[Congenital pachyonychia type II (Jackson-Lawler syndrome)]. / Pachyonychia congenita Typ II (Jackson-Lawler-Syndrom).

Pachyonychia congenita (PC) is a rare ectodermal dysplasia with variable expression. The condition is usually inherited as an autosomal dominant trait. Several classifications of PC have been proposed. Feinstein and colleagues suggested four clinical types of PC. Type II, the Jackson-Lawler-Syndrome, is characterized by multiple epidermal cysts, palmoplantar bullae and hyperhidrosis as well as natal teeth in addition to the main findings of pachyonychia, palmoplantar hyperkeratosis and follicular keratosis. We report two patients (father and son) with Jackson-Lawler-Syndrome and describe in detail pathogenesis, diagnostic criteria and treatment approaches as well as the different classifications of pachyonychia congenita.

Photopatch testing: the 12-year experience of the German, Austrian, and Swiss photopatch test group.

BACKGROUND: In 1984, the German, Austrian, and Swiss Photopatch Test Group was founded to standardize the photopatch test procedure and to investigate photoallergic reactions, as well as the epidemiology of photoallergy, in central Europe. Therefore in a first test period from 1985-1990, 32 test substances were applied on the backs of patients suspected to be photosensitive. After evaluation of these data, some substances were dismissed, and others were additionally integrated into the test tray. Thus a modified test tray comprising 26 test substances was used for the second test period (1991-1997). OBJECTIVE: According to the standard photopatch test procedure defined in the first test period, the aim of this multicenter study was to apply compounds from the modified second test tray to a large group of photosensitive patients. After evaluation of the second test period (1991-1997), the outcome was compared with the results of the first period. On the basis of these results and influenced by concurrently published case reports, a third modified photopatch test tray has been established. METHODS: After the application of a duplicate test tray for 24 hours, one test site was irradiated with 10 J/cm(2) UVA, and the other test site served as the control area. Readings were performed immediately and 24, 48, and 72 hours after irradiation. Test reactions were qualitatively graded according to a 4-point scale and classified by the investigators of the participating centers. In 49 participating clinics 1261 photopatch tests were performed. All data were subjected to computer-assisted analysis by using a specially developed software to classify all positive test reactions as plain contact or photoinduced reactions (nonspecific, toxic, or allergic photoreactions) and to define substance-specific reaction patterns. RESULTS: In test period 1 data of 1129 patients were evaluated. From 2859 positive test reactions, 28.6% were excluded as plain contact reactions, 71.4% were found to be photoinduced reactions, and 3.8% were classified as photoallergic. In test period 2 data of 1261 patients were evaluated. One thousand four hundred fifteen positive test reactions were observed, and of these, 28.7% were excluded as plain contact reactions, 71.3% were classified as photoinduced reactions, and 8.1% were classified as photoallergic reactions. In both test periods nonsteroidal anti-inflammatory drugs, disinfectants, and phenothiazines represented the leading photoallergens in the evaluated central European region. By using computer-assisted reaction pattern analysis, substance-specific reaction patterns could be distinguished. These substance-specific reaction patterns comprised 4 main categories: the well-known decrescendo (phototoxic) and crescendo (photoallergic) reaction patterns, as well as a combined and a plateau pattern. CONCLUSION: The test modification after the first test period led to a notably reduced number of positive (mainly nonphotoallergic and thus nonrelevant) test reactions per patient in the second test period (from 2.6 to 1.1). In contrast, the percentage of photoallergic reactions increased significantly from 3.8% to 8.1% of all positive test reactions. Test modifications after the first test period led to a remarkably improved specificity of the photopatch test. Furthermore, substance-specific reaction patterns observed in test period 1 were confirmed in test period 2.

[UV-therapy in HIV-infected patients]. / UV-Therapie bei HIV-Patienten.

