对超声凝胶中丙二醇发生过敏接触性皮炎1例(发生在肝细胞癌动脉导管栓塞治疗皮肤损伤泄露处)

A 63-year-old man with hepatocellular carcinoma consequent to chronic viral hepatitis C presented with severe dermatitis on the lower right side of the back after transcatheter arterial chemoembolization (TACE) via the 10th intercostal artery, because his hepatic artery had already collapsed due to repeated usage for TACE. The regional skin showed redness and hardness with pustules. Histologically, there was epidermal and appendage necrosis, as well as exocytosis of red blood cells. With a diagnosis of skin injury due to leakage of lipiodol ultrafluid, mitomycin, and epirubicin, administered via the 10th intercostal artery, onto the skin tissue, topical application of 0.06%fluocinonide-containing cream was prescribed. After several weeks of conservative local treatment, the leakage skin injury improved significantly, leaving pigmentation, hardness, and a small necrotic mass, as reported elsewhere (Honda T, Matsushima S, Fujii S, et al. A case of skin injury following transcatheter arterial chemotherapy through intercostal artery for hepatocellular carcinoma. Skin Res 2003; 2: 18-22)-. Subsequently, the patient again consulted the Dermatology Department with further dermatitis in an almost identical skin region on the right side of the abdomen (irregularly spreading erythematous and edematous eruptions with itching; Fig. 1). As he had undergone an ultrasonic examination 2 days earlier, allergic contact dermatitis from the ultrasonic gel was suspected. The contact dermatitis was treated with a topical corticosteroid hormone-containing ointment. Patch testing was performed with Ultra Phonic Conductivity Gel (Pharmaceutical Innovations Inc., Newark, NJ, USA), with which the patient had undergone a series of ultrasonic examinations, and Sono Jelly (Toshiba Medical Supply Co., Ltd., Tokyo, Japan) as a reference, as well as white petroleum as a negative control. A positive result was obtained for Ultra Phonic Conductivity Gel, whereas Sono Jelly was negative (Fig. 2a). Pharmaceutical Innovations Inc. kindly supplied the ingredients of the gel: propylene glycol (PG), preservative in PG, color in PG, thickener 1, and thickener 2. The company gave no further details about the preservative, color, and thickeners. Patch testing was performed using these five materials, resulting in a positive reaction for PG, preservative in PG, and color in PG. Thickeners 1 and 2 and lipiodol ultrafluid were negative (Fig. 2b). On the assumption that the causative chemical was PG, commercially obtained PG, free of preservative and color, was then patch tested (original, 10%aqueous, 1%aqueous, and 0.1%aqueous solutions), resulting in original strongly positive, 10%positive, and 1%and 0.1%weakly positive (Fig. 2c). The medical records showed that the patient had received 16 ultrasonic examinations with the same ultrasonic gel before the leakage skin injury. The 17th examination was performed 2 days after leakage dermatitis, and the 18th 3 months after the injury,when contact dermatitis occurred. The 19th examination was performed using Sono Jelly, which contains no PG, and no skin problems were observed.

角蛋白5的尾部区域1649碱基缺失突变的临床异质性:1个单纯型大疱性表皮松解症伴斑状色素沉着的日本家系

Twenty-five-and 22-y-old Japanese women, who are cousins, presented with distal skin fragility, widespread small, pigmented macules, and toenail deformity. Blisters occurred between the epidermis and the dermis with degeneration of the basal cells, suggesting epidermolysis bullosa simplex with mottled pigmentation (EBS-MP). Electron microscopy of the pigmented spots demonstrated vacuolization of basal cells as well as disturbed junctional structures and incontinence of pigmentation. Gene analysis resulted in detection of a heterozygous deletion of a guanine nucleotide in exon 9 at position 1649. P25L mutation was not detected in either case. It is possible that EBS-MP occurs not only based on the P25L mutation of the keratin 5 molecule, but also because of other types of mutations of epidermal keratin genes.

