Two sporadic cases of idiopathic multiple minute digitate hyperkeratosis

Multiple minute digitate hyperkeratosis (MMDH) is a skin disease of unknown aetiology characterized clinically by multiple minute asymptomatic keratotic lesions with spiky horny projections. The disorder has been classified into early (congenital) and late (acquired) onset forms, the latter occurring as a presenting sign of concomitant inflammatory, metabolic or malignant disease. Here we report two cases of late onset MMDH without any associated pathology. These cases emphasize that some cases of late-onset MMDH may be idiopathic.     

浙江省339例迟发型白癜风临床分析

目的 探讨迟发型白癜风的临床特征和流行病学情况.方法 采用问卷调查形式对339例迟发型白癜风患者(发病年龄>30岁)进行临床研究,并以早发型患者(发病年龄<30岁)作对照,用SPSS 16.0软件包对数据进行分析.结果 339例迟发型白癜风患者中男110例占32.4%,女229例占67.6%,女性显著多于男性.而早发型患者男女比例差异无统计学意义.平均发病年龄(45.4±10.8)岁,228例(67.3%)患者在31～50岁之间发病.头颈部是最常见的发病部位,迟发型白癜风患者首发于上肢的比例显著高于早发型白癜风患者.迟发型白癜风患者节段型、局限型比例显著低于早发型患者的比例,散发型、肢端型患者比例显著高于早发型患者.252例(74.3%)迟发型患者处于进展期,105例(31.0%)患者伴有毛发变白.迟发型患者伴发自身免疫性或内分泌疾病的比例(8.8%)与早发型患者(5.9%)比较,差异无统计学意义,但伴发甲状腺疾病及糖尿病的比例显著高于早发型患者(P<0.05).迟发型患者家族史阳性比例为9.1%,早发型患者为12.8%,两组间差异无统计学意义.与早发型患者相比,迟发型患者中环境因素为诱因者所占比例明显增高,且以精神因素为主.结论 迟发型白癜风患者和早发型患者在临床特征和流行病学方面存在一定的差异.     

青春发育开始时间与女生心理状况、问题行为关系的研究

目的研究青春发育开始时间与女生心理状况、问题行为的关系。方法选取2017年1月至2018年7月深圳市福田区妇幼保健院青少年门诊接受咨询的150例女生作为研究对象。利用青春期发育量表进行分组,将其分为早熟组(5分)35例、适时发育组(6分~17分)95例和晚熟组(18分)20例,对三组心理状况、问题行为进行对比和分析。结果三组焦虑评分、抑郁评分、问题行为评分相比较,早熟组>晚熟组>适时发育组,差异具有统计学意义(P<0.05)。结论青春发育开始时间过早或者是过晚均会给女性心理状态、问题行为带来严重不利影响,必须予以足够的重视并采取积极的干预措施以引导青春期女生身心健康成长。     

Two cases of late onset Ota's naevus

Ota's naevus is among the dermal melanocytoses that show a distinct pattern involving skin innervated by the trigeminal nerve. Most cases present at birth or manifest clinically in early childhood. Cases of acquired lesions in adult onset have been reported rarely. We present two cases of late onset Ota's naevus which were confirmed by skin biopsies. Both patients underwent Q-switched alexandrite laser treatment with a dose of 8.0 J/cm2 given four or five times at 6 weekly intervals and showed some improvement.     

Late-onset hydroa vacciniforme: two case reports

Hydroa vacciniforme (HV) is a rare blistering photodermatosis that heals with vacciniform scarring, with onset usually in childhood and spontaneous resolution by early adulthood. We report two cases of HV seen at the National Skin Centre, Singapore, both with a late onset at 20 years of age, during compulsory military service, and one with a less typical non-vesicular presentation, that initially caused some diagnostic difficulties.     

Congenital erythropoietic porphyria associated with myelodysplasia presenting in a 72-year-old man: report of a case and review of the literature

Congenital erythropoietic porphyria (CEP) is a rare autosomal recessive disease owing to the deficient activity of uroporphyrinogen III synthase, the fourth enzyme in the porphyrin-haem synthetic pathway. Of the porphyrias, it is the most mutilating type, usually presenting early in life. To date, 12 documented cases of adult onset CEP have been reported. We report the second oldest documented patient with late onset CEP with incidental findings of thrombocytopenia and myelodysplasia with bone-marrow sideroblasts. We further discuss several current and future treatment options for this therapeutically challenging disease.     

