Clinical experiences with photodynamic therapy for recurrent malignancies of the lower female genital tract.

Photodynamic therapy (PDT) with Photofrin II was administered over a 7-year period to 17 patients with recurrent carcinoma in situ (CIS) of the vulva, vagina, and perianum. Ten patients were treated two or three times after average intervening periods of 23 and 19 months, respectively. A histologically complete response at 3 months after the PDT session was achieved for 27 of 38 (71%) anatomic sites. Ten patients of this group remain free of recurrences for periods of 2-7 years. Condylomata acuminata associated with CIS in 16 of 17 patients recurred rapidly in 7 patients after PDT. Only short-term palliative results were achieved for 4 patients treated with PDT for invasive carcinoma. Fifteen patients experienced a significant skin reaction to direct sunlight during a 7-week period post-PDT.

[Clinical manifestations of primary Sjogren's syndrome]. / Kliniske manifestasjoner ved primaert Sjögrens syndrom.

The article reviews the symptoms and signs of primary Sjögren's syndrome. It describes the clinical and laboratory investigations of such patients, and briefly discusses the occurrence of malignancy in Sjögren's syndrome.

The pathologist's view of Lyme disease.

Lyme disease is a multisystem disease caused by the spirochete Borrelia burgdorferi and is transmitted to humans primarily through Ixodid ticks. The clinical spectrum of the disease is continuing to expand while in its wake the pathology and histopathologic manifestations are being uncovered. We review the pathology of Lyme disease in man beginning with the tick bite. We present the pathologic changes of the rash, erythema migrans, as well as the neurologic, cardiac, and arthritic changes of the disease. We can expand our understanding of the immunobiology of Lyme disease by studying the interactions of B. burgdorferi in an experimental animal model.

Anti-SSA(Ro) antibody: a connective tissue disease marker.

The characteristics of 100 patients with anti-SSA(Ro) antibody were studied. By accepted criteria 34 patients had systemic lupus erythematosus, 25 had other known connective tissue diseases (CTD) and 9 had no significant evidence of a CTD. Thirty-two patients had an unclassified CTD characterized in most instances by arthritis, dermatitis, and a positive fluorescent antinuclear antibody test. The results support the concept of a continuous spectrum of autoimmune disease in which anti-SSA(Ro) antibody may be a marker and in which a variety of disorders may be found that cannot be classified by accepted criteria.

[Pulmonary histiocytosis X]. / Die pulmonale Histiocytosis X.

Four patients, aged between 17 and 56 years, were found to have pulmonary histiocytosis X. Radiologically they had reticulo-nodular increases in density and cystic changes in the lungs, especially in the upper fields. Pneumothorax and bone lesions were common. Despite the employment of transbronchial lung biopsies, open lung biopsy was almost always necessary to confirm the diagnosis. Broncho-alveolar lavage in one patient revealed 28% lymphocytes: T-suppressor cells predominated over T-helper cells (49% and 45%, respectively). Cell distribution returned to normal after steroid treatment.

Variations in host responses and the pathogenesis of human onchocerciasis.

Different immune responses to Onchocerca volvulus cause considerable variation in clinical manifestations of human onchocerciasis. Onchocercal lesions result from inflammatory reactions involving immunologic mechanisms; the role of the immune system in pathogenesis is emphasized by the phenomena accompanying accelerated worm destruction during microfilaricidal chemotherapy (e.g., eosinophilia, changes in total immunoglobulin level, and anaphylactic symptoms). Although most pathologic changes are associated with the microfilarial stage, the extent to which circulating antibodies are directed against antigens in the adult worm or its uterine constituents is unknown. Microfilarial destruction can be mediated by antibody to the surface-associated antigens of the worms and enhanced by complement; a correlation exists between the presence of these antibodies and punctate keratitis. Heterogeneous immunologic components are associated with the surface of dermal and nodular microfilariae in vivo. Preliminary findings indicate that the level of O. volvulus-specific immune complexes is inversely proportional to the microfilarial load. To monitor a patient's clinical status and immunologic response, a quantitative system dividing symptoms into those associated with active responses to the microfilariae and those representing long-term consequences of these reactions is suggested.