老年人皮肤局部应用17β-雌二醇可通过体内途径刺激TGF-β信号增加细胞外基质蛋白质合成

To investigate the effects of topically applied 17β-estradiol on the expression of extracellular matrix proteins in aged human skin, 17β-estradiol (0.01%) and its vehicle (70%propylene glycol, 30%ethanol) were applied to aged (68-82 y, eight females and five males) human buttock skin under occlusion for 2 wk (three times per week). Topical 17β-estradiol was found to increase the expression of type 1 procollagen mRNA and protein significantly in human aged skin in vivo. In addition, MMP-1 protein levels were reduced by topical 17β-estradiol. The expressions of TGF-β1,TGF-βtype II receptor, and Sma and Mad related (Smad)3 were increased by topical 17β-estradiol in aged human skin, and TGF-β1 neutralizing antibody inhibited 17β-estradiol-induced procollagen synthesis in cultured fibroblasts. We also found that the expressions of tropoelastin and fibrillin-1 mRNA and protein, and elastic fibers in aged skin were also increased by topical 17β-estradiol. Topical 17β-estradiol also increased keratinocyte proliferation and the epidermal thickness in aged human skin. We also observed the same effects of topical 17β-estradiol in young skin. In conclusion, our results suggest that topical 17β-estradiol treatment may improve the cutaneous function of aged human skin by improving the connective tissue and increasing epidermal thickness.

激素对妊娠及产后银屑病的影响

Objectives: To investigate prospectively how psoriasis fluctuates in pregnancy and post partum and to correlate hormone levels in pregnancy (progesterone and estrogens) with psoriatic change. Design: Psoriatic body surface area (BSA) in pregnant patients with psoriasis (study group) and nonpregnant, menstruating patients with psoriasis (control group) were assessed 5 times over a year. Hormone levels (progesterone and estrogens) were measured in the study group and correlated with change in BSA. Setting: University- affiliated obstetric and dermatology clinics. Patients: Forty- seven pregnant patients in the psoriasis group and 27 nonpregnant, menstruating patients in the control group. Results: During pregnancy, 55% of the patients reported improvement, 21% reported no change, and 23% reported worsening. However, post partum, only 9% of patients reported improvement, 26% reported no change, and 65% reported worsening. Psoriatic BSA decreased significantly from 10to20 weeks’ gestation (P < .001) compared with controls, whereas BSA increased significantly by 6 weeks post partum (P=.001) compared with controls. In patients with 10% or greater psoriatic BSA who reported improvement (n=16; mean BSA, 40% ), lesions decreased by 83.8% during pregnancy. There were significant or near significant correlations between improvement in BSA and estradiol (P=.009, r=0.648), estriol (P=.06, r=0.491), and the ratio of estrogen to progesterone (P=.006, r=0.671). Conclusion: High levels of estrogen correlated with improvement in psoriasis,whereas progesterone levels did not correlate with psoriatic change.

外胚层发育异常/皮肤脆性综合征的组织病理学和超微结构研究

Ectodermal dysplasia/skin fragility syndrome (EDSFS)- (MIM604536) is a newly described autosomal recessive disorder characterized by skin fragility and blistering, palmoplantar keratoderma, abnormal hair growth, nail dystrophy, and occasionally defective sweating. It results from mutations in the PKP1 gene encoding plakophilin 1 (PKP1), which is an important component of stratifying epithelial desmosomes and a nuclear component of many cell types. Our study was performed to further characterize the histopathology of EDSFS in different cutaneous sites with a special emphasis on the hypotrichosis and keratoderma. A total of 4 biopsies were obtained from 2 EDSFS female patients, aged 9 days to 4 years. The biopsies were taken from the blistering skin of the leg and trunk, the hyperkeratotic skin of the sole, and the hypotrichotic scalp. The observed histopathologic features included: widened intercellular spaces, suprabasal intraepidermal clefts and blisters with acantholytic keratinocytes, detachments of the upper epidermal layers due to disadhesion, varying degrees of dyskeratosis thatweremuchmore pronounced in the plantar hyperkeratotic skin, and increased number of catagen- telogen hair follicles. The electron- microscopic observations attributed the disadhesion and acantholysis to reduced numbers of small hypoplastic desmosomes, and the dyskeratosis to the detachment of intracellular keratin filaments from the desmosomes with perinuclear condensation, which might also underlie the plantar keratoderma. The hair follicle findings suggest disturbance in the hair cycle, which might be attributed to disturbed nuclear PKP1 function or result from aberrant desmosomal signaling.

