[Ascites and suspected acute abdomen in hereditary angioedema due to C1 inhibitor deficiency]. / Aszites und vermeintliches akutes Abdomen bei hereditärem Angioödem durch C1-Inhibitor-Mangel.

HISTORY AND CLINICAL FINDINGS: A 35-year-old man, for 8 years known to have hereditary angio-oedema with recurrent cutaneous swellings and occasional attacks of gastrointestinal pain, developed very painful, colic-like upper abdominal symptoms and frequent vomiting. INVESTIGATIONS: Routine laboratory tests were normal, except for leucocytosis of 18,200 WBC/microliter. The plasma concentrations of C1-esterase inhibitor (5.6 mg/dl) and of complement factor C4 (10.0 mg/dl) were reduced. Computed tomography revealed about 500 ml free fluid, a perihepatic effusion and definite oedematous thickening of the ileal wall. TREATMENT AND COURSE: During conservative treatment with infusions and no food by mouth the symptoms regressed. Abdominal ultrasonography was normal (no free intraabdominal fluid). Since discharge (now more than 15 months ago) the patient has been on danazole medication (200 mg/d). CONCLUSION: Recurrent gastrointestinal colics are typical of for hereditary angio-oedema and can imitate an acute abdomen. Concurrent ascites has only recently been described. Appropriate instruction of the patient and his/her medical practitioner is important to avoid unnecessary laparotomy.

[Botryomycosis caused by fusobacteria]. / Botryomykose durch Fusobakterien.

In the Anglo-American literature botrymycosis is described as a chronic cutaneous granulomatous reaction to bacterial infection, containing granules resembling the sulphur granules seen in actinomycosis. The diagnostic and therapeutic aspects are discussed with reference to a recently observed patient. Fusobacterium nucleatum was isolated as the bacterial cause of the pathologic disorder.

[Eosinophilic cellulitis--clinical and histological aspects]. / Eosinophile Zellulitis--klinische und histologische Aspekte.

Eosinophilic cellulitis (Wells' syndrome) is a rare skin disease whose aetiology is still unknown. The onset is acute, and different disease stages may exist simultaneously during the course. Histological examination shows flame figures as a characteristic, but not pathognomonic, feature. In later stages the infiltrate adopts granulomatous characteristics. Malignant haematological disorders, which frequently occur with this disease, should be excluded.

Pathogenesis of paraneoplastic follicular hyperkeratotic spicules in multiple myeloma. Follicular and epidermal accumulation of IgG dysprotein and cryoglobulin.

We describe a 62-year-old man with multiple myeloma who developed horny spicules on his face, particularly on his nose. IgG-lambda monoclonal gammopathy was detected, and the serum dysprotein was shown to be a cryoglobulin, which forms a cryogel at low temperatures. Light and electron microscopic and immunohistochemical examinations showed an intercellular precipitation and massive accumulation of the IgG dysprotein and cryoglobulin between the keratinocytes of the upper epidermis and the infundibular epithelium. The follicles were dilated and filled with parakeratotic cells, the protein deposits between them and a rudimentary hair thus resulting in the clinically visible symptoms of horny spicules. The limitation or the predominance of the symptoms in cold-exposed body regions, the morphological identification of the dysprotein deposits as cryoglobulin or cryogel, and the laboratory findings concerning the temperature and pH dependence of the precipitation of the IgG dysprotein reveal that the paraneoplastic horny spicules are a hitherto unknown clinical manifestation of cryoglobulinemia.

Persistent skin ulcers, mutilations, and acro-osteolysis in hereditary sensory and autonomic neuropathy with phospholipid excretion. Report of a family.

