorescence revealed perivascular deposits of C3. Uroporphyrin and coproporphyrin levels of serum, urine and dialysate were repeatedly normal, confirming pseudoporphyria. The patient took furosemide (500 mg daily). Replacement of furosemide by ethacrynic acid led to spontaneous healing of the blisters. The patient was free of further lesions at the 1-year follow-up.

[The ABCD rule in dermatoscopy: analysis of 500 melanocytic lesions]. / Die ABCD Regel in der Dermatoskopie: Analyse von 500 melanozytären Läsionen.

500 melanocytic lesions were examined by dermatoscopy using the ABCD rule prior to excision and histologic diagnosis. Regular nevi (n = 272) exhibited a score of 3.55 +/- 0.87, nevi with histologic signs of dysplasia (n = 190) a score of 4.0 +/- 0.68 and melanomas (n = 30) a score of 5.08 +/- 1.24. This study suggests that the ABCD rule of dermatoscopy greatly facilitates the evaluation of melanocytic lesions. When the dermatoscopic score is higher than 4.2, melanoma should be considered.

Cutaneous malakoplakia on the forehead.

A case of cutaneous malakoplakia in an 81-year-old woman in reported. A reddish slowly growing plaque had appeared on her forehead 13 years previously. Histopathology revealed a dense dermal granulomatous infiltrate consisting of lymphocytes and numerous histiocytes containing Michaelis-Gutmann bodies. Ciprofloxacin treatment resulted in partial involution of the lesion.

Necrotizing cutaneous lesions caused by interferon beta injections in a patient with multiple sclerosis.

We report a patient with multiple sclerosis who was receiving subcutaneous injections of recombinant interferon beta. During the third month of treatment, painful necrotizing cutaneous lesions appeared at the injection sites. The possible pathogenesis of interferon-induced skin necrosis is discussed.

Relapsing linear acantholytic dermatosis.

The case of an 81-year-old man with relapsing linear acantholytic dermatosis is described. This is the second description of this disease entity, which is characterized by the histopathologic and ultrastructural features of Hailey-Hailey disease. Clinically, it is characterized by skin lesions that wax and wane in a systematic pattern following the lines of Blaschko.

[Specific IgM tests in syphilis diagnosis]. / Spezifische IgM-Teste in der Syphilis-Diagnose.

A total of 359 sera of untreated patients with syphilis were examined by three methods for the detection of Treponema pallidum specific IgM antibodies, the 19S-IgM-FTA-ABS test, the IgM solid phase haemadsorption assay (IgM-SPHA), and the IgM Captia assay. The results were compared and evaluated. In primary syphilis, the 19S-IgM-FTA-ABS and IgM-captia yielded reactive results in all patients, whereas only 40% were positive in the IgM-SPHA; the corresponding values for early latent syphilis wee 96.0%, 89.8% and 73.1%, respectively. In secondary syphilis, the reactivity of one serum out of 27 was missed by IgM captia and that of another, by the IgM-SPHA. Mean values (ELISA units = extinction/cut-off) of IgM-captia were higher in primary (2.25) than in secondary syphilis (1.70). In neurosyphilis, only the IgM-SPHA test detected reactivity in all sera, sensitivity for 19S-IgM FTA-ABS and IgM-Captia was 50.0% and 23.1%, respectively. Specificity of the IgM-Captia test results, determined in 386 sera, was 91.2%. The results of specific IgM tests are essential in the diagnosis of congenital syphilis as well as in the recognition of reinfection; they indicate the need for treatment and are useful in the assessment of the effectiveness of therapy.

[Dyshidrosiform bullous pemphigoid]. / Das dyshidrosiforme bullöse Pemphigoid.