19例全身性念珠菌病伴皮肤损害的临床病理回顾

Background: A diagnosis of systemic candidiasis is often delayed or missed owing to the absence of sensitive, specific, and timely diagnostic tools. Skin lesions are not common, but they can help to rapidly establish a diagnosis. We report on a 14- year experience of systemic candidiasis with skin lesions in our institution. We report the prevalence, clinical findings, histologic findings, etiologic Candida species, underlying conditions, treatment modalities, and outcomes of the cases and compare them with the previous reports. Methods: We reviewed the medical records and laboratory data of patients diagnosed with systemic candidiasis from June 1989 to September 2002 at Asan Medical Center, Seoul, Korea. We thoroughly reviewed the data on those patients with characteristic skin lesions. We included the cases in which Candida organisms were either shown or cultured from the skin. We also included the patients who had developed the characteristic rash at the onset of infection if there was no other possible explanation for the rash. Results: Of 53 documented systemic candidiasis cases, 19 (35.8% ) had the characteristic skin lesions. Fifteen patients (78.9% ) had hematologic problems and were neutropenic. The skin lesions were a maculopapular or nodular rash and plaques. In addition to the trunk and proximal extremities, the rash also involved the face and distal extremities. The rashes were mostly purpuric, not consistently associated with underlying thrombocytopenia but also associated with underlying vascular damage as a result of Candida organisms. The underlying vascular damage also caused intraepidermal necrotic and vesicular change. One case of transepidermal elimination of organisms was newly found. The most common causative species was Candida tropicalis in the 19 patients with skin lesions, in contrast with Candida albicans in a total of 53 patients. The mortality rate was 84.2% . Conclusions: The prevalence of systemic candidiasis- associated skin lesions may be higher than previously reported. Dermatologists should be familiar with the clinical appearance of skin lesions and suspect this fatal infection when seeing neutropenic patients with a resistant fever and accompanying rash.

间断性高剂量糖皮质激素联合低剂量甲氨蝶呤治疗重度局限性硬皮病

Objective: To evaluate the efficacy of pulsed high-dose corticosteroids combined with orally administered low-dose methotrexate therapy in patients with severe localized scleroderma (LS). Design: A prospective, nonrandomized, open pilot study. Setting: Dermatology department at a university hospital in Bochum, Germany. Patients: Fifteen patients with histologically confirmed severe LS. Interventions: Oral methotrexate (15 mg/wk) combined with pulsed intravenous methylprednisolone (1000 mg for 3 days monthly) for at least 6 months. Main Outcome Measures: Treatment outcome was evaluated by means of a clinical score, 20-MHz ultrasonography, and histopathologic analysis. Safety assessment included the monitoring of adverse effects and clinical laboratory parameters. Results: One patient discontinued therapy. In most of the remaining 14 patients, significant elimination of all signs of active disease (inflammation) and remarkable softening of formerly affected sclerotic skin that resulted in a decrease of the mean±SD clinical score from 10.9±5.3 at the beginning to 5.5±2.5 at the end of therapy was observed (P< .001). Clinical improvement was confirmed by histologic and ultrasonographic assessments. No serious adverse effects were noted. Conclusions: These data suggest that pulsed high-dose corticosteroids combined with orally administered low-dose methotrexate therapy is beneficial and safe in the treatment of patients with LS. This treatment regimen should especially be considered for severe forms of LS in which conventional treatments have failed.

联合应用抗疟药治疗皮肤型皮肌炎的回顾性研究

Objective: To observe whether the use of antimalarials in combination resulted in significant improvement in the cutaneous signs and symptoms of patients with dermatomyositis who did not otherwise respond to the use of single-agent antimalarial therapy. Design: Retrospective case series of 17 patients treated between January 1, 1991, and December 31, 2002. Setting: An ambulatory medical dermatology clinic in an academic center. Patients: Patients had adult-onset dermatomyositis with predominantly cutaneous symptoms and a follow-up period at our clinic of at least 6 months. Cases in which it was not possible to assess the effect of treatment on cutaneous symptoms were not included. Intervention: Treatment regimens varied and included the use of antimalarials, prednisone, methotrexate, and other medications. Main Outcome Measures: Physician-observed and patient-reported improvement based on erythema, pruritus, and extent of affected skin. Results: Seven of 17 patients experienced at least near clearance in cutaneous symptoms with the use of antimalarial therapy alone: 4 of these patients required combination therapy (hydroxychloroquine sulfate-quinacrine hydrochloride or chloroquine phosphate-quinacrine),while 3 of them responded well to antimalarial monotherapy. The median time required to reach the response milestones on the final working therapeutic regimen was 3 months (mean, 4.8 months; range, 2-14 months). Six patients did not respond significantly to any type of therapy, including nonantimalarials. Conclusion: Our experience suggests that a significant subgroup of patients whose skin lesions have been unresponsive to a single antimalarial benefit from combination therapy with hydroxychloroquine and quinacrine or chloroquine and quinacrine, but controlled clinical trials are warranted to assess the extent of benefit.

