Acrodermatitis enteropathica is a rare autosomal recessive disease that results from a defect in zinc metabolism. It is clinically characterized by a phenotypic triad of periorificial and acral dermatitis, diarrhea, and alopecia. Oral zinc therapy gives a rapid excellent clinical response and reduces mortality. We report three female pediatric siblings who presented with periorificial and acral dermatitis, diffuse alopecia, nail dystrophy, irritable mood, and stunted growth. A diagnosis of acrodermatitis enteropathicawas confirmed with markedly decreased levels of serum zinc. The patients were successfully treated with oral zinc sulfate at a dose of 5mg/kg/day for the first two weeks then maintained on a dose at 2mg/kg/day.
Zinc ; Blister

Objective To investigate the clinical characteristics and genetic mutations in Kindler syndrome(KS)and provide a theoretical basis for the diagnosis and treatment of KS. Methods The clinical data of one case of KS from Peking Union Medical College Hospital and 185 cases reported in literature were collected. The gene mutation types,patient clinical data,and tumor characteristics were statistically analyzed. Results A total of 186 cases were enrolled,including 110 males and 76 females,with the mean age of(28±16)years. The data of gene mutation and specific clinical manifestations were available in 151 and 94 patients,respectively. The main clinical manifestations of KS included poikiloderma,occurrence of blister in childhood,and photosensitivity,and the secondary clinical manifestations included oral inflammation,palmoplantar keratoderma,webbing/pseudoainhum,dysphagia,urethral stricture and so on.Oral inflammation(r=0.234,P=0.023),palmoplantar keratoderma(r=0.325,P=0.001),webbing/pseudoainhum(r=0.247,P=0.016),dysphagia(r=0.333,P=0.001),urethral stricture(r=0.280,P=0.006)were significantly correlated with age,showing significantly higher incidence in the patients over 32 years old.Urethral stricture(χ²=11.292,P=0.001)and anal stenosis(χ²=4.014,P=0.045)were significantly correlated with sex,with higher incidence in males.Eighty different mutations were found in 151 patients,and the most common gene mutation was c.676C>T.Forty-one tumors occurred in 27 patients,among which squamous cell carcinoma accounted for 92.7%. The gene mutation site had no significant correlation with squamous cell carcinoma or patient country. Conclusions The c.676C>T in FERMT1 gene is the most common mutation in KS.The patients are prone to squamous cell carcinoma and mainly attacked at the exposure sites(hand and mouth).
Adolescent ; Adult ; Ainhum ; Blister ; Carcinoma, Squamous Cell ; Child ; Constriction, Pathologic ; Deglutition Disorders/complications* ; Epidermolysis Bullosa ; Female ; Humans ; Inflammation ; Keratoderma, Palmoplantar/complications* ; Male ; Membrane Proteins ; Mutation ; Neoplasm Proteins/genetics* ; Periodontal Diseases ; Photosensitivity Disorders ; Urethral Stricture/complications* ; Young Adult

