A monoclonal Ro-antibody and the serum of a Ro-positive patient with subacute cutaneous lupus erythematosus (SCLE) react with basal layers of human epidermis.

Skin lesions, especially at areas exposed to sunlight, prove to be a major form of manifestation of diseases related to Ro-antibodies and neonatal-, 'ANA-negative-', and cutaneous types of lupus erythematosus. A monoclonal Ro-antibody established by our group reacts with a 60 kD polypeptide in extracts from human spleen, whereas in extracts from human epidermis the monoclonal Ro-antibody and a purified Ro-antibody from a monospecific serum of a patient with subacute cutaneous lupus erythematosus reacted with a 60 kD and a 48 kD protein. Performing immunofluorescence microscopy on HEp2-cells both antibodies showed a nuclear speckled staining pattern and a reaction with cytokeratin filaments. On skin biopsies obtained from the patient, predominantly the basal layers of the proliferative stratum germinativum demonstrated a high rate of immunofluorescence; antigen-expression seems to depend on sunlight exposure. From these data we assume that, especially in subacute cutaneous lupus erythematosus, cross-reaction of the Ro-antibody with one of the lower molecular keratins happens in that area of human epidermis which histologically shows cellular destruction.

[Further information on the alkali resistance test]. / Zur weiteren Kenntnis der Alkaliresistenz-Probe.

In 90 patients the alkali resistance (AR) according to Burckhardt was measured on the back of the hand and on the thigh. Subjects with healthy skin (n = 30) reacted in a normal way. Those with healed hand eczema (n = 30) had a diminished AR in both areas tested; subjects with atopic eczema (n = 30) presented an even more diminished AR. Thus, in this broadened scope, simultaneous determination of AR on the back of the hand and on the thigh with the AR-test seems to be valuable in the recognition of an eczematous disposition.

[Functional Fc receptor defect of granulocytes in a patient with Sjögren's syndrome]. / Funktioneller Fc-Rezeptordefekt von Granulozyten bei einem Patienten mit Sjögren-Syndrom.

A patient with Sjögren's syndrome exhibited markedly impaired stimulation of her granulocytes via Fc-receptors (FcR), four other stimuli elicited a normal chemiluminescent response. Unexpectedly, granulocytes of the control bore 39,000, of the patient, however, 86,000 FcR/granulocyte.

Functional Fc-receptor defect of polymorphonuclear leukocytes in a patient with Sjögren's syndrome.

We investigated polymorphonuclear leukocyte (PMN) function in a 50-year-old woman with primary Sjögren's syndrome (SS). The respiratory burst of PMN was monitored by luminol-enhanced chemiluminescence using zymosan, opsonized zymosan, zymosan-activated serum, and phorbol-myristate-acetate, as well as serial dilutions of aggregated immunoglobulin (aggIg) as Fc-receptor (FcR) stimulus. The effects of serum on the chemiluminescent response as well as the binding of aggIg to PMN were also compared. We found the patient's PMN not to respond to stimulation by aggIg, only the highest concentration (greater than 180 micrograms/ml) induced a marginal chemiluminescent response in the patient. By contrast, incubation of the patient's PMN with other stimuli resulted in responses similar to those in a healthy control. Binding of aggIg to PMN was higher in the patient (3.6% vs 1.5% of the radioactivity added in the control). Sera of patient and control induced similar chemiluminescence on PMN as did that of another human serum. Our data indicate a selective functional FcR defect of PMN despite unimpaired binding of aggIg in a patient with SS.

[Highly differentiated squamous cell carcinoma of the shoulder with perineural invasion]. / Hoch ausdifferenziertes Plattenepithelkarzinom der Schulterregion mit frühzeitigem perineuralem Tiefenwachstum.

A de novo carcinoma of only a few weeks' duration is reported in a 34-year-old man. Histological findings revealed a well-differentiated squamous cell carcinoma with unexpected early spread deep in the dermis and subcutis and a striking affinity to the neural structures.

[Simultaneous occurrence of lupus erythematosus and hepatic porphyria--coincidence or chance association?]. / Gemeinsames Vorkommen eines Lupus erythematodes und einer hepatischen Porphyrie--Koinzidenz oder zufällige Assoziation?

We report on two cases of coexisting lupus erythematosus and hepatic porphyria and give a review of the corresponding literature. It is not clear yet, if the coincidence is accidental or if there exist any unknown relationship. 29 patients with these two conditions were found in the literature.

[Undifferentiated cutaneous angiosarcoma of the head: identification by the endothelial marker Ulex europaeus agglutinin I]. / Undifferenziertes kutane Angiosarkom des Kopfes: Identifizierung durch Endothelmarker Ulex europaeus Agglutinin I.

Cutaneous angiosarcoma of the head is a rare tumor of the elderly and can occur in an undifferentiated form without any clinical or histological signs of the vascular origin of this tumor. In these cases, the tumor can be identified by using endothelial cell markers, such as factor-VIII-related antigen and ulex europaeus agglutinin I, in an immunofluorescence technique or a peroxidase-antiperoxidase method. A 78-year-old patient is described who died within 18 months from such a tumor, which was diagnosed using the endothelial cell marker, ulex europaeus agglutinin I.

[Morphologic knowledge of polyneuropathy in progressive scleroderma]. / Zur morphologischen Kenntnis der Polyneuropathie bei progressiver Sklerodermie.

The occurrence of polyneuropathy in connection with systemic sclerosis is extremely rare. Proliferative changes leading to mechanical compression of the nerves have been ascribed to an etiological involvement. In the case reported here, polyneuropathy could be verified morphologically (nervebiopsy). The elevated IgG in the cerebrospinal fluid suggests inflammatory changes of the nerves to be the cause of polyneuropathy.

[Visible hypertrophy of the musculus corrugator supercilii]. / Uber eine blickmässig auffällige Hypertrophie des Musculus corrugator supercilii.

A patient with constant intumescences of the forehead due to bilateral hypertrophy of mimic muscles is reported.

[Symptomatic scleroderma caused by bleomycin]. / Symptomatische Sklerodermie durch Bleomyzin.

Side effects of bleomycin therapy are most frequent in the skin and as pulmonary fibrosis because of the nearly selective concentration in these organs. The cutaneous manifestations include a variety of clinical symptoms, most frequently erythema and infiltrations with marked hyperpigmentation. A 38-year-old patient is reported who received 615 mg bleomycin for therapy of a malignant testicular teratoma and in the following time developed numerous morphoea-like lesions, acrocyanosis, Raynaud's phenomenon, acrosclerosis, and cutaneous calcinosis. Histologically, the alterations were similar to those of scleroderma. In contrast to progressive systemic sclerosis the symptomatic character of the lesions is evident, for the skin changes resolved several months after the drug was discontinued.

Manometric assessment of oesophageal involvement in progressive systemic sclerosis, morphoea and Raynaud's disease.

Oesophageal function was studied by radiography and manometry in fifty-one patients with progressive system sclerosis (PSS), fourteen patients with morphoea, twelve patients with Raynaud's disease and twenty-one normal subjects. Upper oesophageal sphincter pressure was not affected. Peristaltic contractions of the upper third of the oesophagus were significantly decreased in PSS and morphoea, but the lower two-thirds were affected only in PSS. Lower oesophageal sphincter pressure was significantly decreased in PSS but was normal in morphoea and Raynaud's disease. There was a significant correlation between oesophageal dysfunction and the duration of PSS. Manometry was better than radiography at detecting motor abnormalities, with a positivity of 86% compared with 66% for radiography. Oesophageal manometry should be employed routinely in the investigation of patients with suspected PSS or morphoea.