组织细胞样Sweet综合征:幼稚中性粒细胞的皮肤浸润

Objective: To describe a series of 41 patients with fresh lesions of Sweet syndrome in which the histopathologic study demonstrated an inflammatory infiltrate mostly composed of histiocytoid mononuclear cells. Design: Histopathologic, immunohistochemical, and cytogenetic studies of the inflammatory infiltrate in a case series of histiocytoid Sweet syndrome. Setting: University departments of dermatology and a private laboratory of dermatopathology. Methods: Conventional histopathologic study as well as immunohistochemical investigations were performed using the alkaline phosphatase antialkaline phosphatase technique with a large panel of antibodies. In some cases, fluorescent in situ hybridization studies were performed to investigate the presence of the bcr/abl gene fusion. Results: Immunohistochemical studies demonstrated that most cells of the infiltrate showed immunoreactivity for CD15, CD43, CD45, CD68, MAC-386, HAM56, and lysozyme, which is consistent with a monocytic-histiocytic immunoprofile. However, intense myeloperoxidase reactivity was detected in most of the cells with histiocytic appearance, which raised the possibility of specific cutaneous involvement by myelogenous leukemia. Nevertheless, cytologic peripheral blood examinations, fluorescent in situ hybridization studies to investigate the bcr/abl gene fusion, and follow-up of the patients, taken all together, ruled out this possibility. Conclusions: This case series demonstrates that some fresh cutaneous lesions of Sweet syndrome are histopathologically characterized by an infiltrate mostly composed of cells that may be misinterpreted as histiocytes, when in fact they are immature myeloid cells. We named this histopathologic variant histiocytoid Sweet syndrome, which should not be mistaken with leukemia cutis or other inflammatory dermatoses that are histopathologically characterized by histiocytes interstitially arranged between collagen bundles of the dermis.

Churg-Strauss综合征的初始皮肤症状与多重单一神经病变一致

Background: Churg-Strauss syndrome (CSS), also known as allergic granulomatous angiitis, is a rare entity that is characterized by systemic vasculitis in patients with a history of asthma. Patients with CSS show a marked peripheral blood eosinophilia, but the pathogenesis remains unknown. Observations: A retrospective review was performed in 9 cases of CSS in whom cutaneous findings were present as an initial manifestation. All 9 patients had purpura and petechiae as well as severe pain and paresthesias of the lower extremities. Four patients (44%) used leukotriene receptor antagonists to treat their asthma, and 3 (75%) of them developed CSS within 3 months. Five patients (56%) were positive for perinuclear antineutrophil cytoplasmic antibodies before therapy, but in all 5 the levels of perinuclear antineutrophil cytoplasmic antibody normalized. Serum IgE levels were elevated in all patients before treatment but decreased after treatment. Histologically, all patients demonstrated leukocytoclastic vasculitis and eosinophilic infiltration. Eight biopsy specimens (73%) revealed marked eosinophilia around the nerve fibers in the dermis. Palisading granulomas in association with vessel-based changes were present in 4 (36%) of 11 biopsy specimens. Conclusions: These characteristic cutaneous clinical patterns that are consistent with the presence of mononeuropathy multiplexes in the lower extremities may help physicians establish an earlier diagnosis. Both eosinophils and IgE, as well as perinuclear antineutrophil cytoplasmic antibodies to some degree, likely participate in skin lesion development in CSS. Furthermore, there appears to be a correlation between treatment with leukotriene receptor antagonists and the onset of CSS in some cases.

