Helix-coil transitions in DNA using a pH variation method: case of a melting paradox as a function of ionic strength

We describe a 31-year-old Japanese woman with generalized pustular psoriasis treated with PUVA who subsequently developed a bullous disease. Throughout the disease course, there was no phase of psoriasis vulgaris. Although several reports describe coexistence of psoriasis vulgaris and bullous disease such as bullous periphigoid, coexistence of generalized pustular psoriasis without any phase of psoriasis vulgaris and bullous disease is rare. As for the bullous disease, direct immunofluorescence study showed IgG and C3 deposition along the basement membrane zone. Indirect immunofluorescence disclosed IgG antibasement membrane zone antibodies. Indirect immunofluorescence on 1 mol/l sodium chloride-split skin demonstrated linear IgG staining almost exclusively on the dermal side of the split. Western immunoblot analysis revealed that the antibody was directed to neither epidermolysis bullosa acquisita antigen nor bullous pemphigoid antigens. Considering the unusual clinical course, we suspect the possibility of a novel autoimmune blistering disease.     

In-vitro photobactericidal activity of aminoacridines

We report four patients with severe erythrodermic, pustular psoriasis, or plaque-type psoriasis, who were treated with a combination of acitretin and bath PUVA. After 4 weeks out-patient treatment, the psoriasis in all patients had improved by > or = 90%. No patient had relapsed when reviewed at 3 months. No significant side-effects were seen with the combined retinoid/bath PUVA treatment. Acitretin and bath PUVA may be safely combined for the treatment of severe psoriasis.     

A patient with psoriatic arthritis due to generalized pustular psoriasis (von Zumbusch type)

Pustular psoriasis is a rare skin disease that is observed in about 1% of all patients with psoriasis. We encounted a patient with psoriatic arthritis (PsA) due to pustular psoriasis. The patient was a 31-year old male. He visited our hospital due to generalized eruption and pain in multiple joints. Treatment was initiated under a diagnosis of psoriasis vulgaris and associated PsA. However, eruption extended to the entire body and became pustular, and fever developed. Since PsA symptoms were simultaneously aggravated, and body movements become difficult, he was admitted. A diagnosis of generalized pustular psoriasis (von Zumbusch) and associated symmetrical polyarthritis was made. Local therapy was performed. As systemic treatment, oral administration of an corticosteroid and weekly low-dose pulse methotrexate therapy were performed. The skin symptoms and PsA symptoms rapidly improved. At present, about one year after the initiation of treatment, the eruption almost completely disappeared, and joint pain does not present any problem in daily life.     

Exfoliatio areata linguae et mucosae oris: a mucous membrane manifestation of psoriasis pustulosa?

Lesions of the oral mucosa are frequently described in association with psoriasis, particularly in the pustular type. Controversy surrounds the question whether mucosal lesions can be considered as oral manifestation of psoriasis. Two patients presented with concurrent pustular psoriasis and mucosal lesions with the characteristic picture of geographic tongue. Histopathology of the mucosa showed typical features of psoriasis such as marked acanthosis, clubbing of the rete ridges, focal parakeratosis and neutrophilic infiltrates. There was parallel improvement of the skin and the mucosal lesions with systemic retinoid treatment. On the basis of the histopathological features and the clinical course we favour the hypothesis that geographic tongue is an oral manifestation of pustular psoriasis.     

Twin sisters with impetigo herpetiformis

Impetigo herpetiformis is a rare and serious pustular eruption resembling pustular psoriasis that mostly occurs in pregnant women. The etiology of the disease is unclear and it is not known if genetic factors play a role. We present two sisters (twins) with impetigo herpetiformis supporting an inheritance pattern.     

