Palisaded neutrophilic granulomatous dermatitis presenting as an unusual skin manifestation in a patient with Behçet's disease.

A 32-year-old Korean woman with painful oral ulcers and a sore throat presented with multiple erythematosus papules on both legs. Histological examination of the papular lesions on the legs demonstrated palisaded granuloma with degeneration of collagen fibres in the dermis, compatible with palisaded neutrophilic granulomatous dermatitis (PNGD). This condition is known to be an unusual disease entity associated with various systemic autoimmune diseases, such as rheumatoid arthritis, systemic lupus erythematosus, other connective tissue diseases, and systemic vasculitis. To our knowledge a case with typical Behçet's disease coinciding with PNGD among systemic autoimmune diseases has not been described before.

A case report: isolated a heavy chain monoclonal gammopathy in a patient with polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy and skin change syndrome.

A 45-year-old South-Korean man presented with abdominal distension, progressive paresthesia and motor weakness of both lower extremities. Our case was identified as polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy and skin change (POEMS) syndrome based on diagnostic criteria. Circulating M components of POEMS syndrome consist mainly of IgG or IgA-lambda and rarely IgM-lambda, IgG-kappa or isolated light chains. In this case, the M-band on serum protein electrophoresis and isolated IgA heavy chain on serum immunofixation electrophoresis were demonstrated, but there was no abnormal light chain. We suggest that this case may be associated with a pattern of abnormal secretion of monoclonal protein or a coincidence of a heavy chain disease in POEMS syndrome, even though the latter possibility may be very rare.

Effects of peroxisome proliferator-activated receptor-gamma (PPAR-gamma) on the expression of inflammatory cytokines and apoptosis induction in rheumatoid synovial fibroblasts and monocytes.

This study was performed to investigate whether peroxisome proliterator-activated receptor-gamma (PPAR-gamma) exerted an anti-inflammatory effect on rheumatoid synovial cells and inhibited dysregulated proliferation. The expression of PPAR-gamma mRNA in cultured human synoviocytes and THP-1 cells was analysed by RT-PCR. PPAR-gamma was expressed in normal, osteoarthritis (OA), rheumatoid arthritis (RA) synovial cells as well as a human monocytic cell line, THP-1. In RA and OA synoviocytes, the induction of inflammatory cytokine mRNA expression such as TNF-alpha and IL-1beta was significantly inhibited by the natural PPAR-gamma agonist, 15 deoxy-Delta(12,14)prostaglandin J(2)(15d-PGJ(2)). The effect of PPAR-gamma on the nuclear factor (NF)-kappaB activity was tested by electrophoretic mobility shift assay (EMSA). Both troglitazone and 15d-PGJ(2)markedly inhibited TNF-alpha-induced NF-kappaB activation at 30 microM. However, PPAR-gamma agonist neither reduced proliferation nor induced apoptosis in RA synoviocytes when measured by XTT assay and fluorescence activated cell sorter (FACS) analysis. In contrast, it induced apoptosis in a dose-dependent manner in THP-1 cells and augmented TNF-related apoptosis-inducing ligand (TRAIL)-induced apoptosis as well. In conclusion, these data demonstrate that PPAR-gamma is expressed in human synoviocytes and THP-1 cells, and the PPAR-gamma activation inhibits expression of inflammatory cytokines in RA synoviocytes. Furthermore, PPAR-gamma activation induces apoptosis by itself and augments TRAIL/Apo2L-induced apoptosis in THP-1 cells. These results suggest that PPAR-gamma agonists may provide a new therapeutic approach for RA.

Evaluation of survival rate after follicular unit transplantation using the KNU implanter.