寻常型天疱疮血清学诊断中抗桥粒芯蛋白抗体与间接免疫荧光检测的比较

Background: Indirect immunofluorescence (IIF) is the standard method for the detection of pemphigus autoantibodies. Commercially available enzyme- linked immunosorbent assays (ELISAs) have recently become available to measure serum antibodies (Abs) against desmoglein1 (Dsg1) and desmoglein3 (Dsg3). It has been suggested that patients with mucosal- dominant pemphigus vulgaris (PV) have serum Abs against Dsg3 only, patients with mucocutaneous PV have Abs to both Dsg1 and Dsg3, and patients with pemphigus foliaceus (PF) have Abs against Dsg1 only. Aim: To compare the sensitivity and specificity of the IIF and ELISA tests in the diagnosis of pemphigus and its subsets. Methods: Thirty- three patients with PV and five patients with PF were studied, and compared with 50 healthy individuals or patients with unrelated skin diseases. Monkey esophagus was used as a substrate for the IIF test. Results: The IIF and ELISA tests were each positive in 26 of the 32 (81% ) PV patients, and in none (0% ) and 3 (6% ) of the 50 controls, respectively. Both the IIF and ELISA results were concordant in 69% of the PV patients, and only one of these two tests was positive in the remaining 31% of patients. Forty- six per cent of the PV patients with a positive ELISA test did not have the PV phenotype (mucosal or mucocutaneous) predicted by their autoantibody profile. Conclusion: The IIF and ELISA tests may be used as complementary tests for the serologic diagnosis of pemphigus.

致残性泛发性硬斑病:2例成人的临床表现

Disabling pansclerotic morphea involves all layers of the skin, extending through the dermis and subcutaneous tissues to involvemuscle, tendon, and bone. It is distinguished from generalized scleroderma by its lack of systemic involvement. Onset usually occurs before the age of 14 years. We describe adultonset disabling pansclerotic morphea in two previously healthy young men. In both cases, the onset of disease was explosive, with rapid progression,widespread cutaneous involvement, and severe disablement caused by mutilating contracture deformities. Increased susceptibility of sclerodermatous tissue to recalcitrant ulceration and malignant transformation with development of nonmelanoma skin cancers was also observed. Treatment of this disease continues to present a therapeutic dilemma with only sporadic remission despite multimodality therapy.

奥马佐单抗不能改善严重的成人特应性皮炎

Atopic dermatitis has been associated with elevated levels of IgE.Omalizumab is a monoclonal anti-IgE antibody currently approved for the treatment of asthma. We report the failure of omalizumab to improve atopic dermatitis in 3 patients when administered for 4 months.

暴发性痤疮

Acne fulminans is a syndrome of fulminant, necrotizing acne associated with bone lesions, constitutional symptoms, and laboratory abnormalities. A case report of an adolescent male with acne fulminans following withdrawal of isotretinoin and prednisone is presented.

肾移植后发生皮肤病对生活质量的影响

Background: The immunosuppressive therapy a patient requires to sustain a functioning renal allograft in the long term is associated with various skin complications. While quality of life (QoL) after renal transplantation has been studied, no publications document the effect of post- transplant dermatological complications on QoL. Objectives: The objective of the study was to document the prevalence of the skin diseases that commonly occur in association with post- transplant immunosuppression. A general dermatological quality of life questionnaire, the Dermatology Life Quality Index (DLQI), was used to assess the QoL effect of these cutaneous complications. The study was designed to examine further the impact of age, sex, duration since transplant and immunosuppressive regimen on the DLQI score of renal transplant recipients (RTR). Methods: One hundred and seventy- three RTR completed the DLQI,were interviewed and examined for evidence of common post- transplant skin diseases. Results: Sixteen per cent of RTR had DLQI scores>6, reflecting a significant impact on their QoL. Dry skin, itch, hypertrichosis, sebaceous gland hyperplasia, acne, genital warts and a history of >4 herpes simplex virus type 1 infections in the past year were all found to have a significant impact on the quality of life (P < 0.05). Multivariate analysis revealed that the greatest impact on QoL was in RTR who were younger, female and with multiple skin problems (P < 0.05). Conclusions: The dermatological complications of immunosuppressive therapy are common in RTR and can significantly impair QoL in certain individuals. Visible, infectious and cosmetic skin problems had most impact on QoL while a history of skin cancer had a lesser impact. Early dermatological referral and careful choice of immunosuppression may enhance the QoL, particularly in young and female RTR.