We examined the prevalence of HIV-associated cutaneous disorders as well as the frequency and efficiency of UV-radiation in HIV-infected patients by evaluating the clinical records of 1310 patients seen between and 1994 and 1996. 41 patients were treated with UVB-radiation and 4 patients with UVA1-radiation, while three patients received PUVA-therapy. UV-radiation proved to be efficient and safe in the treatment of pruritic papular eruption, atopic dermatitis and psoriasis. Although some in vitro studies and studies with transgenic mice have suggested a negative impact of UV radiation on the immunosystem of HIV-positive patients, no progression of the HIV infection due to phototherapy has been proven.

[Palpable migratory acriform erythema and lymphocytic infiltration of the skin--different presentations of the same entity?]. / Erythema migrans arciforme et palpabile und Lymphocytic Infiltration of the Skin--unterschiedliche Erscheinungsformen derselben Entität?

Palpable arciform migratory erythema of Clark (PAME) has been described as a rare member in the group of T-cell pseudolymphoma. The clinical picture of infiltrated annular erythema developing into large migrating lesions with the trunk as predilection site is distinctive from other pseudolymphomas. Because of the very similar histology and immunohistochemistry in comparison to the more frequent lymphocytic infiltration of the skin of Jessner and Kanof (LIS) doubts have been raised about the existence of PAME as an unique entity. A 46-year old patient presented with the typical picture of PAME on his trunk while showing typical lesions of LIS on his neck and face. Both diseases showed synchronous intermittent remissions in response to different therapeutic modalities. Multiple biopsies were subsequently performed and histological, immunohistochemical and molecular biological examinations did not reveal relevant differences between PAME and LIS in this patient. We conclude that the findings in this key case suggest that the T-cell pseudolymphoma PAME is not an unique entity, but a special clinical presentation of LIS.

[Acral papular lichen myxedematosus]. / Akraler papulöser Lichen myxoedematosus.

In 1986 Rongioletti introduced the term acral persistent papular mucinosis for persistent papules on the dorsum of the hands and distal forearms. Skin specimens reveal mucin deposits which resemble those in lichen myxedematosus (LM). Laboratory results are usually unremarkable. Our 54-year-old female patient showed a 2 1/2 year history of skin lesions in the typical acral distribution pattern. This entity appears to be an acral papular subtype of LM. In the classification of LM, acral papular lichen myxedematosus should replace the discrete papular form. This latter form has been well known for many years, but is not sufficiently definable and is of secondary clinical importance.

Allergic contact dermatitis from pyoctanin.

Although pyoctanin is widely used in medicine, there are hardly any reports of contact allergy to it. Most reports of side-effects refer to irritant reactions after topical application. We describe 2 patients who developed pyoctanin contact dermatitis after long-term application on damaged skin. Positive patch test results down to concentrations of 0.0025% and 0.05% aqueous pyoctanin solution, respectively, were found.

[Erysipelas after leg vein harvesting for aortocoronary bypass operation]. / Erysipele nach Entnahme von Beinvenen für eine aortokoronare Bypassoperation.

We have seen 4 patients in 1995 who showed erysipelas in the leg used for vein graft harvesting for coronary bypass surgery. None of the patients had experienced erysipelas prior to surgery. One patient had his first attack erysipelas while the others experienced recurrent disease. Venectomy puts these patients at risk for later erysipelas. Therefore careful foot hygiene, eradication for mycotic infections and adequate therapy for initial erysipelas are mandatory in cardiac high risk patients after coronary bypass surgery.

Localized cicatricial pemphigoid of the Brunsting-Perry type with transition into disseminated cicatricial pemphigoid. Report of a case proved by preembedding immunogold electron microscopy.