Cutaneous manifestations of serum sickness in patients receiving antithymocyte globulin.

We have prospectively evaluated the cutaneous manifestations of serum sickness in thirty-five patients treated with horse antithymocyte globulin for bone marrow failure. Twenty-one patients (21/35) were treated with antithymocyte globulin (15 mg/kg/day) for 10 days, and fourteen of thirty-five patients were treated with antithymocyte globulin (15 mg/kg/day) for 14 days and then every other day for an additional 14 days. Clinical evidence of serum sickness developed in thirty patients and included fever and malaise (100%), cutaneous eruptions (93%), arthralgias and myalgias (67%), gastrointestinal complaints (67%), and lymphadenopathy (13%). Cutaneous findings consisted of morbilliform eruptions (n = 19), urticaria (n = 1), or a combination of these two reaction patterns (n = 8). Cutaneous manifestations of serum sickness began on day 7 +/- 1 and lasted for 12 +/- 2 days for the group as a whole. Biopsies of lesional skin revealed mild perivascular lymphohistiocytic infiltrates by light microscopy in these leukopenic patients. Direct immunofluorescence microscopy of lesional skin from patients with serum sickness demonstrated immunoreactants in seven of nine subjects (78%). Immunoreactants were confined to the walls of dermal blood vessels and consisted of IgM (7/9), C3 (6/9), IgE (5/9), and IgA (4/9). IgG (horse or human) was not identified in any of these specimens. Twenty-one patients (21/28) also developed an erythematous eruption on the sides of the fingers, toes, palms, and soles 12 to 48 hours prior to their morbilliform eruption. This study describes the cutaneous manifestations of human serum sickness occurring during therapy with horse antithymocyte globulin, documents a cutaneous sign of serum sickness, and suggests that the cutaneous eruptions associated with human serum sickness are immunologically mediated.

Clinical findings in four children with biotinidase deficiency detected through a statewide neonatal screening program.

Four children with biotinidase deficiency were identified during the first year of a neonatal screening program for this disease in the Commonwealth of Virginia. Two unrelated probands were identified among the 81,243 newborn infants who were screened. In addition, two siblings of one of these infants were found to be affected. Both probands had mild neurologic symptoms at two and four months, respectively, and the two older children had more severe neurologic abnormalities, cutaneous findings, and developmental delay at two and three years of age. However, none of the affected children had acute metabolic decompensation. Previous studies have shown that the administration of biotin to affected children can be a lifesaving procedure that can reverse acute symptoms and prevent irreversible neurologic damage. Our findings demonstrate that subtle neurologic abnormalities may appear as early as at two months of age and that developmental abnormalities may occur even in the absence of episodes of overt metabolic decompensation. Since screening and treatment are both inexpensive and effective and the incidence of the disease is well within the range of that of other metabolic diseases for which screening is performed, biotinidase deficiency should be added to the group of metabolic diseases for which screening is done in the neonatal period.

Cutaneous necrotizing vasculitis complicating Takayasu's arteritis with a review of cutaneous manifestations.

We report a case of a 32-year-old man with chronic active Takayasu's arteritis complicated by cutaneous necrotizing vasculitis. Symptoms of the early phase of his disease coexisted with complications of the late phase.

Preliminary clinical observations with recombinant interleukin-2 in patients with AIDS or LAS.