Objective: To assess the current situation of early treatment of partial-thickness burn wounds by professional burn medical staff in China, and to further promote the standardized early clinical treatment of partial-thickness burn wounds. Methods: A cross-sectional investigation was conducted. From November 2020 to February 2021, the self-designed questionnaire for the early treatment of partial-thickness burn wounds was published through the "questionnaire star" website and shared through WeChat to conduct a convenient sampling survey of domestic medical staff engaged in burn specialty who met the inclusion criteria. The number, region, and grade of the affiliated hospital, the age, gender, occupation, and seniority of the respondents were recorded. The respondents were divided into physician group and nurse group, senior group and junior group, eastern region group and non-eastern region group, primary and secondary hospital group and tertiary hospital group. Then the seniority, grade of the affiliated hospital, region of the affiliated hospital of the respondents in physician group and nurse group, conventional treatment of partial-thickness burn blisters, reasons for retaining vesicular skin, reasons for removing vesicular skin, and the conventional selection and optimal solution recommendation of topical drugs or dressings for partial-thickness burn wounds in the early stage of respondents in each of all the groups were recorded. Data were statistically analyzed with chi-square test. Results: The survey covered 31 provinces, municipalities, and autonomous regions in China (except for Hong Kong, Macau, and Taiwan regions). A total of 979 questionnaires were recovered, which were all valid. The 979 respondents came from 449 hospitals across the country, including 203 hospitals in the eastern region, 116 hospitals in the western region, 99 hospitals in the central region, and 31 hospitals in the northeast region, 348 tertiary hospitals, 79 secondary hospitals, and 22 primary hospitals. The age of the respondents was (39±10) years. There were 543 males and 436 females, 656 physicians and 323 nurses, 473 juniors and 506 seniors, 460 in the eastern regions and 519 in the non-eastern regions, 818 in tertiary hospitals and 161 in primary and secondary hospitals. There were statistically significant differences in the composition of different seniority in the respondents between physician group and nurse group (χ²=44.32, P<0.01), while there were no statistically significant differences in grade or region of the affiliated hospital of the respondents between physician group and nurse group (P>0.05). There were no statistically significant differences in the conventional treatment of partial-thickness burn blisters among respondents between different occupational groups, seniority groups, and region of the affiliated hospital groups (P>0.05).The respondents in different grade of the affiliated hospital groups differed significantly in the conventional treatment of partial-thickness burn blisters (χ²=6.24, P<0.05). Compared with respondents in nurse group, larger percentage of respondents in physician group chose to retain vesicular skin for protecting the wounds and providing a moist environment, and alleviating the pain of dressing change (with χ² values of 21.22 and 19.96, respectively, P values below 0.01), and smaller percentage of respondents in physician group chose to retain vesicular skin for prevention of wound infection (χ²=23.55, P<0.01). The reasons for retaining vesicular skin of respondents between physician group and nurse group were similar in accelerating wound healing, alleviating pigmentation and scar hyperplasia post wound healing (P>0.05). Compared with respondents in junior group, larger percentage of respondents in senior group chose to retain vesicular skin for protecting the wounds and providing a moist environment and alleviating the pain of dressing change (with χ² values of 10.36 and 4.60, respectively, P<0.05 or P<0.01), and smaller percentage of respondents in senior group chose to retain vesicular skin for prevention of wound infection (χ²=8.20, P<0.01). The reasons for retaining vesicular skin of respondents in senior group and junior group were similar in accelerating wound healing, alleviating pigmentation