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Basal cell carcinomas (BCC) are caused by various factors such as ultraviolet light, ionizing radiation, possibly human papilloma viruses and chemical agents. Among the latter inorganic trivalent arsenic has long been recognized as an important cause of BCC. A 70-year-old woman developed multiple basal cell carcinomas mainly on the trunk after taking sodium hydrogen carbonate contaminated with arsenic for many years for chronic gastritis.

Ulcera crurum stellen ein atiopathogenetisch besonders vielfaltiges Symptom dar. Am haufigsten liegen Vaskulopathien zugrunde, seltener metabolische, neuropathische oder hamatologische Erkrankungen. Auch Neoplasien, Kollagenosen, Infektionen, Traumata und Pannikulitiden mussen differentialdiagnostisch bedacht werden. Vorgestellt wird eine 38jahrige Patientin, bei der ein hereditarer Prolidasemangel als Ursache ihrer seit der Kindheit bestehenden, therapieresistenten Ulcera crurum gesichert werden konnte. Auser den progredienten, sehr schmerzhaften Beingeschwuren zeigte die Patientin weitere Merkmale des Prolidasemangel-Syndroms, wie intellektuelle Minderbegabung, verminderte Korpergrose, eine ausgepragte Karies und auffallende Wangenteleangiektasien. Der hereditare Prolidasemangel ist eine sehr seltene autosomal-rezessiv vererbte Erkrankung. Ursachlich sind heterogene Mutationen im Prolidase-Gen mit Auswirkungen auf den gesamten Proteinstoffwechsel. Die labordiagnostische Sicherung der Verdachtsdiagnose kann mittels Hochspannungselektrophorese und Ionenaustausch-Chromatographie des Urins erfolgen. Die hereditare Prolidasedefizienz ist nicht heilbar, die bisher mitgeteilten symptomatischen Therapieversuche blieben weitgehend erfolglos.

Full-thickness skin grafts have to be defatted to improve graft take and to avoid differences in thickness between the graft and the recipient site. The conventional method of thinning the graft with surgical scissors or a scalpel often leaves an irregular surface and is rather time consuming. Especially with a very thin preparation there is a risk of perforating the graft. We present a new surgical device which allows trimming a graft evenly in a considerably shorter period of time.

Basal cell carcinomas (BCC) are caused by various factors such as ultraviolet light, ionizing radiation, possibly human papilloma viruses and chemical agents. Among the latter inorganic trivalent arsenic has long been recognized as an important cause of BCC. A 70-year-old woman developed multiple basal cell carcinomas mainly on the trunk after taking sodium hydrogen carbonate contaminated with arsenic for many years for chronic gastritis.

We report a case of hypocomplementemic urticarial vasculitis (HUV). The clinical course was characterized by urticaria, angio-edema, pericarditis, joint pain and conjunctivitis. The laboratory findings revealed moderate proteinuria, erythrocyturia, normal renal function, normotension, reduction of C3, C4 and C1q complement with elevated C1q antibodies. Antinuclear antibodies were inconstantly positive. Renal biopsy showed a mild form of membranous glomerulonephritis. Despite intensive therapeutic measures (dapsone, immunosuppressives and immunoglobulins), relapses of urticaria occurred frequently. HUV is probably a minor form of systemic lupus erythematosus.

Hereditary alpha 1-antitrypsin deficiency may be very rarely associated with chronic panniculitis and is therefore often diagnosed only with delay.The typical clinical features of the disease are described in a 37-year-old woman. This case underlines the importance of determining alpha 1-antitrypsin levels in patients with chronic relapsing panniculitis. Therapeutic approaches are discussed.

The dermatology clinic Ludwigshafen was founded in 1910. Dr. Siegfried Fuss was head of the clinic for almost 40 years. The clinic's history reflects the rapid industrial growth of the city, the destruction of two world wars and the progress of dermatology during this century. Today, the clinic is an academic teaching hospital affiliated with the University of Mainz with 45 beds and offers a broad spectrum of modern dematological diagnostic procedures and therapies.

