Peptidylarginine deiminase (PAD) (EC 126.96.36.199) catalyzes deimination of arginine residues in proteins in a calcium-ion-dependent manner , generating citrulline residues . The deiminated proteins show lowered isoelectric points, affecting protein interactions dependent on ionic charge. Stability of hydrogen bonds may also be disrupted. Substrate proteins of PAD in the epidermis include keratin K 1 and filaggrin, but not loricrin. Because keratin K 1 is assumed to be associated with filaggrin through ionic zipper interaction and with loricrin through velcro interaction, respectively, alteration of PAD-dependent protein deimination would have a significant biological effect on terminal differentiation of keratinocytes. Psoriatic hyperproliferative epidermis is characterized by decreased deiminated proteins, especially keratin K 1. Whether the finding is associated with a defect of PAD in the psoriatic hyperproliferative epidermis remains to be determined.
We investigated 94 primary and 17 metastatic lesions of malignant skin tumors, 51 lesions of lymph nodes, and 109 lesions of benign skin tumors with power Doppler sonography. Blood flow signals were detected in 89% of malignant tumors excluding carcinoma in situ; on the other hand, it was delected in only 4% of benign tumors. We concluded that power Doppler sonography is a very useful noninvasive tool for differential diagnosis of malignant skin tumors, especially in distinguishing malignant melanoma from benign lesions.
We investigated the relationship between dental focal infections and pustulosis palmaris et plantaris (PPP) by studying the effects of treatment of dental focal infections in 60 patients with PPP who had visitedour department clinic from November 1995 to June 1999. Dental focal infections, which include chronic peria-pical disease and marginal periodontitis, were detected in 54 PPP patients. Improvement of the skin lesions was observed in 20 (64.5%)out of 31 patients who had received dental therapy. Only 1 (14.3%) out of 7 patients who had received no treatment had natural improvement of the PPP lesions. The present results suggest that dental focal infection may be one of important causative or exacerbation factors for PPP and that dental examinations as well as tonsillar examinations should be done prior to treatment for PPP.
Of the 158 Japanese patients with systemic sclerosis (SSc) who were followed up during the period from 1991 to 1999 in our department, 29.1% had anti-DNA topoisomerase 1 antibody (anti-Topo 1 antibody), 23.4% had anticentromere antibody (ACA), and 8.9% had anti-U 1 RNP antibody. Significant associations between the antinuclear antibody (ANA) and organ involvement were noted: anti-Topo 1 antibody with digital pitting scar, trunkal sclerosis, and lung fibrosis; ACA with esophageal hypomotility, Sjögren’s syndrome, and primary biliary cirrhosis. Cumulative survival rates at 10 years after diagnosis of SSc was lowest in anti-Topo 1 positive diffuse cutaneous SSc (dcSSc) (86.4%). The most frequent cause of death related with SSc was cardiopulmonary disease due to lung fibrosis (9/15, 60%; 2 were anti-Topo 1 antibody positive limited cutaneous SSc (lcSSc), 3 were anti-Topo 1 antibody positive diffuse cutaneus SSc (dcSSc), 2 were U 1 RNP positive lcSSc, 2 were specific antibodies-negative lcSSc). In anti-Topo 1 antibody positive lcSSc and dcSSc, both the lung involvement rates and severe lung disease represented by low vital capacity (<60%) were high. In specific antibodies-negative lcSSc, the severe lung disease rate was high, although the lung involvement rate was low. Careful observation of the progression of pulmonary interstitial fibrosis is needed not only in cases of anti-Topo 1 antibody positive SSc but also in specific antibodies-negative lcSSc with lung fibrosis.
Eighty-seven out of 100 adult patients with intractable atopic dermatitis (AD) were strongly suggested of having addictive scratching behavior from interviews, clinical findings, or diaries of scratching, which is habitual, and often induced by emotional stress. Psychosocial stress, especially that stemming from familial or occupational problems, was shown to have exacerbated the dermatitis in 70 (80.5%) of those patients with addictive scratching behavior. The most frequent psychiatric diagnosis was psychological factors affecting the medical condition［DSM-IV］in 63 out of 87 patients (72.4%). The relationship between the clinical course of AD and the degree and recognition of addictive scratching was studied among 63 patients who were followed up for at least 6 months. Forty-seven patients (74.6%) recognized their addictive scratching, but 16 (25.4%) did not. Among those who did, scratching was reduced in 46.8% and the AD inproved in 46.8%, slightly improved in 44.7%, and unchanged in 8.5%. Contrarily, among patients who failed to recognize it, scratching reduced only in 31.3% and AD improved in 31.3%, slightly improved in 31.3%, and was unchanged in 37.5%. Thus patients who recognized their addictive scratching behavior showed better long-term clinical improvement. Psychosocial stress should always be carefully considered, and encouraging patients to recognize and reduce their addictive scratching is beneficial.
A 57-year-old woman presented with a three-month history of an erythematous indurative lesion on her right flank. Physical examination revealed dry eye and no palpable lymph nodes. Biopsy specimens from the lesion showed a dense and diffuse cell infiltration and lymphoid follicures. From the margin of the follicles, monocytoid cells with round nuclei infiltrated into interstitial space and eccrine ducts and formed lympho-epithelial lesions. A subcutaneous nodule appeared later on the right chest and showed that the follicular cell infiltration, or follicular colonization, consisted of centrocyte-like (CCL) cells. Immunohistochemically, these cells were positive for CD 79 a, L-26, and bcl-2 and negative for UCHL-1, CD 3, CD 5, CD 10, and CD 23, indicative of a B cell profile. DNA hybridization analysis revealed rearrangement of the immunoglobulin light chain Ckappa. Serum antinuclear antibody and SS-A antibody were positive; Schirmer’s test was also positive. A biopsy of the small salivary gland of the lip showed a periductal mononuclear cell infiltration. We diagnosed her with Sjögren Syndrome with cutaneous marginal zone B-cell lymphoma of the MALT type. The patient was treated with combination chemotherapy (cyclophosphamide, vincristin, adreamycin and prednisone) and achieved complete remission with a duration of nine months.
A woman was first admitted to our hospital in 1987 at the age of 56 vears because of cancer of the uterine cervix. Total hysterectomy was performed, and 50 Gy of 60CO was irradiated to the whole pelvis postoperatively. After these therapies, the patient enjoyed good health for twelve years. In 1999, she was admitted again because of a large pelvic tumor which adhered to pelvic bone. Pelvic CT showed a low density mass which invaded soft tissue and surrounded the right os pubis; however there was no obvious bone destruction. Chest CT showed multiple coin lesions in both lungs. A biopsy specimen of the lesion showed characteristic bimorphic pattern composed of highly cellular proliferation of undifferentiated small cells and zones of differentiated cartilaginous tissue.