and scar hyperplasia post wound healing (P>0.05). The 5 reasons for the respondents between eastern region group and non-eastern region group, primary and secondary hospital group and tertiary hospital group chose to retain vesicular skin were all similar (P>0.05). Compared with those in physician group, significantly higher percentage of respondents in nurse group were in favor of the following 6 reasons for removing the vesicular skin, including convenience for using more ideal dressings to protect the wounds, prevention of wound infection, facilitating the effect of topical drugs on the wounds, the likely rupture of blisters and wound contamination, accelerating wound healing, and alleviating pigmentation and scar hyperplasia post wound healing (with χ² values of 4.35, 25.59, 11.83, 16.76, 46.31, and 17.54, respectively, P<0.05 or P<0.01). Compared with respondents in senior group, larger percentage of respondents in junior group chose to remove vesicular skin for the reasons such as the likely blister rupture and wound contamination, preventing wound infection, accelerating wound healing, and alleviating pigmentation and scar hyperplasia post wound healing (with χ² values of 17.25, 18.63, 14.83, and 10.23, respectively, P values below 0.01). Compared with respondents in non-eastern region group, larger percentage of respondents in eastern region group chose to remove vesicular skin for preventing wound infection and the likely rupture of blisters and wound contamination (with χ² values of 9.30 and 8.65, respectively, P values below 0.01). The 6 reasons for the respondents between tertiary hospital group and primary and secondary hospital group choose to remove vesicular skin were similar (P>0.05). Compared with respondents in physician group, larger percentage of respondents in nurse group chose to use moisturizing materials for partial-thickness burn wounds in the early stage (χ²=6.18, P<0.05), and smaller percentage of respondents in nurse group chose other topical drugs or dressings (χ²=5.20, P<0.05). Compared with respondents in junior group, larger percentage of respondents in senior group chose to use moisturizing materials and other topical drugs or dressings for partial-thickness burn wounds in the early stage (with χ² values of 4.97 and 21.80, respectively, P<0.05 or P<0.01). Compared with respondents in non-eastern region group, larger percentage of respondents in eastern region group chose to use topical antimicrobial drugs for partial-thickness burn wounds in the early stage (χ²=4.09, P<0.05), and smaller percentage of respondents in eastern region group chose to use other topical drugs or dressings for the partial-thickness burn wounds in the early stage (χ²=5.63, P<0.05). Compared with respondents in primary and secondary hospital group, larger percentage of respondents in tertiary hospital group chose to use biological dressings for partial-thickness burn wounds in the early stage (χ²=9.38, P<0.01). The optimal solution recommendation of topical drugs or dressings for partial-thickness burn wounds in the early stage varied significantly among the respondents between different occupational groups and seniority groups (with χ² values of 39.58 and 19.93, respectively, P values below 0.01). There were no statistically significant differences between eastern and non-eastern region groups, tertiary hospital group and primary and secondary hospital groups in optimal solution recommendation of topical drugs or dressings for partial-thickness burn wounds in the early stage (P>0.05). Conclusions: The conventional treatment measures of partial-thickness burn blisters and reasons for preserving blister skin by professional burn medical staff in China are relatively consistent, but there are great differences in the selection of reasons for removing blister skin, the conventional selection and optimal solution recommendation of topical drugs or dressings for partial-thickness burn wounds in the early stage. Therefore, it is urgent to establish a clinical treatment standard for partial-thickness burn wounds.
Adult ; Blister ; Burns/drug therapy* ; Cicatrix/pathology* ; Cross-Sectional Studies ; Female ; Humans ; Hyperplasia ; Male ; Medical Staff ; Middle Aged ; Occupations ; Pain ; Soft Tissue Injuries ; Wound Infection