Leg ulcers may be caused by many different diseases. Most frequently, they are due to vasculopathies, to a lesser extent to metabolic, neuropathic or hematologic diseases. Neoplasms, connective tissue diseases, infections, trauma, and panniculitis should also be included in the differential diagnosis. A 38-year-old Caucasian female patient with hereditary prolidase deficiency developed progressive and very painful leg ulcers. The ulcers first appeared in childhood and did not respond to various treatments. Additional features of prolidase deficiency included mental retardation, short stature, extensive dental caries, and multiple malar teleangiectases. Hereditary prolidase deficiency is a very rare autosomal recessive disease. It is caused by heterogeneous mutations of the prolidase gene and affects many aspects of protein metabolism. Ion exchange chromatography and high voltage electrophoresis of urine can prove the suspected diagnosis. So far, there is no efficient therapy for hereditary prolidase deficiency. All reported treatment attempts have ended in failure.

Hypohidrosis and anhidrosis can be caused by various diseases. Diabetes mellitus, Sjogren syndrome, pure autonomic failure, Fabry disease, Ross syndrome, thyroid dysfunction, paraneoplastic autonomic dysfunction, and congenital absence of sweat glands are possible diagnoses.1 A less common cause of hypohidrosis/anhidrosis is the acquired idiopathic generalized anhidrosis (AIGA). Until now, 64 cases of AIGA have been reported, 62 being Japanese.2 We here report a European patient with AIGA. A 39-year-old white man presented with a 6-month history of progressive heat intolerance and lack of sweating except for the axillary zone and parts of his face. The patient also reported tachycardia and general fatigue. He had no history of dry mouth, dry eyes, concomitant sharp pain, or urticaria. The patient’s history and family history were unremarkable. The patient did not take any medications. Physical and neurologic examination including autonomic functional …

Proteus syndrome is a rare congenital disorder in which the major clinical manifestations are skeletal deformities and hamartomatous tumors. The case of a 42-year-old woman with right hemihypertrophy, macrodactyly of the right hallux, extreme kyphoscoliosis and bilateral plantar connective tissue naevi is reported. Proteus syndrome is reviewed with reference to this patient.

We report on a 48-year-old female patient suffering from lupus erythematosus associated with C2 deficiency, who developed squamous cell carcinoma on a discoid lesion of her lower lip. Vermilionectomy (Langenbec-v. Bruns' technique) resulted in a good cosmetic and functional outcome.

Following ingestion of aspirin (1 g) complement consumption could be demonstrated in 18/20 healthy volunteers. A slight to moderate decrease of whole complement activity (CH50) and of the components C1, C4 and C2 was found. In addition, reduced levels of the C1 inactivator (C1 IA) were measured in 19/20 probands. The drop in C titers and C1 IA was only detectable within a short period of time and 30 min after ingestion of the drug the titers were usually returning to normal. In view of these findings, the possible role of complement in adverse reactions to aspirin is discussed.

Aspirin (acetylsalicylic acid), ASA and sodium salicylate (NaSA) activate the complement system in vitro. The activation involves the components of the classical pathway. It is, however, antibody independent. Evidence for activation are (1) consumption of C and C component activities; (2) activation of C1 evidenced by release of C1s from the macromolecular complex, and (3) C3 and C5 conversion.

Salicylates used as acetylsalicylic acid (aspirin) or sodium salicylate, activates the complement system in vivo and in vitro in the absence of a specific antibody. Along with the complement activation, various biological activities such as chemotactic activity, leukocyte mobilizing activity, and smooth muscle contracting activity are generated. In vivo biological activities appear within minutes after application of the drug and are extremely transient.