皮肤三色斑点征:一种特殊的神经皮肤综合征

An 11- year- old girl had multiple, disseminated, rather small café - au- laitmacules and hypochromic spots involving the neck, the trunk and the legs. In part, the two types of macules showed a spatial proximity, suggesting didymosis (twin spotting). The term cutis tricolor has been proposed to describe congenital paired hyperchromic and hypochromic macules on a background of intermediate skin. Because the spots present in this case were much smaller than those described in previous cases of cutis tricolor, we here propose the distinguishing term “ cutis tricolor parvimaculata” . The underlying gene locus may be a hot spot for postzygotic recombinations, giving rise to multiple twin spots. Moreover, the girl had developed seizures from the age of 10 years, and a large oligodendroglioma involving the left frontal lobe was found. A causal relationship between the cutaneous phenotype and the cerebral tumor is unproven but likely. The skin lesions were reminiscent of a disorder described by Westerhof et al. in 1978 under the term “ hereditary congenital hypopigmented and hyperpigmented macules” . So far, however, it is not clear whether cutis tricolor parvimaculata is identical with or different from this disorder.

套细胞淋巴瘤相关的虫噬样反应:临床病理学、免疫病理学及分子研究

A cutaneous eruption simulating insect bites has been repeatedly described in association with chronic lymphocytic leukemia (CLL). It was only rarely described with mantle cell lymphoma (MCL). Our study was performed to elucidate the clinical, histologic, immunopathological, and molecular characteristics of insect bite like reaction (IBLR) associated with MCL. The clinical presentation and histologic findings in 3 IBLR cases associated with MCL were found to be similar to 3 IBLR cases associated with CLL. The eruptions consisted of itchy erythematous papules, nodules, plaques, and vesicles. Non- vesicular lesions were characterized histologically by normal or mildly spongiotic epidermis. Vesicular lesions were characterized by marked spongiosis and intraepidermal spongiotic vesicles containing eosinophils, or marked subepidermal edema occasionally leading to a dermoepidermal separation. Most of the lesions were characterized by superficial and mid dermal to deep perivascular and interstitial, and occasionally periadnexal, inflammatory- cell infiltrate consisting of mononuclear cells and eosinophils. The densities of the infiltrates varied and the inflammatory- cell infiltrate extended often into the fat lobules. Neutrophils and nuclear dust were found more frequently and abundantly in the IBLR lesions associated with MCL. Immunophenotyping, direct immunofluorescence (DIF)- tests, and IgH gene rearrangement studies were performed in the lesions associated with MCL only. The majority of the infiltrating lymphocytes were CD3+ , CD5+ and CD43+ , more CD4+ than CD8+ , and only a small minority was CD20+ . The cells did not stain for bcl- 1 protein and CD30, and with no evidence of clonality. The DIF test result was negative. The IBLR eruption associated with MCL resembles clinically and histologically IBLR associated with CLL. The eruption seems to be reactive rather than neoplastic, because there is no evidence of MCL involvement in the skin lesions.

局部外用甲基氨基酮戊酸(Metvix~)-PDT治疗颜面和头皮光化性角化症:两种给药法的随机多中心比较研究

Photodynamic therapy (PDT) with topical methyl aminolevulinate (MAL) administered in two treatment sessions separated by 1 week is an effective treatment for actinic keratoses. This open prospective study compared the efficacy and safety of MAL- PDT given as a single treatment with two treatments of MAL- PDT 1 week apart. Two hundred and eleven patients with 413 thin to moderately thick actinic keratoses were randomized to either a single treatment with PDT using topical MAL (regimen I; n=105) or two treatments 1 week apart (regimen II; n=106). Each treatment involved surface debridement, application of Metvix(r) cream (160 mg/g) for 3 h, followed by illumination with red light using a light- emitting diode system (peak wave length 634± 3 nm, light dose 37 J/cm2). Thirtyseven lesions (19% ) with a non- complete response 3 months after a single treatment were re- treated. All patients were followed up 3 months after the last treatment. A total of 400 lesions, 198 initially treated once and 202 treated twice, were evaluable. Complete response rate for thin lesions after a single treatment was 93% (95% CI=87- 97% ), which was similar to 89% (82- 96% ) after repeated treatment. Response rates were lower after single treatment of thicker lesions (70% (60- 78% )- vs 84% (77- 91% )), but improved after repeated treatment (88% (82- 94% )). The conclusion of this study is that single treatment with topical MAL- PDT is effective for thin actinic keratosis lesions; however, repeated treatment is recommended for thicker or non- responding lesions.