BACKGROUND: Many hair transplant surgeons have advocated the use of micrograft megasessions for the purpose of a more natural looking end result, especially in the Oriental with higher skin/hair color contrast and darker, coarse, straight hairs. But it also has some fundamental limitations. Most important are a low graft yield and a low density after transplantation. There are several reports about the survival rate of micrografts in Caucasians, though the results are variable, but few reports about the survival rate and fate of micrografts in Orientals. OBJECTIVE: To compare the survival rate of one-hair follicular units with that of two-hair follicular units using the KNU implanter, to ascertain the average survival rate of micrografts (one- and two-hair follicular units), and to evaluate the fate of grafted hair according to time. METHODS: Two templates of 1.5 cm2 were made by tattooing on both sides of the frontoparietal recess areas in 11 patients with male pattern baldness (beyond Norwood type IIIa). The authors planted one-hair unit micrografts in the left template and two-hair unit micrografts in the right template, which were prepared by the concept of follicular unit, and counted the surviving number of follicular units at 1 and 3 months and total hairs at 6 and 12 months after transplantation. RESULTS: The mean survival rate by the number of follicular units was 47.3 and 57.4% at 1 and 3 months after transplantation, respectively. The mean survival rate by the total number of hairs was 92.0 and 90.4% at 6 and 12 months after transplantation, respectively. CONCLUSION: About 50% of the transplanted hairs fell out in 1 month, but at 6 months the survival rate of follicular unit transplantation using the KNU implanter showed a good result (92%), and there were no significant differences in the mean survival rate of follicular unit and total hairs between one-hair and two-hair units at 1, 3, 6, and 12 months after transplantation.

Transverse myelitis in a patient with long-standing ankylosing spondylitis.

Ankylosing spondylitis is reported to involve not only the joints but other organs as well. Among these extra-articular involvements, uncommon complications associated with nervous system such as single root lesions, compression of the myelum and cauda equina syndrome have also been documented. Here we present a patient with long-standing ankylosing spondylitis who developed spastic paraparesis. Extensive study to find the cause of a spastic paraparesis failed and therefore led to the conclusion that this patient was suffering from transverse myelitis. Similar reports in the past have been attributed to an association with multiple sclerosis; however, we suggest that the findings support the diagnosis of a rare complication of ankylosing spondylitis with an unknown etiology.

Successful treatment of protein-losing enteropathy due to AA amyloidosis with somatostatin analogue and high dose steroid in ankylosing spondylitis.

Secondary amyloidosis is an occasional complication of ankylosing spondylitis (AS) and in most cases renal amyloidosis presents with proteinuria, nephrotic syndrome and decreased renal function. We describe a 32-year-old male patient with AS manifested by frequent diarrhea, intermittent abdominal pain and low serum albumin levels. He has suffered from severe inflammatory back pain for 14 years with multiple peripheral joint involvement. Protein-losing enteropathy due to gastrointestinal amyloidosis was diagnosed with 99mTc-human albumin scintigraphy, fecal alpha-1 antitrypsin clearance and colonoscopic biopsy with Congo red staining. Somatostatin analogue octreotide and prednisolone were introduced with successful result.

Seronegative spondyloarthropathy initiated by physical trauma.

We undertook this study to demonstrate the pattern of onset and the course of arthritis on the traumatised joint in spondyloarthropathy (SpA) initiated by physical trauma. Among 288 patients with SpA, 12 (4.2%) whose arthropathies were associated with trauma were reviewed retrospectively. There were seven patients with ankylosing spondylitis (AS), three with juvenile onset AS and two undifferentiated SpA. The type of trauma was direct injury to the joint and injuries at other sites, except in spinal surgery, for example. In eight cases the initial evidence of disease was peripheral arthritis. The disease first occurred in traumatised joints in five cases. Only three cases showed recurrent inflammatory episodes in the traumatised joints throughout the disease course. SpA initiated by trauma initially manifested as peripheral arthritis at the traumatised joints in about half of the cases. Inflammatory episodes preferentially involved other joints apart from the traumatised joints throughout the whole course of the disease.

Mixed connective tissue disease associated with skin defects of livedoid vasculitis.

A 21-year-old woman who had a 2-year history of mixed connective tissue disease (MCTD) developed rapidly evolving ulcers consistent with livedoid vasculitis (LV) in all distal extremities. She presented clinically with Raynaud's phenomenon, polyarthritis and swollen hands; serologically with high titres of ANA and anti-nRNP; and immunogenetically with HLA-DR4 and HLA-DR53. Although there was initial success in treatment except for the skin defects over the ankles, the patient died from disseminated intravascular coagulation. We suggest that LV may be a poor prognostic manifestation in MCTD.