Okadaic acid enhances prostaglandin E1-induced alkaline phosphatase activity in osteoblast-like cells: regulation at a point downstream from protein kinase A

A variety of pharmacologic agents have been known to induce pustular psoriasis. We describe a patient with a positive personal and family history of psoriasis who developed an extensive annular pustular eruption 3 weeks after starting hydroxychloroquine (Plaquenil) for arthritis. The drug was discontinued, and she received 3 weeks of systemic and topical corticosteroids; in spite of the therapeutic intervention, showers of new lesions appeared daily, and progressed to involve 75% of the body. The development of new lesions stopped, and the older lesions began to clear after one dose of 7.5 mg of methotrexate. Subsequently, methotrexate therapy was stopped because of mild transaminase elevation; the pustular lesions then flared. New lesions stopped appearing after four doses of weekly methotrexate. The patient remains clear of lesions 6 months later.     

Socioeconomic status and HRT use

A two-month-old female German shepherd dog was presented with a history of ulcerated central pads of all four feet. On physical examination, the footpads were swollen, soft and ulcerated. On the nasal planum there was a partially depigmented nodule. Multiple biopsies were taken from the cutaneous lesions and histological examination revealed focal collagen degeneration and vasculitis. A diagnosis of familial cutaneous vasculopathy was made. Seven months later the nasal nodule had disappeared and the footpads were still swollen but non-ulcerated. At this time, the dog manifested a generalised pustular demodicosis.     

High concentrations of ammonia, but not volatile amines, in gastric juice of subjects with Helicobacter pylori infection

A variety of external stimuli are accepted as important in modifying the severity of psoriasis. We sought to determine whether there is any difference in the influence of external factors on psoriasis in relation to extent of involvement or clinical type. A total of 870 psoriasis patients seen between 1982 and 1995 were categorized as mild, moderate, or severe on the basis of extent of the disease, and as guttate, nummular/plaque, or exfoliative/generalized pustular according to clinical type. We then performed a questionnaire survey concerning the influence of external factors such as seasonal changes, sunlight, stress, and pregnancy. These data sets were combined and analysed. The majority of patients stated favorable effects of summer, sunlight, and pregnancy and adverse effects of winter and stress. A statistically significant correlation was noted between the extent of psoriasis and the proportion of patients stating that their disease worsened at times of psychological stress (p < 0.01). We confirmed that psoriasis patients with more extensive involvement experience greater fluctuations in their condition, notice these changes, and therefore relate them to psychological stress.     

Methotrexate for psoriasis

Methotrexate is an effective antipsoriatic agent and has been widely used to treat severe psoriasis since the 1960s. It is especially useful in acute generalized pustular psoriasis, psoriatic erythroderma, psoriatic arthritis and for extensive chronic plaque psoriasis in patients who are inadequately controlled by topical therapy alone. It has not, however, been formally compared with other systemic treatments for severe psoriasis such as cyclosporin, retinoids or photochemotherapy with psoralen and UVA (PUVA), but in comparison with these other therapies it is inexpensive, with correct use, its safety profile is favourable. In summary, therefore, it can be used as a short-term option to gain control of unstable psoriasis such as pustular psoriasis or erythroderma before returning to other modes of treatment, or more often, as long-term maintenance treatment. The most important potential side-effect is acute myelosuppression, which is the cause of most of the rare deaths attributable to this therapy for psoriasis. Myelosuppression is more likely in the elderly, in patients with renal impairment and/or folate depletion, and with overdose or drug interactions. Long-term therapy carries with it a risk of liver fibrosis which is related to the dosage regimen employed, and is increased by exposure to other hepatic toxins, particularly alcohol. The correlation between the risk of development of liver fibrosis, cumulative lifetime dose and duration of treatment with methotrexate is not clear-cut, but may have been overstated in some studies.     

Transfer of autoimmune thyroiditis and resolution of palmoplantar pustular psoriasis following allogeneic bone marrow transplantation

We report an unusual case of a patient who was cured of one autoimmune disease (palmoplantar pustular psoriasis (PPP)) but developed another autoimmune disease (autoimmune thyroiditis) after allogeneic BMT. A 40-year-old man suffering from AML with PPP underwent allogeneic BMT from his HLA-identical sister for the treatment of AML. The patient experienced complete clearance of the cutaneous PPP despite the cessation of immunosuppressive therapy for over 2 years. However, he developed hyperthyroidism with anti-thyroglobulin antibodies 5 months after BMT, although he had showed normal thyroid functions without anti-thyroglobulin antibodies before BMT. The donor had no history of thyroid diseases and showed normal thyroid functions but was positive for anti-thyroglobulin antibodies. Thus, even when the donor is in a subclinical state, autoimmune thyroiditis may be transferred from donors to recipients by BMT.     

