[Familial congenital hypotrichosis with "uncombable hair," retinal pigmentary dystrophy, juvenile cataract and brachymetacarpia: another entity of the ectodermal dysplasia group].

A new ectodermal dysplasia syndrome is reported in a family of Caucasian ancestry. The syndrome consists of congenital hypotrichosis with "uncombable hair", juvenile cataract, retinal pigmentary dystrophy, oligodontia and brachymetacarpia, and is inherited as an autosomal dominant trait.

[Differential diagnosis and therapy of recurrent aphthae].

The differential diagnosis of aphthous stomatitis includes several viral diseases, such as gingivostomatitis herpetica, recurrent intraoral herpes simplex, herpangina, hand-, foot-and mouth disease, as well as drug eruptions, traumatic ulcers, and aphthoid lesions in neutropenia. The therapy of aphthous stomatitis is designed to control pain, shorten the course of present lesions, and prevent the development of new lesions.

[Psoriasis and bullous pemphigoid].

The coexistence of psoriasis and bullous pemphigoid in 44 patients has been described in the literature. In the majority of cases no causative agent was found for the development of bullous pemphigoid, but in many cases the bullous eruption was related to antipsoriatic treatment such as PUVA, anthralin, tar and salicylic acid. A 46-year-old man with a 20-year history of psoriasis who developed bullous pemphigoid without any previous therapy is described.

Autoantibody-mediated acquired deficiency of C1 inhibitor.

During the past 25 years, three forms of deficiency of the inhibitor of the first component of complement (C1 inhibitor) with angioedema have been recognized; two forms are hereditary and one is acquired. As compared with hereditary angioedema, the syndrome of acquired C1-inhibitor deficiency is rare, and it is usually associated with lymphoproliferative diseases. We report another type of acquired C1-inhibitor deficiency with angioedema.

[Contraception and pregnancy in hereditary angioedema].

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.

[Formation of IgG antibodies to C1 inhibitor as the cause of life-threatening angioedema].

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.

Impaired function of numerically augmented Fc-receptors on granulocytes in a HLA B8+ patient with palmoplantar pustulosis.

We examined granulocytes or polymorphonuclear leukocytes (PMN) in an HLA B8+ patient with palmoplantar pustulosis (PPP). Controls included another patient with PPP, however, lacking this antigen and a healthy, HLA B8+ person. Chemiluminescence (CL) served to monitor the respiratory burst in PMN comparing as stimuli zymosan, opsonized zymosan, phorbol myristate acetate, as well as aggregated immunoglobulin (aggIg), the latter as Fc-receptor (FcR) stimulus.

[Paraffin granuloma of the penis].

Granulomatous foreign-body reactions following paraffin injections can produce diagnostic difficulties, especially if the patient denies the injections. The paraffin injections serve to enlarge the penis and are mostly performed by psychiatrically patients. Undesired side effects that can occur are solid granulomatous infiltrations or penis deviation with impotentia coeundi.

[Contact dermatitis caused by PVP-iodine].

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.

Immunological detection of actin in isolated cilia from quail oviduct.

Cilia from quail oviduct were isolated with their membrane. The ultrastructural study revealed a good preservation of cilia in the purified fraction. Electrophoresis on SDS-PAGE showed a reproducible pattern of ciliary proteins, the major bands being those of tubulins 57 kDa and dyneins above 250 kDa.

Stimulation of T cells by autologous mononuclear leukocytes and epidermal cells in psoriasis.

Based on reports suggesting aberrant cell-mediated immunity and altered infiltration of immunocompetent cells into the skin in psoriasis, we studied the stimulation of T cells by autologous non-T mononuclear leukocytes (autologous mixed lymphocyte reaction, AMLR) and by epidermal cells isolated from lesional and clinically uninvolved skin in psoriasis (autologous mixed epidermal cell lymphocyte reaction, AMECLR). Age- and sex-matched individuals served as controls. We found that the AMLR in psoriasis (n = 11) was similar to that in healthy controls (n = 16); furthermore, cell proliferation was alike in the presence of either 5% AB-serum or autologous serum.

[Disseminated lichenoid form of lupus vulgaris].

The disseminated forms of skin tuberculosis are extremely uncommon and can occur as acute miliary tuberculosis with skin involvement, which is a severe disease with a rapid course, or as slowly developing lupus vulgaris, i.e., "postexanthematic lupus vulgaris" or "lupus vulgaris disseminatus".

[Pregnancy after malignant melanoma. A follow-up of 23 patients].

There is little known on the risks associated with pregnancy occurring after primary therapy of a malignant melanoma. Out of a total of 2850 melanoma patients there were 23 women (20 in stage I, 3 in stage II) in whom 33 pregnancies occurred in the course of the follow-up. No metastases developed in 18 patients with a total of 25 pregnancies whereas a relapse occurred in 3 patients in stage I and 2 patients in stage II.

[Artificial penile nodules (tancho nodules) in Southeast Asian men].

Especially in Thailand, but also in other countries in Southeast Asia, the practice of implanting glass or other kinds of beads in the subcutaneous tissue of the penis for the purpose of increasing the sexual pleasure of the partner has become common. The inserted glass beads do not usually give rise to symptoms and normally they are only detected by chance. Here we describe our experience with a 27-year-old Thai with a penile nodule made from the polished material of a tooth brush.

[Undifferentiated cutaneous angiosarcoma of the head: identification by the endothelial marker 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.

[Paraneoplastic multicentric reticulohistiocytosis: induced and inductable by iodine containing x-ray contrast media].

Following lymphography, a 41-year-old woman developed arthritis and papules of multicentric reticulohistiocytosis three years after the beginning of a carcinoma of the cervix, now inoperable and with vulvar metastases. Two further inevitable x-ray investigations with different contrast media led to exacerbation of the disease within 24 hours. 12 month after onset, all symptoms of multicentric reticulohistiocytosis receded spontaneously, and further investigations with contrast media were well tolerated.

Effects of ethanol and acetaldehyde on phagocytic functions.

Although a number of skin diseases are characterized by the presence of an increased number of phagocytes in their lesions, the effects of alcohol on phagocytic functions are not clearly understood. Therefore, we measured the influence of ethanol and acetaldehyde on the generation of oxygen radicals, chemotaxis and the release of lysosomal enzymes from human phagocytes. We added 0.

[Localized cutaneous siderosis following intramuscular iron injections].

Iron-induced discolorations at the injection site are rare because oral application is now preferred. A 31-year-old women is described with bilateral gluteal localized siderosis, who had received intramuscular iron injections 9 months before.

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.

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.

[Multiple lymphocytoma at the point of puncture as complication of acupuncture treatment. Traumatic origin of lymphocytoma].

In general, side effects of acupuncture are rare and this also concerns dermatological diseases caused by acupuncture. Mostly, infectious diseases occur by inoculation of the infectious agents, especially cutaneous bacterial diseases as staphylococcal folliculitis and abscesses, favoured by insufficient sterilisation of the needles. A 34-year-old female patient is reported, who developed multiple lymphocytomata cutis over the ears in the course of an acupuncture.