We observed three children in a Turkish family who from early childhood had deformations of the feet and torpid ulcers with subfocal osteomyelitis and osteolysis, which subsequently led to amputations. The fingers showed ainhumlike constriction bands and spontaneous amputations. Neurologic studies revealed an almost complete sensory and autonomic loss affecting all modalities and a marked involvement of motor fibers. The clinical symptoms fulfill many of the hallmarks of hereditary sensory and autonomic neuropathy type II, including autosomal recessive inheritance, onset of symptoms in childhood, and mutilating acropathy. A high urinary excretion of sphingomyelin and lecithin suggests that the pathogenic mechanism may be a disorder of phospholipid metabolism.

[Prurigo and further diagnostically significant skin symptoms in strongyloidosis]. / Prurigo und weitere diagnostisch bedeutsame Hautsymptome bei Strongyloidose.

An increasing incidence of strongyloidosis must be expected in European countries as a result of the increasing numbers of immigrants, as well as holiday-makers returning from tropical regions. In addition to gastrointestinal symptoms, dermatological complaints are predominant. Only rarely are cutaneous symptoms the only clinical manifestation of disease. The penetration of filariform larvae may cause "ground itch." In cases of chronic disease, larva currens is the most obvious sign and consists of linear urticarial wheals evoked by larva migration. The most common non-specific symptoms are rashes, pruritus and urticaria. A further symptom of strongyloidosis, intensely itching prurigo, is described in a 20-year-old female Thai. Remission was achieved following tiabendazole therapy.

[Regional intra-arterial chemotherapy with Dacarbazine in isolated liver metastases of a malignant melanoma]. / Regionale intraarterielle Chemotherapie mit Dacarbazin bei isolierten Lebermetastasen eines malignen Melanoms.

A 58-year-old woman underwent regional intra-arterial chemotherapy for hepatic metastases of a malignant melanoma, because at the time no other organ metastasis was demonstrable. Dacarbazine was injected as a bolus on five successive days, four weeks apart, after which there were several days of fever and the urinary melanogen test became positive. At first the patient clearly improved and was again able to walk. But four months after the start of treatment she died in a hepatic coma.

[Multiple simultaneous hematomas of the finger and toe nails with subsequent onychomadesis in pemphigus vulgaris]. / Multiple gleichzeitige Hämatome der Finger- und Zehennägel mit nachfolgender Onychomadesis bei Pemphigus vulgaris.

Multiple simultaneous subungual and intraungual haemorrhages were seen in eight fingernails and several toenails of a patient suffering from pemphigus vulgaris. As other causes of nail haematomas, such as traumas, were absent, this symptom was referred to the underlying disease. Nail and nailbed involvement in pemphigus vulgaris is extremely rare, and onychomadesis is the form that has been reported more frequently than any others.

[Formation of IgG antibodies to C1 inhibitor as the cause of life-threatening angioedema]. / IgG-Antikörperbildung gegen C1-Inhibitor als Ursache lebensbedrohlicher Angioödeme.

A clinical picture with recurrent (in some cases potentially fatal) edema of skin and internal organs based not on a hereditary C1 inhibitor deficiency, but an acquired loss of C1 inhibitor activity due to antibodies is described for the first time in two patients. The clinical symptoms commenced in middle age patients between 40 and 46 years old. Anti C1 antibodies of the IgG were found in both patients. Quantitatively, these C1 inhibitor protein was in the lower range of normal, whereas no inhibitor activity could be demonstrated functionally. The function of the complement components C1, C2 and C4 was greatly reduced. The therapeutic use of C1 inhibitor concentrate at a high doses (6 X 500 U) as well as administration of high-dose corticosteroids in several emergency situations was unsuccessful.

[Contraception and pregnancy in hereditary angioedema]. / Kontrazeption und Schwangerschaft beim hereditären Angioödem.

The frequent first clinical manifestation of hereditary angioedema (HAE) in pubertal patients and the more frequent attacks of edema during menstruation indicate that the condition is affected by hormones. Medication with oral contraceptives containing estrogens can lead to severe attacks of edema. On the other hand, HAE mostly shows a favorable development in pregnancy. Despite the substantial trauma of delivery, complications are rare. In view of the literature report of a death and our own observation of potentially dangerous edema in the postpartal phase in one patient, it appears to us to be advisable to administer 500 units C1 inactivator prophylactically before the expulsive phase of birth in HAE patients. Oral contraceptives should not be administered.