Dermoid in the filum terminale of a newborn with myelomeningocele

Tumor necrosis factor-alpha (TNF-alpha) has been implicated as one of the critical mediators of psoriasis. Evidence for an important role on the progression of the disease is increasing, while recent clinical studies have suggested its beneficial role in the disease. Based on the results of our sequential analysis of the serum cytokine levels in a patient with pustular psoriasis, we speculate that immunologic effects of constitutive local release of TNF-alpha may be quite different from those of its systemic injection.     

HLA class I and II alleles and susceptibility to generalized pustular psoriasis: significant associations with HLA-Cw1 and HLA-DQB1*0303

HLA alleles in generalized pustular psoriasis (GPP) were investigated to clarify the etiology and/or pathogenesis of this disease. Not only serological typing of HLA class I and II antigens but also genotyping of HLA class II alleles were carried out in twenty-six unrelated Japanese patients with GPP. These patients were classified according to their history of psoriasis vulgaris (PV). Serological typing revealed a significantly high incidence of HLA-Cw1 (Pc = 0.04) in the patients as compared with Japanese healthy controls. The frequency of HLA-B46 was particularly high in the patients with GPP and a previous history of PV. Genotyping of HLA class II alleles showed a highly significant increase in HLA-DQB1*0303 (Pc = 0.01) in the patients vs. the healthy controls. In particular, HLA-DQB1*0303 was significantly more frequent in the patients with no prior history of PV than in those with a history of PV. Analysis on linkage disequilibrium showed remarkably different patterns for HLA class II haplotypes between the patients and the healthy controls. Based on the comparative analysis among the amino acid sequences of the beta 1-domain of the HLA-DQB1*03 alleles, proline at residue 55 was suggested to be important as a common amino acid for determination of the susceptibility to GPP. These results revealed not only an association between the etiology and/or pathogenesis of GPP and HLA, but also different mechanisms of the immune response between the patients with GPP and PV.     

Severe tinea barbae due to Trichophyton verrucosum infection in dairy farmers

Trichophyton verrucosum infection, which is acquired from the hide of dairy cattle, is the cause of tinea barbae, a severe pustular eruption on the face and neck. We present the cases of five patients, three of whom had severe pustular tinea barbae and two of whom had eruptions of the forearms. All five patients were dairy farmers. On physical examination, all five patients were afebrile and did not appear toxic. Peripheral leukocyte counts of the three patients with pustular tinea barbae were normal. Gram stain and bacterial cultures of the draining pustules were negative in all three cases. Potassium hydroxide preparation showed hyphae, and cultures yielded T. verrucosum in all three cases. Four patients received therapy with fluconazole, and one received therapy with griseofulvin; in all cases, the lesions healed. T. verrucosum can cause pustular tinea barbae in farmers that may be mistaken for a Staphylococcus aureus infection by clinicians, including infectious disease experts. The answer to a simple question, "Are you a dairy farmer?", may suggest the diagnosis of T. verrucosum in the proper clinical setting.     

Laparoscopic resection of intra-abdominal testicular tumor

Synovitis, Acne, Pustulosis, Hyperostosis and Osteitis (SAPHO) syndrome is an osteoarticular-skin syndrome characterised by sterile inflammatory arthro-osteitis of the anterior chest wall. It is associated with various skin conditions including palmoplantar pustulosis, severe acnes and pustular psoriasis. A link between SAPHO syndrome and seronegative spondyloarthropathies has been suggested over the last 10 years. SAPHO syndrome mainly affects young and middle-aged adults. Treatment of SAPHO syndrome remains empirical as the underlying aetiopathogenesis is unclear.     