Secondary renal amyloidosis in adult onset Still's disease: case report and review of the literature.

We report a 25-year-old Korean woman with Adult onset Still's disease (AOSD) presented with renal amyloidosis, which had developed four years after disease onset. We successfully treated her with prednisolone, colchicine and cyclophosphamide. A review of the literature uncovered about 10 cases, most of which were treated by various regimens that resulted in poor outcomes. Renal amyloidosis should be suspected in patients with AOSD who have unexplained proteinuria. Although the mechanism of renal amyloid deposition is not well known, earlier histopathologic diagnosis and choice of regimen may affect prognosis.

High toxicity of sulfasalazine in adult-onset Still's disease.

OBJECTIVE: Sulfasalazine (SSZ) is an anti-rheumatic drug that has been used to treat chronic arthritis. In many reports, the use of SSZ in children with systemic onset juvenile rheumatoid arthritis (JRA) revealed frequent side effects which required discontinuation of the drug. We examined whether there were frequent side effects of SSZ in patients with adult-onset Still's Disease (AOSD). METHODS: From July 1990 to April 1998, we followed 41 AOSD patients. Ten were given SSZ for the treatment of arthritis and the side effects were studied. We also studied 109 consecutive patients with RA who had been given SSZ, as a control group. In addition, we retrospectively studied the side effects and efficacy of SSZ in both groups through their medical records. RESULTS: Six patients (60%, p < 0.01) with AOSD experienced side effects ranging from mild ones like abdominal pain, nausea and vomiting, urticaria, and facial flushing to severe ones such as high fever, hypotension, and severe myelosuppression as well as fulminant hepatitis, which led to the death of one patient. However, 16 patients (14.7%) with RA stopped using SSZ due to mild side effects such as rash, urticaria, gastrointestinal troubles, mild leukopenia, and fever. Three AOSD patients (30%, p = 0.053) and 15 RA patients (13.8%) stopped using SSZ due to its inefficacy. CONCLUSION: We conclude that SSZ appears to have frequent severe side effects in AOSD, as in systemic onset JRA. These potential adverse effects of SSZ should be considered when it is used to treat chronic arthritides with systemic symptoms. Further study of SSZ in the treatment of AOSD in a multi-center, placebo-controlled environment is needed.

Posterior tibial neuropathy by a Baker's cyst: case report.

Baker's cysts are rare cause of peripheral nerve entrapment and only a few cases of tibial nerve entrapment resulting from the popliteal cyst in the calf muscle have been reported in the literature. We present a case of rheumatoid arthritis complicated by a Baker's cyst with a tibial nerve entrapment. It is important to diagnose a Baker's cyst early and to differentiate it from thrombophlebitis, a popliteal aneurysm, tumor or muscle tear to effect optimal therapy and to obviate a potential neuropathy. Prompt recognition of these cases may save the patients unnecessary procedures and delay in treatment.

The changes in hair growth pattern after autologous hair transplantation.

BACKGROUND: Recently donor dominance has been emphasized in autologous hair transplantation while the influence of the recipient site has been considered negligible. In fact, there have been few studies that show this. OBJECTIVE: This study was performed to examine the influence of the recipient site on transplanted hairs. A clinical study of 19 leprosy patients was performed. These patients had received single hair transplantation due to madarosis and were admitted to The Leprosy Mission, Jesus Hospital, Taegu, Korea, or had visited its outpatient clinic. METHODS: In this study, the rate of growth, thickness of shaft, and graying rate between the transplanted eyebrow hair in the recipient site and scalp hair near the donor site were compared to observe the changes in the growth pattern of the hairs after transplantation. RESULTS: For most of the patients, the growth rate and graying rate of transplanted hairs were lower than those of hairs in the donor site. CONCLUSION: It seems that the recipient site may have an influence on the transplanted hairs. Further studies are needed, including clinical, histopathologic, and molecular biological methods.

Multiple lymphadenopathy induced by wear debris after total knee replacement.

Lymphadenopathy induced by wear debris after total joint arthroplasty is very rare. In this article we report on a patient with rheumatoid arthritis who developed multiple lymphadenopathy after total knee joint replacement.