对1991～2000年伊朗哈马丹Sina医院收住院治疗的12例麻风患者的临床病理研究

Background. Leprosy is considered a chronic disabling condition. Many clinical and immunological aspects of the disease remain ill defined. Aim. The study of clinico-pathological and laboratory findings of patients with leprosy admitted to Sina Hospital, Hamadan, Iran, from 1991 to 2000. Methods and pa-tients. This is a descriptive retrospective cross-sectional study. The statistical communit y comprised all patients diagnosed leprosy. This diagnosis was clinical and conf irmed through pathology(skin-biopsy)andlaboratory(peripheralsmear)measures. Res ults. In this study, the disease was more common in males than females with a mean age of 48.5 ±16.2 years. Most of the patients were more than 40 years old. Among 12 patients in this study, six cases were urb an and six cases were rural. Six cases were living in Hamadan province and two c ases migrated to Hamadan province (one of them from Afghanistan and the other fr om Kurdestan). Clinical diagnosis was confirmed by pathology in 11 cases, but in one case the clinical diagnosis did not match the pathology. In four cases the clinical diagnosis did not match the peripheral smear. Eight cases were admitted just once. Four cases had a history of recurrence and readmission (two patients had one time recurrence and the other two patients had two recurrences). There was no difference in the clinical findings between first presentation and recurr ence. From the point of complication and disability, extremity disability was mo re common than eye disability. Increased severity of complications was found in patients with a delayed diagnosis and incomplete treatment. Conclusion. This stu dy showed that a rapid and correct diagnosis and complete treatment was necessar y for prevention of complication and disability in patients with leprosy. Also t he accuracy of pathology (skin biopsy) in the diagnosis exceeded the peripheral smear. Skin biopsy is recommended to confirm the diagnosis in all cases of lepro sy. In the absence of pathology, patients must be considered as multibacillary p atients and treated as such.

细胞毒性皮肤归巢CD8^+淋巴细胞在皮肤细胞毒性T细胞淋巴瘤和苔藓样糠疹中的作用

Background: Pityriasis lichenoides (PL) is a rare cutaneous lymphoproliferative disorder of unknown origin. Malignant transitions of PL have been described, but are very rare. We recently observed the fatal course of a 26- year- old patient who presented with a clinical picture resembling PL but had cytotoxic CD8+ T- cell lymphoma of the skin (CxCTL). This case prompted us to reinvestigate the role of cytotoxic T lymphocytes in PL and its relationship to antiviral immunity. Methods: Skin biopsy specimens of 11 patients with PL and two biopsy specimens of CxCTL were included. In all, 5 biopsy specimens taken from healthy skin and 5 samples of varicella- zoster virus (VZV) skin lesions were analyzed for control purposes. The inflammatory infiltrate was characterized by immunohistochemistry using monoclonal antibodies against CD3, CD4, CD8, CD20, cutaneous lymphocyte- associated antigen (CLA), CCR4, CXCR3, GranzymeB, Tia- 1, andMxA. Flowcytometry was used to analyze the expression of chemokine receptors on peripheral blood mononuclear cells in CxCTL. Results: The CxCTL skin lesions were dominated by a dense infiltration of CD8+ cytotoxic lymphocytes with a skin- homing CLA+ CCR4+ phenotype. PL and VZV skin lesions were also characterized by a predominantly CD8+ T cellular infiltrate with strong expression of the cytotoxic molecules Granzyme B and Tia- 1 and the skin- homing molecules CLA and CCR4. Coexpression analyses confirmed that skin CLA+ CD8 + cytotoxic T cells are present in CxCTL, VZV, and PL skin lesions. Strong lesional production of the antiviral protein MxA, which is specifically induced by type I interferons, could be found in all investigated disorders. The study was based on histologic, immunohistologic, and flow cytometric analyses in a limited number of patients, because of the rareness of the investigated diseases. Conclusion: Our results revealed a striking similarity between the immunohistologic picture of malignant CxCTL, benign PL, and VZV skin lesions. Strong expression of the antiviral protein MxA in all disorders supports the view that a common antiviral immune response pattern leads to aberrant skin recruitment of CLA+ CCR4+ cytotoxic T lymphocytes in PL and CxCTL.