Lobular panniculitis at the site of subcutaneous interferon beta injections for the treatment of multiple sclerosis can histologically mimic pancreatic panniculitis. A study of 12 cases

Background:  Thrombosis, mucinosis and necrosis are well-described complications of subcutaneous interferon beta injections.
Methods:  We report 12 incisional biopsies from subcutaneous interferon beta injection sites in 12 multiple sclerosis (MS) patients from a single neurologist's practice.
Results:  We identified abscesses (two cases) or induration (two cases) in acute clinical lesions and lipoatrophy (eight cases) in chronic lesions (biopsied over a year after symptom onset at injection sites). Biopsies from three acute lesions showed vascular thrombosis, dermal mucinosis, lobular neutrophilic panniculitis, necrosis, calcification and hemosiderin deposition (biopsied 2 weeks to 2 months after symptom onset). Two cases contained sterile abscesses. Five of the eight chronic cases presented as hard, indurated lipoatrophy with livedo reticularis. Their biopsies showed subcutaneous calcification and lipoatrophy. Biopsies from the early calcific suppurative and late calcific atrophic phases histologically resembled the early and late phases of subcutaneous saponification in pancreatic panniculitis.
Conclusions:  Reactions at the site of subcutaneous interferon beta injections are common. Lipoatrophy can be clinically identified in 39 of 85 MS patients (46%) receiving subcutaneous interferon beta injections for 1 year or longer in our practice. A reaction to interferon should be considered in the differential diagnosis of biopsies that show features of pancreatic panniculitis.     

Characterization of early and late onset psoriasis in the Korean population.

It has been proposed that two types of psoriasis can be characterized based upon age of onset. The purpose of our study was to investigate the characteristics of early and late onset psoriasis in the Korean population. A total of 986 psoriasis patients were included in this study, and the age of onset frequency proved to be bimodal. Family history in the first-degree relatives was significantly higher in the early onset group (< 40 years old) when compared with the late onset group (> or = 40 years old). A series of statistical analyses concerning the correlation between the extent of involvement and age of onset showed that earlier onset is related to more extensive involvement. A questionnaire survey concerning the influence of various external factors upon their psoriasis was given to a subgroup of 800 psoriasis patients. Multiple logistic regression analysis, controlled for confounding factors such as current age, sex and extent of involvement, revealed that early onset psoriasis patients showed significantly increased tendencies to worsen at times of psychological stress and in winter, and to improve in summer, compared with late onset psoriasis patients. In conclusion, distribution of the age of onset revealed two peaks in Korean psoriasis patients, and psoriasis with an onset prior to the age of 40 years was associated with increased inheritability, greater susceptibility to seasonal changes and more psychological stress than psoriasis with later onset.     

Psoriasis of early and late onset: characterization of two types of psoriasis vulgaris

In 2,147 patients suffering from psoriasis, evaluation of the age of onset revealed two peaks, one occurring at the age of 16 years (female) or 22 years (males) and a second peak at the age of 60 years (female) or 57 years (males). Human lymphocyte antigen (HLA) tissue typing in 112 randomly assigned patients showed that HLA-Cw6, known to be at disequilibrium in psoriasis, is present in 85.3% of patients with early onset. In contrast, 14.7% patients with late onset showed this marker. Parents (father or mother) were affected in approximately half of the patients with early onset and in none belonging to the group with late onset. Furthermore, psoriasis in patients with early onset follows an irregular course and shows a strong tendency to become generalized. On the basis of clearly defined criteria (e.g., age of onset, heritability, and clinical course of disease), nonpustular psoriasis shows two distinct forms, one of which is hereditary, with early onset, and the other is sporadic and occurs in older age.     

Multiple minute digitate hyperkeratoses report of two cases with an updated review and proposal for a new classification.

Multiple minute digitate hyperkeratoses (MMDH) is a relatively rarely described disorder of keratinization of unknown origin. We noticed that MMDH has been reported in the literature under a variety of synonyms. We observed two patients with MMDH and reviewed the literature. We concluded that MMDH is a skin disorder which can be properly separated from other disorders of keratinization and classified as a distinct entity. Two forms of MMDH exist: MMDH of early onset and MMDH of late onset. Both forms are described occurring in a widespread and in a localized pattern. The early onset form may be hereditary, the late onset form may present as part of a paraneoplastic syndrome.     

