家族性慢性良性天疱疮

J A M A CAD D ERMATOLV OLUME 72, N UMBER 4National Skin Center a and National University Hospital,b SingaporeSupported by the National Medical Research Coun-cil, Singapore.Conflicts of interest: None declared. Correspondence to: Steven Thng Tien Guan, MBBS, MRCP(UK), National Skin Center, 1 Mandalay Road, Singapore 308205E-mail: steventhng@.sgREFERENCES1. Gawkrodger DJ, Ormerod AD, Shaw L, et al. Guideline forthe diagnosis and management of vitiligo. Br J Dermatol.2008; 159:1051-1076.2. Anbar TS, Westerhof W, Abdel-Rahman AT, El-Khavvat MA.Evaluation of the effects of NB-UVB in both segmental and non-segmental vitiligo affecting different body sites.Photodermatol Photoimmunol Photomed. 2006;22:157-163. 3. Novak Z, Bonis B, Baltas E, et al. Xenon chlorideultraviolet B laser is more effective in treating psoriasis and in inducing T cell apoptosis than narrow band ultraviolet B. J Photochem Photobiol B. 2002;67:32-38.4. Bianchi B, Campolmi P, Mavilia L, Danesi A, Rossi R,Cappugi P. Monochromatic excimer light (308 nm): an immunohisto-chemical study of cutaneous T cells and apoptosis-related molecules in psoriasis. J Eur Acad Dermatol Venereol. 2003;17: 408-413.5. Morelli JG, Kincannon J, Yohn JJ, Zekman T, Weston WL,Norris DA. Leukotriene C4 and TGF-alpha are stimulator of human melanocyte migration in vitro. J Invest Dermatol.1992; 98:290-295./10.1016/j.jaad.2014.12.026 Successful treatment of refractory Hailey-Hailey disease with a 595-nm pulsed dyelaser: A series of 7 casesTo the Editor: Hailey-Hailey disease is a rare and historically difficult to treat genodermatosis charac-terized by recurrent bullous eruptions in flexural regions. Among laser modalities, carbon-dioxide laser ablation has been most commonly used to treat refractory disease; however, severe erythema, pro-longed healing times, and significant pain limit its therapeutic usefulness.1Other destructive therapies (eg, argon plasma coagulation2and cold atmo-spheric plasma3) have been met with poor to inter-mediate success. In 2006, Fisher and Geronemus4described tentative success in treating 1 patient with Hailey-Hailey disease using a 595-nm pulsed dye laser (PDL); however no follow-up studies, to our knowledge, have confirmed this finding.Seven patients with biopsy-confirmed, recalci-trant Hailey-Hailey disease were offered PDL after failing to respond to accepted medical treat-ments (eg, trigger avoidance, steroids, antibiotics,Letters 735 antimicrobials). After observing striking improve-ment with PDL therapy, their cases were retrospec-tively aggregated and studied. All of the participants were female with reported disease onset between ages 19 to 39 years. The most common areas of involvement were the inguinal folds, abdomen,inframammary folds, and axillae (Table I).During treatment with PDL, the affected areas were anesthetized with topical benzocaine, lidocaine, tetracaine (20%/8%/4%) and treated with a 595-nm PDL using subpurpuric settings (pulse duration 6-10 milliseconds, spot size 7-10 mm, fluence 7-10 J/cm2). Treatments were repeated every 2 to 4 weeks (average of 5 total treatments). Postprocedure care instructions included cleansing with dilute vinegar water (1 tablespoon white vinegar in 1 pint water), twice-daily topical petroleum jelly application, and heat and friction avoidance. Two patients (1 and 3) experienced presumed herpes simplex virus flares (1 within the PDL-treated area) during the study period, although this was not confirmed through testing. Oral valacyclovir completely resolved both patients’ outbreaks with no further complication.Six of 7 patients experienced varying degrees of resolution (Fig 1) whereas 1 participant declined further treatment because of perceived ineffective-ness. Time to resolution ranged from 2 weeks to 2 months and 2 patients exhibited no signs of relapse after treatment. Of the 4 patients who relapsed, only 1 exhibited a severe flare, which required oral steroids. Patients reported subjective improvement in their time to relapse, which ranged from 1 to 4 months and averaged 2 months.Although the definitive mechanism for improve-ment in Hailey-Hailey disease treated with PDL has yet to be elucidated, we propose the following possibilities that may serve as avenues of further research. First, PDL-induced photothermal injury to the dermis has been found to promote cutaneous collagen production and dermal remodeling, poten-tially resulting in improved wound healing.5In addition, PDL may reduce the heat characteristically produced at sites of lesional skin and decrease flare recurrence by targeting and diminishing small dermal blood vessels. With guarded optimism, we have demonstrated that improvement and clearance of Hailey-Hailey disease is possible using PDL, which may provide a safe and effective alternative to other therapies. Further study is warranted to confirm our findings, to better understand the mechanism by which these outcomes occurred, and to explore the use of 595-nm PDL in the treatment of other acantholytic skin diseases.736 Letters J A M A CAD D ERMATOLA PRIL 2015Table I. Patient characteristics and outcomesAge attreatment Areas of No. of Fluence, Days to Degree of Days to Degree ofCase initiation, y involvement treatments J/cm2 resolution resolution relapse relapse