Sachiko Akimoto

Antineutrophil cytoplasmic autoantibodies in patients with systemic sclerosis

Perinuclear type of antineutrophil cytoplasmic antibodies (p‐ANCA) have been found in patients with periarteritis nodosa. Churg‐Strauss arteritis. or pauci‐immune idiopathic crescentic glomerulonephritis. Recently, the association of p‐ANCA and normotensive renal failure, in patients with systemic sclerosis (SSc), was reported. We have studied the incidence of p‐ANCA in patients with SSc and have investigated its relationship to clinical and laboratory findings. Sera from 77 patients with SSc were examined by the indirect immunofluorescence (IIF) test, employing an ethanol‐fixed human neutrophil and enzyme‐linked immunosorbent assay, using purified myeloperoxidase (MPO) as an antigen (MPO‐ELISA). The sera from seven patients (9‐1%) were p‐ANCA positive, by both IIF and MPO‐ELISA. One patient died from systemic necrotizing angiitis but the remaining six patients have shown no symptoms of systemic vasculitis or of renal involvement. There was a tendency for patients positive for p‐ANCA (anti‐MPO antibody) to also be positive for other autoantibodies. such as anti‐Sc1‐70 antibody, anti‐centromere antibody, anti‐microsome antibody, anti‐thyroglobulin antibody and rheumatoid factor. Although the incidence of p‐ANCA (anti‐MPO antibody) is low in patients with SSc, and its clinical significance in SSc needs further investigations, this could be a serological marker for certain symptoms in SSc.

Immunological features of patients with primary biliary cirrhosis (PBC) overlapping systemic sclerosis: A comparison with patients with PBC alone

To characterize the immunological features of patients with primary biliary cirrhosis (PBC) overlapping systemic sclerosis (SSc), 26 patients with PBC were classified according to the presence of scleroderma‐related features (Raynauds phenomenon, sclerodactyly etc.). The patients were classified into 10 patients with PBC overlapping SSc (PBC‐SSc), four patients with some scleroderma‐related features although not meeting the criteria of SSc (PBC‐SSc spectrum) and 12 patients with PBC alone. Sera from PBC‐SSc showed a significantly higher positivity to anti‐centromere antibody (P < 0.01) and to E1β of pyruvate dehydrogenase complex (P < 0.005) than those from patients with PBC alone. The same tendency was observed in PBC‐SSc spectrum patients. Patients with PBC exhibiting scleroderma‐related features, in both the PBC‐SSc and PBC‐SSc spectrum, may comprise a subset in PBC, not only clinically, but also immunologically.

Functions of the stratum corneum in systemic sclerosis as distinct from hypertrophic scar and keloid functions

Both transepidermal water loss (TEWL) and skin surface high-frequency conductance are functions of the skin barrier. Systemic sclerosis (SSc) and hypertrophic scar (HS)/keloid are characterized by abnormal fibrotic changes in the dermis. Since the close interrelationship between the epidermis and the dermis has been well established, we analyzed the stratum corneum functions of forearm skin in 39 SSc patients after assessing the degree of the skin thickening and compared those functions with 10 age-matched normal controls. We also analyzed the stratum corneum functions of HS or keloid lesions in seven patients using the same methods, and compared those functions to adjacent or contralateral normal skin in identical patients. Neither the TEWL, nor high-frequency conductance of forearm skin in SSc patients were significantly different from those in normal controls. There was no correlation between the levels of TEWL or high-frequency conductance and the degree of skin thickening in SSc. In HS or keloid conditions, high-frequency conductance was significantly elevated (42.5+/-8.9 vs. 26.4+/-5.7, P<0.001). Although TEWL was elevated, there was no statistical significance (48.6+/-39.7 vs. 25.1+/-10.1). Our results revealed that stratum corneum functions are distinct between SSc and HS or keloid. This may reflect the various natures of dermal changes, which in turn differentiate the functions of the stratum corneum in the diseases.

Multiple actinic keratoses and squamous cell carcinomas on the sun‐exposed areas of widespread vitiligo

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Plantar thrombotic nodules with marked neovascularization in diabetes: a symptom which has been overlooked?

A 69‐year‐old woman with diabetes has had multiple subcutaneous nodules on the soles for 11 months. She suffered from uncontrolled diabetes mellitus, hypercholesteremia, and cardiovascular thrombosis and had a past history of photocoagulation therapy for proliferative diabetic retinopathy. Histological examination revealed vascular thrombosis surrounded by neovascularization in the subcutaneous tissue. The basement membranes of vessels were thickened and strongly stained with periodic acid‐Schiff. An increased serum concentration of von Willebrand factor antigen was observed. With control of her diabetes, the nodules almost disappeared. Her curious skin manifestation is speculated to be a diabetes‐mellitus‐related change in which endothelial injury and the subsequent induction of angiogenetic factors may play important roles. It is possible that this kind of skin manifestation has been overlooked or not examined histopathologically.