Coexistence of malignancies with bullous pemphigoid.
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Female↗
Biomedical subjects
S Jablonska
Publications and source records attributed to S Jablonska.
Characterization of two types of human papillomaviruses in lesions of epidermodysplasia verruciformis.
Human papillomaviruses (HPVs) found in lesions of 11 patients suffering from epidermodysplasia verruciformis were compared to HPV type 1 (HPV-1) and HPV type 2 (HPV-2) previously characterized in plantar and common warts, respectively. Complementary RNAs (cRNAs) to HPV-1, HPV-2, and viruses obtained from two patients with epidermodysplasia verruciformis (J.D. HPV and J.K. HPV) were used in cRNA.DNA filter hybridization experiments. No sequence homology was detected between HPV-1 or HPV-2 DNAs and DNAs obtained from the 11 epidermodysplasia verruciformis HPV isolates. Furthermore, with J.D. and J.K. HPV cRNAs, epidermodysplasia verruciformis HPV DNAs fell into two groups showing little, if any, sequence homology. A lower extent of annealing was observed for the DNAs of some isolates showing a genetic heterogeneity within each of the two groups. Almost no antigenic crossreaction was detected by immunodiffusion and indirect immunofluorescence tests, either between epidermodysplasia verruciformis HPVs and HPV-1 or HPV-2 or between J.D. and J.K. HPVs. Viruses belonging to the same group have common antigenic properties, but antigenic differences were observed when two of the viruses sharing only partial DNA sequence homology were compared. Viruses related to J.D. HPV were preferentially associated with flat wart-like lesions of epidermodysplasia verruciformis and were further found in the lesions of five patients bearing multiple flat warts. Viruses related to J.K. HPV were found in morphologically distinct lesions (red spots) present in some patients with epidermodysplasia verruciformis. Thus, we propose to distinguish two other types of HPVs designated provisionally as HPV type 3 (HPV-3) and HPV type 4 (HPV-4), with J.D. and J.K. HPVs as prototypes, respectively. Malignant conversion of some epidermodysplasia verruciformis lesions is more frequently associated with HPV-4 than with HPV-3 infection.
Adult↗
The human papillomaviruses.
Recent biochemical and serological studies have shown the existence of at least four distinct types of human papillomaviruses (HPVs) causing benign skin lesions. These viruses show hardly no antigenic relationships; their DNAs differ by their sensitivity to restriction endonucleases, and show little, if any, sequence homology, as detected by molecular hybridization using complementary RNAs transcribed in vitro. Data on the pathogenicity of HPVs are still incomplete but indicate that some types of benign skin lesions (plantar warts, common warts, flat warts) may be preferentially associated with some types of HPV. Most interesting is that epidermodysplasia verruciformis has been found associated with two types of virus, and that malignant conversion of some lesions has been observed in all the patients infected with one of them. This suggests that at least a HPV may have a higher oncogenic potential, as do rabbit (Shope) papillomavirus and bovine alimentary tract papillomavirus. Much remains to be known on human papilloma-viruses and further studies may lead to the characterization of additional types of HPVs, especially in genital condylomata acuminata and laryngeal papillomas whose malignant conversion, although rare, may be observed. Progress in this field has been and remains hampered by the lack of cell culture systems allowing replication of these highly host and tissue specific viruses, and by the widely variable virus content of the different human lesions known to be associated with a papillomavirus. Further studies are warranted by the possible role of these widespread and epitheliotropic viruses in the origin of some carcinomas in man.
Cell Transformation, Neoplastic↗
Immunological studies in epidermodysplasia verruciformis.
Immunofluorescence and cell mediated immunity studies have been performed in 14 cases of epidermodysplasia verruciformis (EV), 3 of those abortive or regressing in members of the families of the patients with EV. Two different types of human papillomavirus (HPV)--HPV3 and HPV4--have been found in cases of EV. HPV3 was detected also in flat warts without features of EV. There was no cross-reactivity between these two viruses, neither with HPV1 responsible for plantar warts nor with HPV2 inducing common warts. There was a relationship between the type of HPV and the clinical picture of EV as well as the malignant transformation, namely HPV4 has been found to be more oncogenic. Cell mediated immunity (CMI) seems to be an important factor because it was depressed in a vast majority of active cases and preserved in regressing and abortive cases (in the members of the families of EV patients). However, low CMI has been found in EV cases infected with HPV3 and in persistent flat warts also due to HPV3, which did not undergo malignant transformation. In contrast, in a case of EV due to HPV4 a malignant transformation occured in spite of still preserved, although lowered CMI. Various human papillomaviruses seem to differ in their oncogenic potential. HPV1 responsible for plantar warts, and HPV2 for common warts have no evident oncogenic potential, HPV3 inducing both EV and flat warts has a low oncogenicity, whereas HPV4 inducing some cases of EV seems more oncogenic.
Adult↗
[Comments on idiopathic and sclerodermic hemiatrophia faciei with generalized myopathy. Published by U. Runne and K. Fasshauer, Hautarzt 28, 10--17 (1977)].
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Electromyography↗
Substrate specificity of antinuclear antibodies in scleroderma.