Delayed onset filler complication: Two case reports and literature review

Soft tissue augmentation by fillers has become a popular cosmetic tool to offer rejuvenation and aesthetic improvement. Its results are comparable to those previously achieved only by plastic surgery. However, marked increase of filler procedures is associated with a great number of complications. Complications associated with filler injection might have early onset (within days) or late onset (after weeks to years). Delayed complications include infections, foreign body granulomatous reaction, migration of filler material, persistent discoloration, and scarring. Here we report two cases of delayed complications: one case of foreign body granuloma with migration and one case of acute inflammation involving a quiescent granuloma.     

Atypical familial Papillon–Lefèvre syndrome

The Papillon-Lefèvre syndrome is a rare autosomal recessive disorder. Consanguinity seems a notable prerequisite. Papillon-Lefèvre syndrome manifests in the first 6 months of life with rapidly progressive periodontitis and severe alveolar bone destruction leading to early loss of both the deciduous and permanent teeth in association with palmo-plantar hyperkeratosis. We present two unusual cases of familial Papillon-Lefèvre syndrome, one of whom has only late onset of mild skin lesions and the other has severe skin lesions and relatively mild periodontal disease. A number of other cases recently described have also had atypical features.     

Clinical study of psoriasis occurring over the age of 60 years: is elderly‐onset psoriasis a distinct subtype?

Background Previous studies demonstrated clinical differences of early‐ and late‐onset psoriasis. However, epidemiological data and clinical characteristics of psoriasis occurring in geriatric patients have been rarely studied. Objective Assessment of epidemiology and clinical features of psoriasis first occurring over the age of 60 years, so‐called elderly‐onset psoriasis, based on clinical data. Materials and methods Among 4049 patients visiting our psoriasis clinic for the last 27 years, patients were first divided into early‐ (onset age before 30 years) and late‐onset psoriasis (onset age after 30 years) based on our previous studies. Then, patients of late‐onset psoriasis were further divided into middle age‐onset group (onset age between 30 and 60 years) and elderly‐onset group (onset age over 60 years). Clinical characteristics of elderly‐onset psoriasis were compared with early‐ and middle age‐onset groups. We acquired the data both by physician’s assessments and patients’ responses. Results Elderly‐onset patients comprised 3.2% of total patients, 129 out of 4049. They have shown a lower incidence of family history (P < 0.05). The severity assessed by PASI score, body surface extent, and activity of individual lesions demonstrated that psoriasis of the elderly‐onset group was generally milder compared with early‐ and middle age‐onset groups (P < 0.05). In clinical phenotypes, the proportion of guttate type and generalized pustular psoriasis type decreased remarkably, while that of erythroderma type increased (P < 0.05). There was a significant change in the body part of origin comparing early‐ and middle age‐onset groups (P < 0.05). The proportion of scalp increased, while that of knee–elbow and trunk decreased significantly (P < 0.05). Patients’ subjective sensation of disease course improved statistically comparing early‐ and middle age‐onset groups (P < 0.05). There was no significant change in the degree of pruritus on psoriatic lesions and nail involvement (P > 0.05). Conclusion The elderly‐onset group demonstrated milder disease courses and some changes in clinical phenotypes and body part of origin compared with early‐ and middle age‐onset groups. Therefore, it seems that patients whose onset of psoriasis was over the age of 60 years might have distinct clinical features in some clinical aspects.     

009 Skin aging and photoaging effects can be quantified in vivo by fluoresence excitation spectroscopy

Congenital erythropoietic porphyria is a rare autosomal recessive disease due to the deficient activity of uroporphyrinogen III synthase, the fourth enzyme in the porphyrin-heme synthetic pathway. Of the porphyrias, it is the most mutilating type, usually presenting early in life. We present a patient who developed skin fragility of sun-exposed skin and red urine at the age of 72 years. Porphyrin profile showed plasma maximum fluorescence at neutral pH of 617 nm. In plasma, urine and erythrocytes, the predominant porphyrins were uroporphyrin and coproporphyrin. In all specimens, isomer I predominated. Urine δ-aminolevulinic acid and porphobilinogen, and erythrocyte uroporphyrinogen decarboxylase levels were within nomal limits. These finding were consistent with congenital erythropoietic porphyria. Thrombocytopenia and myelodysplasia with bone-marrow sideroblasts were also incidentally discovered. Including our patient, only 12 cases of late onset congenital erythropoietic porphyria have been reported worldwide, with our patient being the oldest. Patients who develop late onset congenital erythropoietic porphyria develop less severe manifestations, possibly due to heterogeneous mutations in the defective enzyme. Of the 12 reported cases, seven had thrombocytopenia, six of which also had myelodysplasia.     