Allyl alcohol is an intermediate widely used in industrial production activities, which has caused many occupational damage in China. In this paper, the data of a case of skin damage and clinical cure of inhalation pneumonia caused by occupational allyl alcohol exposure were analyzed and summarized. The patient accidentally inhaled large amounts of allyl alcohol at work and had skin exposure, which was mainly manifested as aspiration pneumonia, respiratory failure, eye damage and skin bullae. After short-term hormone shock therapy, mechanical ventilation, infection prevention, local treatment and other comprehensive treatment, the patient was clinically cured. This case suggests that chemical pneumonia and respiratory failure may be caused by inhalation of allyl alcohol, and skin bullae may be caused by skin contact. Early treatment and hormone use should be emphasized.
Blister ; Hormones ; Humans ; Pneumonia ; Propanols ; Respiratory Insufficiency

Objective: To investigate the clinical effects of ultra-pulsed fractional carbon dioxide laser (UFCL) in the treatment of mild to moderate microstomia after burns. Methods: A retrospective observational study was conducted on 19 patients with mild to moderate microstomia after burns who were admitted to Inner Mongolia Baogang Hospital from January 2018 to January 2022, including 15 males and 4 females aged (35±14) years. Patients had an average course of 71 d of microstomia, with 8 cases of moderate microstomia and 11 cases of mild microstomia. All the patients received UFCL treatment every 2-3 months until the microstomia was corrected or the treatment bottleneck was reached. The times of UFCL treatment for patients and the time interval from the last treatment to the last follow-up were recorded. Before the first treatment and at the last follow-up, the opening degree of mouth (finger measurement method), oral gap width, and the distance between the upper and lower incisors during mouth opening were recorded. Before the first treatment and at the last treatment, the new Vancouver scar scale (VSS) was used to evaluate the scar. At the last follow-up, the degree of satisfaction was evaluated by the Likert 5 scale by the patients themselves, and the satisfaction ratio was calculated; the adverse reactions such as pigmentation, blisters, infection, and persistent erythema in the treatment area were counted. Data were statistically analyzed with Mann-Whitney rank sum test or paired sample t test. Results: Patients received UFCL treatment of 3 (2, 6) times. The interval from the last treatment to the last follow-up was 26 months at most and 4 months at least. At the last follow-up, the opening degree of mouth of patients was significantly improved than that before treatment (Z=4.68, P<0.01). At the last follow-up, the oral gap width of patients was (35±6) mm, and the distance between upper and lower incisors during mouth opening was (3.2±0.4) cm, which was significantly improved compared with those before treatment (with t values of 10.73 and 18.97, respectively, P<0.01). The VSS score after the last treatment was 4.1±1.6, which was significantly better than that before treatment (t=22.96, P<0.01). At the last follow-up, the satisfaction ratio of patients with treatment was 18/19, and no pigmentation, blisters, infection, persistent erythema, and other adverse reactions of all patients in the treatment area occurred, however, one of the patients reported that the disease recurred about half a year after treatment. Conclusions: UFCL is an effective method for treating mild to moderate microstomia after burns, with which patients are highly satisfied, and it is worth of further study and promotion.
Blister ; Burns/therapy* ; Cicatrix/therapy* ; Female ; Humans ; Lasers, Gas/therapeutic use* ; Male ; Microstomia ; Treatment Outcome

Burns often cause the damaged tissue to produce a large amount of exudate and the formation of blisters on the wound. The burn blister fluid contains a large number of molecules related to wound healing, which can reflect the state of local tissue microenvironment of the burn wound. Analyzing relevant information such as cellular components, signal mediators, and protein molecules in burn blister fluid is helpful to understand the local reaction and tissue microenvironment of burn wounds, and then help clinical burn treatment. In this article, by understanding the production mechanism of burn blister fluid, discussing its role in wound evaluation, and integrating the research progress of burn blister fluid in proteomics, metabolomics, cellular components, and pharmacokinetics, we propose our thoughts and prospects on the research of burn blister fluid, in order to provide assistance for clinical evaluation and treatment of burn wounds, and also provide idea for the follow-up study of burn blister fluid.
Humans ; Blister/metabolism* ; Follow-Up Studies ; Burns/metabolism* ; Exudates and Transudates/metabolism* ; Wound Healing

A girl, aged 12 years, was admitted due to fever and rash for 3 days. The child developed recurrent high fever and rash on both lower extremities 3 days before, and the rash on left lower extremity quickly merged into a patch within 24 hours, with hemorrhage and necrosis in black and purple, large vesicles, and blisters in the center. Laboratory examination showed a reduction in platelet count and significant increases in fibrinogen and D-dimer during the course of the disease. The child was diagnosed with purpura flulminans. She was given meropenem combined with linezolid for anti-infection, injection of gamma globulin for immunoregulation, and low-molecular-weight heparin for anticoagulation. The fluid in the rash blisters was drawn and the wound was treated to prevent infection. The child's temperature returned to normal, with improvement in gangrene. She was discharged after platelet count, fibrinogen, and D-dimer had returned to normal. Purpura fulminans is a rare thrombotic hemorrhagic disease with rapid progression and is commonly seen in children. Without timely treatment, it may cause severe sequelae with high disability and mortality rates. Anti-infection, correction of coagulation function, and local management of gangrene skin are of great importance during treatment.
Blister ; Child ; Exanthema ; Female ; Fever ; Humans ; Lower Extremity ; Necrosis