Die Vererbung hat einen mesbaren Einflus auf die Talgdrusenfunktion. Sehr wahrscheinlich sind mehrere Gene beteiligt. Die quantitative Abstufung der Talgdrusenproduktion resultiert dabei aus variierenden Umwelteinflussen vor dem Hintergrund einer additiven und bei jedem Individuum zu verschiedenen „Summen” fuhrenden Genwirkung. Eine isolierte genetische Betrachtung der atopischen Sebostase ist nach unserem heutigen Wissensstand nicht moglich. Genetic Influence on the Sebaceous Gland Function with Special Consideration of Atopy Twin studies have demonstrated genetic control of sebum production. Probably many separate genes are involved. Xerosis of the skin due to reduced sebum production is an important phenotypic marker of the atopic individual. However, the genetics of sebaceous gland function in atopy cannot be separatedly regarded as yet. Different possible mechanisms of the genetic influence on sebum production are discussed.

Aspirin (acetylsalicylic acid), ASA and sodium salicylate (NaSA) activate the complement system in vitro. The activation involves the components of the classical pathway. It is, however, antibody independent. Evidence for activation are (1) consumption of C and C component activities; (2) activation of C1 evidenced by release of C1s from the macromolecular complex, and (3) C3 and C5 conversion.

Prolidase deficiency is a rare hereditary disease characterized by an iminodipeptiduria especially composed by glycyl-L-proline which is not further degraded. The study of collagen metabolism in fibroblast cultures from three prolidase-deficient patients showed an increase in the rapidly degraded collagen and a decrease in the proline pool. In order to elucidate the mechanism of this metabolic disturbance, glycyl-L-proline was added to the cell cultures. In the control cultures, the addition of this dipeptide caused an increase in the rapidly degraded collagen and a decrease in the proline pool. The effects on the patient fibroblasts depended on the severity of the deficiency. The metabolic function of the dipeptide glycyl-L-proline was discussed in the light of these results.

Die Pathogenese der Aspirinintoleranz ist noch weitgehend ungeklart. Eine Ubereinstimmung besteht bisher nur darin, das Antikorper in den meisten Fallen nicht beteiligt sind. So wurden mehrere antikorperunabhangige Pathomechanismen vorgeschlagen, u. a. eine direkte Freisetzung von Histamin und anderen Mastzellmediatoren (Kallos 1956), eine veranderte Reaktivitat der Kininrezeptoren (Samter u. Beers 1967), eine akkumulative Azetylierung korpereigener Proteine (Farr 1970), die Defizienz eines Enzyminhibitors (Yurchak et al. 1970), eine direkte Komplementaktivierung (Yurchak u. Mitarb. 1970) und — derzeit im Mittelpunkt der Diskussion — die nicht ausreichend regulierte Hemmung der Prostaglandinsynthese (Szczeklik et al. 1975, Parker 1977).

The association of pili torti (twisted hairs) and sensory neural deafness is a very rare heritable disorder which has been described by Bjornstad (1965) as a new genetic entity. We had the opportunity to examine a family with two affected siblings.

Annahernd 250 Erbkrankheiten disponieren zu Krebs und fast 50 von diesen haben Hautsymptome oder betreffen die Haut ausschlieslich [12, 14]. Genau betrachtet liegt die Zahl hoher, denn einige der phanotypisch geordneten Krankheitsbilder haben sich als heterogen erwiesen und werden nun mit wachsender Kenntnis ihrer strukturellen oder biochemischen Basisdefekte aufgegliedert, z. B. die hereditaren Epidermolysen mit inzwischen allein 16 [1] oder das Xeroderma pigmentosum mit 8 voneinander abgrenzbaren Genotypen [3].