Clinical, histopathological and bacteriological study of 52 referral MB cases relapsing after MDT

Fifty-two BB-LL relapse cases referred to our centre during 1997-2003 were investigated in detail. Twenty-four cases had been treated with extended MB-MDT [until smear negativity (NON-FDT)]. The remaining 28 cases (54%) had received one of the fixed duration regimens (FDT), of whom 11 had 24 months and 6 had 12 months of WHO MB-MDT. Eleven cases had received rifampicin/ofloxacin (RO) treatment. Follow-up slit skin smear reports were available for 41 cases, all but three cases had been smear negative at some point after release from treatment. None of the cases showed any clinical or bacteriological evidence of upgrading, i.e. LL to BT where as downgrading BB to BL occurred in five cases. The duration between cessation of treatment and reappearance of lesions (DCTR) varied from 2 to 15 years. The mean DCTR was longest (9.4 years) for the NON-FDT and 24 months MB-MDT cases. The mean DCTR was significantly lower in the 12 months MB-MDT and RO treated cases (6.8 and 6.2 years, respectively). Four of RO treated cases and four cases with multiple episodes of reaction had DCTR less than 5 years. Inadequate treatment/poor killing of Mycobacterium leprae results in early onset relapse, whereas 'persisting' or 'drug resistant mutants' contribute to late onset relapse.     

The clinical features of late onset acne compared with early onset acne in women

Background Little is known about the clinical characteristics of acne based on the age of onset. Objectives The aim of this study was to investigate the clinical characteristics of patients according to the age of onset of acne and evaluate whether the findings were related to regional differences in the density of Propionibacterium acnes or the levels of sebum secretion. Methods A total of 89 women were recruited. The acne lesions were assessed by counting the lesions using standard digital photographs. Digital fluorescent photography for the evaluation of the density of P. acnes were taken and quantitative measurements of facial sebum secretion were performed. Results In women with acne, the age of onset was negatively correlated with the number of comedones and the proportion of comedones. By comparing the number of comedones and the proportion of comedones, onset of acne after 21 years of age was defined as late onset acne. In the patients with late onset acne, the number of comedones, the total number of acne lesions and the proportions of comedones were significantly less than in the patients with early onset acne. However, there were no significant differences in the fluorescence density of P. acnes or the level of sebum secretion between the two groups. Conclusions The results of this study, using objective evaluation tools, suggest that late onset acne has different clinical characteristics. Other possible factors might explain the clinical differences in late onset acne.     

Analysis of phenotypic variation in psoriasis as a function of age at onset and family history

To evaluate the relationship between psoriasis disease severity, age at onset, and family history, we analyzed 537 US psoriatics, most of whom were from Michigan. Total body surface area involvement (%TBSA), presence or absence of joint complaints, and nail involvement were measured. Analysis of familial psoriatics revealed that %TBSA was 15.1% when onset was early, but only 8.7% when onset was late ( P=0.00003). The opposite trend was seen when psoriasis was sporadic: %TBSA was 14.3% when onset was early (相似文献   

Group B streptococcal infection: a review and update.

The problems posed by the sudden increase in serious group B streptococcal disease among infants since the early 1970s are described and discussed. Virtually all offspring of colonised mothers harbour the organism and infection develops in about 1% of these infants. The mortality rate, even with immediate antibiotic treatment of early onset disease, is 30%; delay in treatment leads to much higher mortality. Late onset disease starting around the seventh to ninth day of life, but sometimes as late as the second month, is less frequently fatal. Preventive measures include active and passive immunisation or intravenous ampicillin during labour. Experimental evidence indicates that each of these methods gives protection.     

Group B streptococcal infection: a review and update.

The problems posed by the sudden increase in serious group B streptococcal disease among infants since the early 1970s are described and discussed. Virtually all offspring of colonised mothers harbour the organism and infection develops in about 1% of these infants. The mortality rate, even with immediate antibiotic treatment of early onset disease, is 30%; delay in treatment leads to much higher mortality. Late onset disease starting around the seventh to ninth day of life, but sometimes as late as the second month, is less frequently fatal. Preventive measures include active and passive immunisation or intravenous ampicillin during labour. Experimental evidence indicates that each of these methods gives protection.