Familial pemphigus vulgaris in mother and daughter.

BACKGROUND: Pemphigus vulgaris is an autoimmune disease in which genetics appears to be of basic importance. Although association with certain human leukocyte antigen (HLA) alleles has been found in some ethnic groups and individuals, no true disease susceptibility genes have been established, and familial cases are very unusual. METHODS: We report a Polish family with pemphigus vulgaris in the mother and daughter. The diagnosis was confirmed by cytologic, histologic, and immunofluorescence studies. RESULTS: The course was severe and the disease long-lasting in the mother, probably due to treatment with small doses of corticosteroids without immunosuppressive drugs. In the daughter, treated with larger doses of corticosteroids and azathioprine, the lesions regressed within 4 months, after which maintenance therapy was instituted with 10 mg of prednisone daily. The HLA studies performed in the daughter and her three children after the mother had died showed identical haplotypes in both the patient and the healthy children. The patient has given birth to a healthy child while still having a high titer of intercellular (IC) antibodies. CONCLUSIONS: The familial occurrence of pemphigus in first-degree relatives is suggestive of inherited susceptibility to the disease, transmitted as a dominant trait. The identical haplotypes in the healthy children of the patient favor the role of other, unknown factors required for the development of the disease in predisposed individuals.

[Chancriform pyoderma as a clinical problem: observation of 45 patients treated at the Dermatology Clinic, Medical Academy, in Cracow 1962-1986]. / Pyodermia chancriformis jako zagadnienie kliniczne: obserwacja 45 chorych leczonych w Klinice Dermatologii AM w Krakowie w latach 1962-1986.

A clinical, bacteriological and serological pictures of 45 patients with chancriform pyoderma are presented, A single ulcer (92.8%), localized on genitals (91.1%) in adult males (93.3%), imitating or very similar to syphilitic primary chancre in our material was observed. A second case in the literature, of chancriform pyoderma on the dorsal surface of the tongue was recorded.

[Results of the treatment of neurosyphilis with high doses of crystalline and procaine penicillins]. / Wyniki leczenia kily ukladu nerwowego wysokimi dawkami penicyliny krystalicznej i prokainowej.

The results of the treatment in two groups of patients with neurosyphilis are compared. In 23 cases, i.v. infusions of crystalline penicillin were given for 10 days in daily dose of 20-24 mega U., combined with probenecid. Then it was followed by i.m. procaine penicillin in daily dose of 1.2 mega U., in total dose 12-50 mega U. The mean duration of follow-up was 7,8 months. The second group (34 cases) was treated with procaine penicillin i.m. in daily dose of 1.2 mega U., the total dose was 24-60 mega U. The mean duration of follow-up was 2.5 years. The differences between the results of the treatment in both groups were not significant. In 3 reinfected patients with early asymptomatic neurosyphilis in spite of intensive treatment with penicillin sero- and cerebro-spinal fluid-resistant syphilis developed.

[Clinical picture of early syphilis in patients with HIV infection in the light of literature data]. / Obraz kliniczny kily wczesnej u osób zakazonych retrowirusem hiv w swietle danych z pismiennictwa.

A literature survey is presented concerning the clinical pattern of early syphilis in subjects infected with HIV. The course of syphilis infection in HIV-positive subjects differs often from the usual one, the clinical picture may be that of the so called malignant syphilis with early development of syphilitic lesions in the central nervous system and involvement of the visual system; Serological reactions differ also, they may give negative results or may appear with delay, sometimes high titres of these reactions are found.

[Cutaneous tuberculosis treated at the Dermatological Clinic, Medical Academy, in Cracow 1963-1987. I. Epidemiological analysis]. / Gruzlica skóry w materiale chorych leczonych w Klinice Dermatologicznej AM w Krakowie w latach 1963-1987. I. Analiza epidemiologiczna.

In the last 25 years 268 (67%) patients were hospitalized for proper tuberculosis of the skin, and 132 (33%) for tuberculids. In the period 1983-1987 the prevalence of skin tuberculosis was 5.8 times lower than in the period 1963-1967. The frequency of the clinical forms of tuberculosis was as follows: tbc. luposa (57.5%), tbc. colliquativa (35.4%), tbc. verrucosa (4.5%) and tbc. ulcerosa (2.6%). Women had tbc. luposa 1.4 times, and tbc. colliquativa 1.5 times more frequently than men, while in men tbc. verrucosa was 3 times and tbc. ulcerosa 2.5 times more frequently than in women. In the whole group the male:female ratio was 1:2.05. In patients aged up to 20 years tbc colliquativa was more frequent (29.5%) than tbc. luposa (5.2%), while in the age group 61-85 years this frequency was reversed: 17.9% and 34.4% respectively. In the division into occupational groups farmers prevailed (3.6%) (t = 2.84). The number of female patients with tbc. luposa significantly less frequently (t = 4.0) got married, probably due to disfiguring changes following the disease.

[Hyperplasia of gingivae in the course of incomplete form of Melkersson-Rosenthal syndrome]. / Rozrost dziasel w przebiegu niepelnej postaci zespolu Melkerssona-Rosenthala.

A case of incomplete Melkersson-Rosenthal (s. M-R) syndrome manifesting itself by the lower lip and cheeks' mucous membrane oedema and hyperplasia of gingivae has been described. Histopathological examination of the cheeks has demonstrated disseminated, perivascular inflammatory infiltrations of sarcoid type, whereas massive infiltration composed nearly exclusively of plasmatic cell has dominated in segments of gingivae. Electron microscopic examination has demonstrated increased migration of the cells resembling lymphocytes through the epithelium towards the surface and progressive morphological changes leading to the disintegration both lymphocytar cells and plasmatic ones and symptoms of interstitium fibrotic processes.

Ringworm-like late syphilides.

A peculiar form of late, superficial, serpiginous, non-ulcerative syphilides, leaving no visible scars, resembling clinically the ringworm of the glabrous skin, showing the granulomatous histopathological structure and relatively abundant T. pallidum, predominantly spread around the blood vessels is described.