Mit einer geschatzten Inzidenz von 1:3000 gehort die Neurofibromatose von Recklinghausen zu den haufigen Erbkrankheiten. Ihre phanotypische Variabilitat ist auserordentlich gros und damit auch die Auspragung der Neurofibrome, die sich als kleinknotig kutan-subkutane Elemente mit allen Ubergangen bis hin zu wammenartigen Tumormassen manifestieren konnen. Nicht selten wird der betreuende Dermatologe mit dem verstandlichen Wunsch konfrontiert, aus asthethischen oder funktionellen Grunden wenigstens einige dieser Tumoren zu entfernen oder zumindest in ihrer Grose zu reduzieren. Die Indikationsstellung wird dabei von deren Ausdehnung, Lokalisation und Wachstumsdynamik bestimmt. Kleinere Neurofibrome konnen exzidiert, mittels Scherenschlag entfernt oder sogar dermabradiert werden. Bei groseren Neurofibromen sind meist nur palliative Eingriffe moglich. Der Versuch einer Exzision „in toto“ ist insbesondere im Gesicht wenig aussichtsreich und kann zu irreversiblen Schaden fuhren. Ein besonderes Problem stellen intra- und postoperative Blutungen infolge der starken Vaskularisierung der Tumoren dar. Am Beispiel einer 25jahrigen Patientin, bei der erschwerend ein Faktor VIII-Mangel (Subhamophilie) hinzukam, werden die dermatochirurgischen Moglichkeiten bei der Neurofibromatose von Recklinghausen diskutiert.

Es wird uber eine 68jahrige Patientin mit einem rezidivierenden, destruierend wachsenden Keratoakanthom im Bereich des linken Oberlides berichtet. Erst die groszugige, mikroskopisch kontrollierte Exzision im Gesunden und Defektdeckung mit einem freien Vollhauttransplantat erbrachte Rezidivfreiheit. Die Beobachtung unterstreicht, das Kurettage, unvollstandige Exzision oder abwartendes Verhalten angesichts eines Keratoakanthoms in funktionell und kosmetisch wichtiger Lokalisation unvertretbar sind.

Complement (C) deficiencies may result from (a) genetic defects of C protein synthesis, (b) increased C consumption by circulating immune complexes, (c) abnormal C inhibitors (C1, C3b), and (d) passive loss of C protein (e.g., by plasmapheresis). The inherited deficiencies of C are of particular interest to both pediatrician and dermatologist since they usually appear early in life and frequently present with cutaneous manifestations. As “natural experiments” they have contributed significantly to our knowledge about the role of single C components and their interrelationship. Moreover, laboratory animal strains such as C4-deficient guinea pigs, C5-deficient mice, or C6-deficient rabbits have become a valuable tool in C research. Isolated C deficiencies in man have been described for all components of the classical pathway activation. In addition, C1 inactivator (C1INA) and C3b inactivator (C3bINA) deficiency are well-known hereditary defects of C regulation. However, no complete deficiencies of the alternative pathway components have been discovered to date.

Aspirin intolerance has become increasingly important in clinical dermatology during the past decade, especially as a diagnostic aid in chronic urticaria. It is recommended, to distinguish clinically between the intolerance syndrome and an intolerance provocation (of preexistent asthma or urticaria). Pathogenetically, various antibody-independent mechanisms are discussed of which most authors favour a defective regulation of arachidonic acid metabolism.

Amyloidosis is an extracellular deposition of the fibrous protein, amyloid, in one or more sites of the body and may cause a great variety of symptoms. Since amyloid deposit associated with aging is an extremely common finding, it has received considerable attention from various disciplines in modern medicine in recent years.

All analgesics can occasionally induce adverse skin reactions. Especially in the early period of pyrazolone therapy, a large variety of cutaneous manifestations were attributed to the use of these drugs. An updated analysis is attempted.

Tick bite fever (boutonneuse fever) is endemic in India, Africa and especially the Mediterranean countries. It is occasionally "imported" into central European countries by tourists. The exanthematic disease is caused by infection with Rickettsia conori, which is usually transmitted by the brown dog tick. A typical primary lesion at the site of the tick's bite is an important diagnostic hint. The treatment of choice are tetracyclines.

Two sisters with hereditary prolidase deficiency are presented. Recurrent and painful leg ulcers are the predominant feature.

Low levels of the fourth component of complement (C4) were found functionally and immunochemically in two sisters (17 and 22 years of age) suffering both from discoid lupus erythematosus. C4-typing of the family revealed that the two sisters and the mother had only two, and the father three of the four genes coding for C4 expressed. All carried the null allele C4B*QO. This observation adds to the increasing evidence that not only complete but also partial genetic complement deficiencies may predispose to lupus erythematosus.