BACKGROUND: In 1979, Provost described two patients with the clinical features of disseminated cicatricial pemphigoid for the first time. Until now, only four additional cases of disseminated cicatricial pemphigoid have been described. Existence of diagnosis of disseminated cicatricial pemphigoid has been discussed controversially because in four cases investigated by electron microscopy the blister formation was found below the lamina densa, which is indicative of an epidermolysis bullosa acquisita. OBSERVATION: A 78-year-old woman is presented with a generalized eruption of blisters leaving behind scars that developed after a 7-year-long history of mild circumscribed recurrent blisters and scarring eruptions that had been diagnosed previously as Brunsting-Perry type of cicatricial pemphigoid. Immunofluorescence antigen mapping disclosed the blister formation above the lamina densa. Electron and immunoelectron microscopy using a preembedding immunogold technique revealed blister formation and antibody binding within the lamina lucida, predominantly below the subbasal dense plate. CONCLUSIONS: The clinical features of disseminated blistering followed by scarring, the immunofluorescence antigen mapping, and the electron and immunoelectron microscopic findings in our case for the first time clearly prove the existence of a disseminated cicatricial pemphigoid.

[Acute primary stage of HIV infection with documented seroconversion]. / Akutes Primärstadium einer HIV-Infektion mit Nachweis einer Serokonversion.

We report a 34-year-old homosexual man who developed a maculopapular, non-itchy exanthema mainly on the trunk in addition to fever up to 39.8 degrees C, general malaise, arthralgias, generalized enlargement of the lymph nodes, watery diarrhoea and weight loss. The patient was in an acute phase of the HIV infection according to standards of WHO and CDC (i.e. acute infection with duration from 3 days to 3 weeks with occasional mononucleosis-like symptoms and positive antibody tests). We documented the seroconversion from HIV-negativity to HIV-positivity 15 days after the onset of the acute illness, concomitant with the resolution of the clinical symptoms. Haematological changes were monitored during the conversion. The infection with HIV-1 was shown by the reduction of T4 helper cells (262/microliters) and the inversion of the CD4:CD8 ratio (< 0.5 during seroconversion). The patient also developed generalized candidiasis owing to the acute immunodeficiency.

[Localized cicatricial bullous pemphigoid of the Brunsting-Perry type]. / Lokalisiertes vernarbendes bullöses Pemphigoid vom Typ Brunsting-Perry.

Localized cicatricial pemphigoid of the Brunsting-Perry type is a very rare bullous condition, which has so far been reported in 51 cases. It is characterized by scarring blisters confined to the head, scalp and neck. Diagnosis can be difficult because of the discrete skin lesions, often repeatedly false-negative direct immunofluorescence, and the absence of circulating antibodies. We report on a 87-year-old male patient with the typical clinical feature of a cicatricial pemphigoid of the Brunsting-Perry type and give a review of the 51 cases published in the world literature.

Progressive multifocal leukoencephalopathy in patients with HIV infection: lack of impact of early diagnosis by stereotactic brain biopsy.

Thirteen patients with HIV-related progressive multifocal leukoencephalopathy (PML), representing an institutional incidence of 4.2%, are reported. All cases were diagnosed by image guided stereotactic brain biopsy shortly after their presentation for neurologic complaints. All patients were males; risk factors included homosexual or bisexual activity or intravenous drug use. At the time of presentation with PML, the mean T4 count was 85 (range 9-240 cells/mm3). The most common neurologic symptoms were cognitive dysfunction and aphasia, whereas gait abnormalities and disordered cognition were the most common neurologic signs. Cerebrospinal fluid analysis was helpful only to rule out other causes of CNS disease. Magnetic resonance imaging, more sensitive than computed tomography (CT) scanning, typically revealed multiple areas of increased intensity on T2 weighted images although unifocal disease was seen in 23% of patients. Despite early stereotactic biopsy and aggressive symptomatic therapy, survival of these patients was poor with a mean of 2.6 months after the onset of neurological symptoms and 2.0 months after biopsy.

[Quality control in epicutaneous testing--reproducibility in the right-left comparison]. / Qualitätskontrolle der Epikutantestung--Reproduzierbarkeit im Rechts-Links-Vergleich.

In 121 patients, 26 substances of a standard test battery were applied symmetrically on both sides of the upper back. The Finn chamber on Scanpor was the test system used, the final reading being taken on day 3 or 4. Of 166 tests, 131 were positive on both sides. Test substances and patients age had no influence. In comparison with the results in the literature, ours show a good reproducibility of 78.9%. Doubtful reactions (?+) were difficult to evaluate. These reactions deserve future research.