Drug reaction with eosinophilia and systemic symptoms (DRESS) syndrome and toxic epidermal necrolysis (TEN) are severe cutaneous adverse reactions. Although viral reactivation is associated with DRESS syndrome, its role in TEN remains unclear. An 80-year-old woman visited our hospital because of fever and skin eruption. DRESS syndrome was diagnosed and was thought to caused by the use of the drug allopurinol. She was treated by discontinuation of the drug and administration of systemic steroids. She recovered from DRESS syndrome and was discharged from the hospital with tapering doses of steroids prescribed. One week after discharge, she visited our hospital again as the skin rash recurred and oral pain as well as oral and ocular mucosal lesions developed. In addition to the skin rash, blisters and Nikolsky's sign that were different from the skin lesions present in the previous DRESS syndrome were observed. Unlike those in DRESS syndrome, the viral serological test results were positive for anti-cytomegalovirus (CMV) IgM and CMV polymerase chain reaction. Therefore, it was thought that TEN was due to reactivation of CMV and she was treated this with ganciclovir and intravenous immunoglobulin. Here, we report a case of TEN caused by viral reactivation after DRESS syndrome developed after use of allopurinol which recovered after steroid treatment.
Aged, 80 and over ; Allopurinol ; Blister ; Cytomegalovirus Infections ; Cytomegalovirus ; Drug Hypersensitivity Syndrome ; Eosinophilia ; Exanthema ; Female ; Fever ; Ganciclovir ; Humans ; Immunoglobulin M ; Immunoglobulins ; Polymerase Chain Reaction ; Serologic Tests ; Skin ; Steroids ; Stevens-Johnson Syndrome

OBJECTIVE: To describe our experiences with a fully equipped high-end digital subtraction angiography (DSA) system within a hybrid operating room (OR).METHODS: A single-plane DSA system with 3-dimensional rotational angiography, cone-beam computed tomography (CBCT), and real-time navigation software was used in our hybrid OR. Between April 2014 and January 2018, 191 sessions of cerebrovascular procedures were performed in our hybrid OR. After the retrospective review of all cases, the procedures were categorized into three subcategorical procedures : combined endovascular and surgical procedure, complementary rescue procedure during intervention and surgery, and frameless stereotaxic operation.RESULTS: Forty-nine of 191 procedures were performed using hybrid techniques. Four cases of blood blister aneurysms and a ruptured posterior inferior cerebellar artery aneurysm were treated using bypass surgery and endovascular trapping. Eight cases of ruptured aneurysm with intracranial hemorrhage (ICH) were treated by partial embolization and surgical clipping. Six cases of ruptured arteriovenous malformation with ICH were treated by Onyx embolization of nidus and subsequent surgical removal of nidus and ICH. Two (5.4%) of the 37 cases of pre-mature rupture during clipping were secured by endovascular coil embolization. In one (0.8%) complicated case of 103 intra-arterial thrombectomy procedures, emergency surgical embolectomy with bypass surgery was performed. In 27 cases of ICH, frameless stereotaxic hematoma aspiration was performed using XperGuide® system (Philips Medical Systems, Best, the Netherlands). All procedures were performed in single sessions without any procedural complications.CONCLUSION: Hybrid OR with a fully equipped DSA system could provide precise and safe treatment strategies for cerebrovascular diseases. Especially, we could suggest a strategy to cope flexibly in complex lesions or unexpected situations in hybrid OR. CBCT with real-time navigation software could augment the usefulness of hybrid OR.
Aneurysm ; Aneurysm, Ruptured ; Angiography ; Angiography, Digital Subtraction ; Arteries ; Arteriovenous Malformations ; Blister ; Cerebrovascular Disorders ; Cone-Beam Computed Tomography ; Embolectomy ; Embolization, Therapeutic ; Emergencies ; Hematoma ; Intracranial Hemorrhages ; Operating Rooms ; Retrospective Studies ; Rupture ; Surgical Instruments ; Thrombectomy

A girl, aged 15 months, attended the hospital due to recurrent skin erythema, blisters, and desquamation for more than 7 months. Giemsa staining and immunohistochemical staining showed mast cell infiltration and degranulation. Hematoxylin staining showed spinous layer edema and blister formation under the epidermis, with a large amount of serous fluid and a small number of inflammatory cells in the blister. Marked edema was observed in the dermis, with diffused mononuclear cell infiltration. The girl was diagnosed with mastocytosis. Mastocytosis should be considered for children with recurrent skin erythema and blisters.
Blister ; Female ; Humans ; Infant ; Mast Cells ; Skin