Studies of antinuclear antibodies (ANA) were carried out in 39 cases of systemic scleroderma and for comparison in 19 cases of systemic lupus erythematosus (SLE) and 4 of mixed connective tissue disease (MCTD) using indirect immunofluorescence (IF) methods under standard conditions. The results on three different substrates--monkey esophagus, guineapig lip and rat liver--are reported. In 48.7% of scleroderma cases ANA showed a substrate specificity. The highest percentage of positive results in scleroderma was obtained on monkey esophagus (97.4%) and the lowest on rat liver (61.5%). In SLE and MCTD, in contrast, only about 13% of the sera displayed such specificity. If only sera with substrate specificity are considered, the positive results on monkey esophagus and rat liver are 94.7% and 21.1%, respectively. Titers of sera reacting positively on 2 or 3 substrates were mostly in agreement, although some sera both in systemic scleroderma and SLE showed higher titers on monkey esophagus. The IF pattern was usually the same regardless of the substrate, Tests for ANA in scleroderma should be performed on at least 2 substrates simultaneously.
Adult↗
Dermal toxicity of 8-methoxypsoralen administered (by gavage) to hairless mice irradiated with long-wave ultraviolet light.
Hairless mice were administered various amounts of 8-methoxypsoralen (8-MOP) by gavage, followed by irradiation with ultraviolet light (UVA) two or more times per week for periods ranging from 1 to 12 months. The minimum phototoxic dose was 20 mg/kg body weight by this route of administration and potential for serious organ toxicity in long-term exposures was investigated. No histologic features of cutaneous malignancy were encountered under test conditions which produced prolonged phototoxicity, deep ulceration, cicatrization, and other deformities. Repeated daily gavaged doses of 20 mg psoralen/kg body weight in conjunction with twice weekly irradiation for 10 min with UVA elicited an erythematous phototoxic reaction, but did not give rise to subsequent skin lesions. 8-MOP in repeated daily gavage doses of 30 mg and 40 mg/kg body weight combined with twice weekly UVA irradiation for 10 min caused severe burning with subsequent scarring, but did not induce malignant tumors in experiments lasting lasting 8 months. No organ toxicity was seen except for toxic liver changes when severe cutaneous burn and pronounced ulcerations were produced. Limited immunologic studies disclosed no abnormalities in this system.
Animals↗
5-Hydroxytryptamine and tryptamine pathways in scleroderma.
Levels of 5-hydroxyindoleacetic acid, indoleacetic acid and total indoles were determined in the urine of 23 patients with systemic scleroderma and 7 patients with cutaneous scleroderma, before and after peroral loading with L-tryptophan (0-1 g/kg body weight). Before loading, 5-hydroxyindoleacetic acid levels were normal in nearly all cases of systemic scleroderma as well as of cutaneous scleroderma; however after loading, in nearly one half of cases there was no normal increase of this metabolite. These results suggest impaired transformation of serotonin into 5-hydroxyindoleacetic acid. A disproportionately high ratio of total indoles to indoleacetic acid suggests the presence of excess of tryptamine. The results of the study may indicate that in scleroderma metabolism of biogenic amines derived from tryptophan is abnormal, probably as a result of impaired activity of monoamine oxidase.
Adolescent↗
Cell-mediated immunity in epidermodysplasia verruciformis.
Investigations were performed in 6 cases of epidermodysplasia verruciformis and 2 healthy family members. Nonspecific cell-mediated immunity (CMI) was studied by measuring response to phytohemagglutinin (PHA) and concanavalin A (Con A), percentrages of E- and EAC-rosette-forming lymphocytes, bacterial skin tests, and allergic reactions to dinitrochloro-benzene (DNCB). Impairment of CMI was manifested by reduction in the percentage of E rosettes, and lowered response to PHA, and- to a lesser degree- to Con A. The immune response to DNCB sensitization was invariably negative. Impairment of CMI was greater in cases of long duration and with extensive lesions. The cases of similar duration and extent of lesions, which never showed tendency to tumor formation, were not different in CMI in comparison with cases with numerous tumors. Only in cases with very advanced tumors CMI was impaired parallel to the gravity of the patient's general condition.
Adolescent↗
Dermatitis herpetiformis and bullous pemphigoid. Intermediate and mixed forms.
Nine patients had clinical and histological features suggestive of both dermatitis herpetiformis (DH) and bullous pemphigold (BP). Five patients responded to treatment with sulfapyridine or sulfones: in two the response was inconsistent, and the disease was controlled by combined treatment with prednisone; in one patient, there was no response to sulfapyridine or sulfones. Immunofluorescence studies showed IgA deposits in a linear homogeneous pattern at the basement membrane zone in all patients, and IgG was present in five. No circulating anti-basement membrane antibodies were detected by repeated immunofluorescence examinations. The authors conclude that the occasional overlapping of BP and DH should not lead to dropping the distinction between the two entities. For overlap cases that cannot be classified as BP or DH, the term "intermediate or mixed form of DH and BP" seems to be most suitable.
Aged↗
[Mixed bullous diseases].