Dyshydrosiform bullous pemphigoid is a clinical variant of bullous pemphigoid in which the lesions appear primarily on palms and soles. Knowledge of this unusual manifestation of bullous pemphigoid may be of practical relevance, since it has to be included in the differential diagnosis of blistering palmoplantar dermatoses. The correct diagnosis is confirmed by histological examination and immunofluorescence, which reveal all the characteristic hallmarks of bullous pemphigoid. Three patients with dyshydrosiform bullous pemphigoid are presented; clinical and histological aspects, findings on immunofluorescence, differential diagnosis and therapy are discussed.

[Recurrent venous thromboses in hypoplasia of the vena cava inferior and factor XII deficiency]. / Rezidivierende Venenthrombosen bei Vena-cava-inferior-Hypoplasie und Faktor-XII-Mangel.

The cause of recurrent pelvic and leg venous thromboses in a 24-year-old man was found to be a combination of two rare anomalies, hypoplasia of the hepatic, prerenal segment of the inferior vena cava and factor XII deficiency (factor XII activity 38%, its antigen 39% of normal), the latter considered a risk factor for thromboembolism. Subsequent fibrinolysis was not successful. No thromboembolic phenomena occurred during the following 16 months of oral anticoagulation with phenprocoumon. When this treatment was discontinued at the patient's behest, there was a recurrence on the contralateral side. Anticoagulation was resumed and has continued now for 1 1/2 years without recurrence. The patient has been largely free of symptoms. Permanent anticoagulation thus seems unavoidable in this case.

Ischemic venous thrombosis caused by a distinct disturbance of the extrinsic clotting system.

Ischemic venous thrombosis that led to necrosis of four toes developed in an 81-year-old man. Despite the extensive thrombosis, results of blood clotting tests showed an extremely low prothrombin time (20%). Plasma mixing studies demonstrated an inhibitor that may have features in common with the lupus anticoagulant. Lupus anticoagulant also simulates hemorrhagic diathesis in vitro, whereas in vivo it is associated with thrombosis and thromboembolism. In contrast to the lupus anticoagulant, the inhibitor found in this patient was active within the extrinsic clotting system. Systemic steroids led to rapid clinical resolution paralleled by normalization of the prothrombin time and disappearance of the inhibitor.

[Detection of causal agents of AIDS using polymerase chain reaction and chemiluminescence measurement]. / Erregernachweis von AIDS mittels Polymerasekettenreaktion und Chemilumineszenzmessung.

The polymerase chain reaction (PCR) allows the detection of minimal amounts of DNA segments, and thus is used for the laboratory diagnosis of HIV (human immunodeficiency virus). In the present study a solution hybridization assay using acridinium ester-labelled probes was applied for detection of amplified HIV-1 DNA segments. Amplification was achieved by 30 cycles of the polymerase chain reaction using SK38/SK39 primers specific for a constant region of the HIV-1 gag region. Amplified products were hybridized with acridinium phenyl ester-labelled probes and measured by chemiluminescence. All of 163 blood samples obtained from HIV-infected patients (CDC II-CDC IV) were reactive, while autoradiography with a 32P-labelled SK19 probe only detected 146 patients (89.5%). The combination of PCR with chemiluminescence avoids the use of radioactive material and is sensitive and quick, allowing the "PCR results" to be reported on the same day.

[Successful use of isotretinoin in type Zumbusch generalized pustular psoriasis following recovered etretinate-induced hepatitis]. / Erfolgreicher Einsatz von Isotretinoin bei Psoriasis pustulosa generalisata Typ Zumbusch nach abgelaufener Etretinathepatitis.

Administration of etretinate in a 29-year-old female patient suffering from severe pustular psoriasis caused a dramatic increase in liver enzymes. Liver biopsy revealed changes characteristic for drug-induced hepatitis. After normalization of liver parameters following withdrawal of etretinate, isotretinoin was administered during a severe pustular relapse. In contrast to etretinate, isotretinoin was well tolerated and resulted in a good therapeutic response. Thus, isotretinoin can be considered as an effective and safe therapeutic alternative for pustular psoriasis even after the occurrence of etretinate-induced hepatitis.