Cricothyroid joint injury

We report a rare case of Churg-Strauss syndrome (CSS) in a 41-year-old Japanese man with a history of middle-age onset of bronchial asthma who had severe abdominal pain. He presented with ileus caused by an annular ulcer of the ileum, attributable to mucosal ischemia resulting from necrotizing vasculitis of the mesenteric artery. He also had marked hypereosinophilia (51.5%), elevated serum IgE levels (34040 IU/ml), and generalized enlargement of the superficial cervical lymph nodes, containing eosinophilic granulomas. A stenotic lesion caused by an annular ulcer in the ileum was found and resected by laparotomy. Microscopic examination of the resected specimen revealed luminal narrowing or occlusion of small arteries in the ulcer base, subserosa, and mesenterium resulting from marked fibrotic intimal thickening with fragmentation or lack of the internal elastic lamina. These findings were diagnosed as vasculitis, scar stage. The postoperative course was uneventful, with the patient receiving a maintenance dose of prednisolone (10-15 mg/day) for 7 years subsequently. We must carefully diagnose and treat patients with middle-age onset asthma, because the symptom may be a lung manifestation of CSS, in which various organs including gastrointestinal tract are involved as a result of systemic necrotizing vasculitis.     

Acute cerebellar ataxia after subclinical hepatitis A infection

Infection is a well-recognized triggering factor for both guttate and chronic plaque psoriasis. We investigated prospectively 13 patients with recalcitrant psoriasis exacerbated by recurrent tonsillitis, who underwent tonsillectomy between 1990 and 1993. There were 12 female patients and one male, with a mean age of 17 yr (range 6-28). Six patients had guttate psoriasis resistant to standard treatments and seven patients had chronic plaque psoriasis exacerbated by tonsillitis that was severe enough to warrant at least one admission to hospital. Patients were followed by chart review and postal questionnaire. Psoriasis was cleared completely after tonsillectomy in five out of the six patients (83%) with guttate psoriasis and was improved in one patient. Two out of seven patients with plaque psoriasis (29%) were cleared, two (29%) were improved and three (42%) were unchanged. We conclude that tonsillectomy may be a successful treatment modality in selected patients with recalcitrant guttate or chronic plaque psoriasis.     

Eosinophilic pustular folliculitis (Ofuji's disease) in myelodysplastic syndrome

We describe a case of eosinophilic pustular folliculitis (EPF, Ofuji's disease) in a 12-year-old male who suffered from myelodysplastic syndrome. Bone marrow study revealed an increase in the eosinophil cell line without peripheral blood eosinophilia in our case. We suggest that the immunologic abberations ascribed to myelodysplastic syndrome and the increase in the eosinophil cell line in the bone marrow might play roles in the development of EPF in our case.     

A preliminary assessment of the efficacy of anticancer measures in malignant stomach neoplasms

Three cases of erosive pustular dermatosis of the scalp are reported. In all patients the dermatosis was characterized by pustular, erosive, and crusted lesions; in addition, two patients had areas of scarring alopecia. The results of laboratory tests, bacteriologic and mycologic investigations, and histopathologic examination were nondiagnostic. Although erosive pustular dermatosis of the scalp is characterized by a nonpathognomonic clinical and histopathologic picture, it probably represents a disease entity.     

Ischemic optic neuritis in Churg-Strauss syndrome

There are few reports of neuro-ophthalmologic involvement in Churg-Strauss syndrome (CSs). We described a case of unilateral optic atrophy in a 46-year-old-white man with CSs. The patient had severe bronchial asthma, allergic rhinitis, hypereosinophilia (8%) and peripheral neuropathy. The visual acuity in his right eye was light perception. At the biomicroscopy there were no corneal and conjunctival lesions. Ophthalmoscopy showed a pale right optic disc and fluorangiography revealed a marked hypofluorescence of the disc at early phase of angiogram. We suggested that the optic atrophy was most probably due to vasculitis of the ciliary arteries.