The toxicity of recombinant Interleukin-2 (IL-2) was studied in patients with acquired immunodeficiency syndrome (AIDS) or persistent lymphadenopathy syndrome (LAS). Increasing doses of the drug from 10(3) Units/m2 to 10(6) U/m2 were given as an intravenous bolus injection. At the high-dose levels some minor effects, such as fever up to 39.5 degrees C, chills, malaise or vomiting, were observed. The administration of 10(6) U/m2 as a 4-hour infusion showed identical results. No particular alterations of laboratory parameters were found. At the high-dose level the serum concentration of neopterin, which is released from macrophages after interferon gamma stimulation, was significantly (p less than 0.001) elevated above pretreatment levels. The clinical observation of daily infusions of 10(6)/m2 for 14 days revealed the same side effects. All patients developed lymphocytosis and eosinophilia. Two patients had suffered from severe diarrhoea for several weeks presumably due to cryptosporidiosis. In both cases diarrhoea ceased under the treatment with IL-2 and did not occur in the following two months.

Skin reactions to foods in patients with atopic dermatitis.

Thirty-seven patients with moderate or severe persistent atopic dermatitis gave a history of skin reactions following the ingestion of certain foods. These reactions fell into three groups: immediate reactions (within one hour) which included angio-oedema, contact urticaria, generalised itching and erythema, or urticaria; late reactions, where patients experienced late urticaria, late angio-oedema, itching, or exacerbation of the dermatitis; dual reactions where immediate reactions were followed by exacerbation of the atopic dermatitis. The foods implicated were either potential allergens or recognised nonimmunological histamine-release agents. Foods which caused an IgE-mediated reaction tended to produce early or dual reactions whereas histamine releasing foods tended to result in late reactions.

Lightning injuries.

The physical properties of lightning are given, including a description of the different observed lightning forms. The wide variety of effects of lightning on humans is reviewed. In the prehospital care of those struck by lightning, emphasis is upon immediate resuscitation of those who appear unresponsive. Recommendations for emergency department evaluation, treatment, and disposition are given. Guidelines to prevent humans from being struck by lightning are discussed.

[Clinical-dispensary finding of patients with primary biliary cirrhosis]. / Klinichno dispanserno nabliudenie pri bolni s purvichna biliarna tsiroza.

Ten patients with primary biliary cirrhosis (PBC) were clinically followed under the conditions of follow-up care. The diagnosis of the patients was made vie biochemical immunologic, histological and instrumental investigations. The average term for follow up was 4 years and 5 months. All patients were actively checked up by summoning every 6 months. The average incidence of hospitalization at the clinic was 1 year and 5 months. Pruritus preceded all other symptoms--1 year and 11 months on the average. Jaundice was established 1 year and 7 months after the manifestation of pruritus and the dirty greyish pigmentation of the skin--1 year and 11 months after pruritus. Hepatomegaly was established 1 year and 7 months after pruritus. Increased alkaline phosphatase, blood bilirubin and cholesterol were observed in all patients and increase of IgM in 80 per cent, and antimitochondrial antibodies--in 70 per cent. Xanthelasma and xanthoma were found only in patients with high levels of cholesterol and total fats. The treatment with dihydrocortison induced stomach complaints (erosive gastritis), intersifying bone chanes in 20 per cent of the patients. X-ray and instrumental methods have a relative value in making the diagnosis of PBC. It could be concluded from the complex treatment that corticosteroids give complications and the treatment with cholestiramine--to attenuation of pruritus. The average survival after making the diagnosis is 6 years.

Vibration syndrome in industry: dermatological viewpoint.

Long-term use of hand-held vibratory tools has been implicated in the development of a clinical condition known under several names including occupational Raynaud's phenomenon, vibration-induced white finger (VWF) disease, and "dead" or "wax" finger. The syndrome is characterized in its early stages by tingling, numbness, or blanching of the finger tips provoked usually by exposure to cold temperatures; later these symptoms may extend to the base of all of the digits on both hands. As vibration exposure continues, the attacks become more frequent and cause manual impairment and social disability. This complex of VWF and associated arterial and related complications is now termed vibration syndrome (VS). Although epidemiologic studies indicate that large percentages of the population of workers at greatest risk are affected, the acceptance of VS as an industrial disease is only recent. This paper reviews some of the salient features of VS from the point of view of the dermatologist, since he may be the first health professional to see patients with this syndrome. Terminology, risk factors, preventative measures, therapy, and occupational guidelines are discussed.