[Contact dermatitis caused by PVP-iodine]. / Kontaktdermatitis durch PVP-Jod.

Allergic contact dermatitis to PVP iodine is rare. Such an occurrence in a 81-year-old woman is reported. Extensive blistering dermatitis developed after local application of a PVP iodine solution. As in all previously described cases of allergic contact dermatitis from PVP iodine, it was a reaction not to the PVP but the free iodine. More important than contact reactions are hyperthyroidism or thyrotoxic crises due to percutaneous or transmucous absorption of iodine or iodide in susceptible persons.

[Nail dyschromia as the leading symptom in chronic mercury poisoning caused by a cosmetic bleaching preparation]. / Nageldyschromie als Leitsymptom einer chronischen Quecksilberintoxikation durch ein kosmetisches Bleichmittel.

Prolonged use of a cosmetic cream containing mercury induced a greenish black nail discoloration in a 56-years-old female patient. Electron microscopy as well as energy-dispersive X-ray analysis identified mercury in the cells of the nail plate. The patient showed mild symptoms of a chronic mercury intoxication with high levels of mercury in serum and urine. Treatment with 2,3-dimercaptopropane-1-sulfonate was effective and well tolerated.

Interaction between C1-INA, coagulation, fibrinolysis and kinin system in hereditary angioneurotic edema (HANE) and urticaria.

The C1-inactivator plays an important role not only in the initial phases of the complement system, but also in those of the coagulation, fibrinolysis and kinin systems. The present study was concerned with the reciprocal influence of decreased C1-inactivator levels in patients with hereditary angioneurotic edema (HANE, HAE). In 13 HANE-I patients there were significantly increased levels of the coagulation factors XII, XI, V, of plasminogen and of alpha 2-antiplasmin, while the factors IX and VII were decreased. Conversely, it emerged that in patients with markedly raised prephase factor levels, angioneurotic edema occurred in the presence of normal or only slightly decreased C1-inactivator levels. However, the ratio between factor XI and C1-INA activity was significantly higher than in normal and urticaria patients. Factor XII, HMWK, XI, VIII and V levels were significantly raised in 10 patients with frank chronic urticaria, while factor VII was lowered. Numerous other factors and inhibitors of the coagulation, fibrinolysis and kinin systems were, however, normal or showed no significant differences.

Hereditary angioneurotic oedema and blood-coagulation: interaction between C1-esterase-inhibitor and the activation factors of the proteolytic enzyme systems.

C-1-inactivator (C-1-INA) does not only exert its important inhibitory functions in the complement system but also in the first step in the activation of the coagulation, fibrinolytic and kallikrein system. We therefore determined in nine patients with hereditary angioneurotic oedema (HANE) with obvious quantitative or functional defects of C-1-INA, and one further patient with Quincke-type oedema of different origin, the coagulation factors of the initial phase such as Hageman factor, plasma thromboplastin antecedent (PTA) and high molecular weight kininogen (HMWK). These factors were further correlated with the concentration as well as functional activity of C-1-INA. Nine of ten patients showed a significant, sometimes even excessive, increase in the levels of factor XII (mean +/- SD = 146% +/- 63), HMWK (mean +/- SD = 126% +/- 56) and PTA (mean +/- SD = 289% +/- 294), and a decrease of C1-esterase inhibitor (C-1-inactivator), which was measured with a immunologic method (mean +/- SD = 9.6 mg/dl +/- 6.6) for its concentration as well as being measured for its activity (mean +/- SD = 30.4% +/- 24.9).

[Melanoma cells in urine sediment]. / Melanomzellen im Urinsediment.

A case of a 53 year old female patient with a metastatic malignant melanoma showing predominantly amelanotic tumor cells in urinary sediment is reported. Autopsy findings secured a metastasis of the ureter with complete penetration.