As mixed bullous diseases are presented the intermediate forms between bullous pemphigoid (BP) and dermatitis herpetiformis (DH) with clinical and histologic features of both diseases, and with linear deposits composed exclusively or mainly of IgA at the basement membrane zone in the direct immunofluorescence test. This type of mixed bullous disease is more common in children (juvenile DH), although typical DH and BP cases as in adults occur also in childhood. Also herpes gestationis seems to be related to mixed bullous diseases with prevalence of immunologic phenomena characteristic of BP, although its IF pattern may be regarded as diagnostic (usually absence of circulating and in vivo bound antibasement antibodies, and presence of some complement components at the dermal-epidermal juncti0n). Especially controversial is in this group a variety of pemphigus with clinical features of DH, and good response to sulfapyridine of some cases, presenting acantholysis and immunological phenomena specific for pemphigus. For this form of pemphigus the term herpetiform pemphigus has been proposed. In the group of mixed bullous diseases should also be included cases of coexistence-in different periods, or concomitantly-of pemphigus and BP.
Adolescent↗
Herpes gestations with identical lesions in the newborn. Passive transfer of the disease?
Cutaneous lesions of herpes gestation is developed in a newborn on the fifth day of life and disappeared spontaneously after three weeks. The immunologic phenomena were analogous in the mother and child. No circulating or in vivo-bound immunoglobulins were demonstrated, but components 3 and 4 of complement were present at the basement membrane zone. We discuss a possible passive transfer of the disease to the newborn, and a supposedly pathogenetic role of immunologic phenomena in herpes gestationis.
Adult↗
Annular atrophic plaques of the face. A variety of atrophic discoid lupus erythematosus?
In a patient whose condition was diagnosed clinically and pathologically as annular atrophic plaques, the lesions bore some resemblance to morphea, lichen sclerosus et atrophicus, and discoid lupus erythematosus (DLE). In a lesion that appeared recently, which was not DLE clinically, the histological characteristics were consistent with DLE, and immunofluorescence examination disclosed an immunofluorescent band at the epidermal-dermal zone. We suggest that annular atrophic plaques may be an atypical variant of DLE, with a tendency to sclerosing.
Facial Dermatoses↗
Immunologic phenomena in herpes gestationis.
Immunofluorescence findings are of diagnostic significance in herpes gestationis. The studies in two cases demonstrated some complement components (chiefly C3 and C4, but no C(1q)), without immunoglobulins at the basement membrane zone and with no circulating anti BMZ antibodies. In one case IgG were found at the BM zone in one biopsy specimen, but were not demonstrable in several other biopsies. The IF findings seem to indicate both the alternate and classical pathways of complement activation, and speak in favor of a relation between herpes gestationis and bullous pemphigoid, in which sometimes the immunoglobulins are also not demonstrable and the immunofluorescent pattern is very much like that of herpes gestationis.
Adult↗
Diagnosis of systemic lupus erythematosus. Importance of antinuclear antibody titers and peripheral staining patterns.
Titers and patterns of antinuclear antibodies (ANA) in sera from 134 normal blood donors, 20 patients with rheumatoid arthritis, 15 patients with systemic scleroderma, and 32 patients with diagnosed or suspected systemic lupus erythematosus (SLE) were studied. The difference between the findings with sera of patients with SLE and normal subjects in terms of high (greater than 160) titers of ANA was greater than in terms of peripheral staining patterns. However, in comparing sera from patients with SLE with sera from patients with other connective tissue diseases, greater differences were found in the incidence of peripheral patterns of ANA compared to differences in the frequency of high ANA titers. Maximum specificity in the diagnosis of SLE was achieved when both titers and patterns of ANA were considered.
Adult↗
Diagnostic importance of immunofluorescence in oral bullous diseases and lupus erythematosus.
Immunofluorescent tests have proved to be of diagnostic importance for pemphigus, bullous pemphigoid, cicatricial pemphigoid, systemic lupus erythematosus, and discoid lupus erythematosus. Immunofluorescence test procedures, necessary specimens, and test findings have been reviewed as aids to dentists in the utilization and interpretation of these tests for the study of oral lesions.
Antibodies, Antinuclear↗
Juvenile dermatitis herpetiformis versus "benign chronic bullous dermatosis of childhood." Are these immunologic diseases?
Seven cases of juvenile dermatitis herpetiformis have been investigated. Immunofluorescence and histologic studies were made in all and jejunal biopsies in three. Immunopathologic results were positive in all cases including one that had previously been reported to be negative. Two groups could be distinguished according to clinical and histologic criteria, response to sulfapyridine, and character of the immunoglobulin deposits. The first corresponded to dermatitis herpetiformis (DH) of adults, with characteristic lesions of the jejunal mucosa; the second corresponded either to bullous pemphigoid (BP), although in the majority of the cases without circulating antibasement-membrane antibodies, or to a mixed type with the combined features of DH and BP. Repeated biopsies with serial sections are essential for demonstrating immune deposits. The question arises whether any immunologically negative cases of "benign chronic bullous dermatosis of childhood" actually exist.
Adult↗