Adverse events1 37 Inguinal folds, 5 8-9 14 Mild to 120 Unknown Presumed HSVabdomen, complete flare withininframammary, PDL-treatedgluteal cleft lesion2 58 Inguinal folds, 5 8-10 14 Mild N/A No relapse Mild discomfortinframammary, reported duringaxilla, back procedure3 41 Inguinal folds, 5 7-9 28 Complete N/A No relapse Presumed HSVabdomen, reported flare beforegluteal cleft, initiation ofback, buttocks, treatmenthips, legs4 71 Abdomen, gluteal 8 7-9 17 Moderate 34 Mild Nonecleft5 76 Inguinal folds, 9 7.5-9.5 56 Moderate 70 Mild Noneaxilla6 36 Inguinal folds, 4 7-9.5 45 Mild 30 Severe Noneinframammary,axilla7 36 Abdomen, 2 7-7.5 N/A N/A N/A Patient reported Noneinframammary, minimalaxilla, chest, improvement,neck, oral decided tomucosa discontinuetreatmentsHSV, Herpes simplex virus; N/A, not applicable; PDL, pulsed dye laser.Fig 1. Hailey-Hailey disease. Maceration and erosion in inguinal fold; at initial visit (A) andon day 60 after 2 treatments with pulsed dye laser on day 0 and day 14 (B).Katherine M. Hunt, BS,a J. Daniel Jensen, MD,b Stephanie B. Walsh, MD,b Mary E. Helms, MHS,c Vera Y. Soong, MD,d Elizabeth S. Jacobson, MD,c Naveed Sami, MD,b Conway C. Huang, MD,bAmy Theos, MD,b and Marian E. Northington, MD b School of Medicine a and Department of Dermatology,b University of Alabama at Bir-mingham; Inverness Dermatology and Laser,Hoover, Alabama c; and Southeastern Derma- tology Center, Birmingham, Alabama dFunding sources: None.Conflicts of interest: None declared.Preliminary results of this investigation were pre-sented at the American Society for Dermatologic Surgery Annual Meeting, November 10-13, 2014, San Diego, California.J A M A CAD D ERMATOLV OLUME 72, N UMBER 4Correspondence to: Katherine M. Hunt, BS, 2716 11 Ave S, Apt 12, Birmingham, AL 35205E-mail: mmarchio@REFERENCES1. Kartamaa M, Reitamo S. Familial benign chronic pemphigus(Hailey-Hailey disease): treatment with carbon dioxide laser vaporization. Arch Dermatol. 1992;128:646-648.2. Stolze I, Hamm H, Weyandt GH. Segmental multilayeredargon plasma coagulation: effective therapy option for perianal and scrotal Hailey-Hailey disease. Colorectal Dis.2011;13:802-804.3. Isbary G, Morfill G, Zimmermann J, et al. Coldatmospheric plasma: a successful treatment of lesions in Hailey-Hailey disease. Arch Dermatol. 2011;147:388-390.4. Fisher GH, Geronemus RG. Improvement of familialbenign pemphigus after treatment with pulsed-dye laser: a case report. Dermatol Surg. 2006;32:966-968.5. Seaton ED, Mouser PE, Charakida A, Alam S, Seldon PM,Chu AC. Nonablative laser and light treatments: histology and tissue effectsea review. Lasers Surg Med. 2003;33:30-39./10.1016/j.jaad.2014.12.023 The readability of patient education materials designed for patients with psoriasis: What have we learned in20 years?To the Editor: Patient education is essential for successful therapeutic outcomes. It is recommended that information be delivered at the 6th grade reading level.1Twenty years ago, Feldman et al2 demonstrated in the Journal of the AmericanLetters 737 Academy of Dermatology that printed patient materials were delivered at reading levels higherthan 6th grade level (Table I). Here we evaluate whether newer online patient education materials developed in the intervening 20 years are morereadable and accessible for patients.To allow comparison with the earlier work of Feldman et al, we identified patient brochures from the websites of the Arthritis Foundation (AF) on the topic of methotrexate and psoriatic arthritis, the American Academy of Dermatology (AAD) on psoriasis, and the National Psoriasis Foundation (NPF) on methotrexate and narrowband ultraviolet B (NBUVB) (Table II). For better intersite compara-bility, we also analyzed information on psoriatic arthritis, methotrexate, and NBUVB from the AAD website, as well as psoriasis and psoriatic arthritis from the NPF website. We analyzed only the first page of the websites because, if the reader does not understand the first page, then he or she is far less likely to continue reading. Analysis utilized standard formulae for Flesch-Kincaid grade level as used by Feldman et al.2We also include the results of analysis using more recently developed reading scales3 (Table I).Flesch-Kincaid grade levels range from 8.7 to 12.7. Although absolute values vary between the different indices, the trends do not. Among the 3 organiza-tions, the AAD appears to have the lowest grade level information. Their information on psoriasis, which has improved since 1994, and their information onTable I. Results of readability analysis of patient information on websitesAAD NPF AFPs PsA M NBUVB Ps PsA M NBUVB Ps PsA M Character count 1314 1734 1594 2492 1254 1402 1481 2726 2228 1296 Syllable count 454 604 524 844 425 463 486 923 740 426 Word count 264 349 286 513 259 273 279 534 433 244 Sentence count 23 28 20 41 15 15 14 38 21 19 Characters per word 5.0 5.0 5.6 4.9 4.8 5.1 5.3 5.1 5.1 5.3 Syllables per word 1.7 1.7 1.8 1.6 1.6 1.7 1.7 1.7 1.7 1.7 Words per sentence 11.5 12.5 14.3 12.5 17.3 18.2 19.9 14.1 20.6 12.8 Flesch reading score 49.7 47.8 37.3 54.9 50.5 44.9 39.2 46.3 41.3 46.1 Flesch-Kincaid grade level 9.2 9.7 11.6 8.7 10.5 11.5 12.7 10.3 12.6 10 Flesch-Kincaid from Feldman et al 11 * * * * * 12 12 8 * 8 1994Gunning-Fog score 9.9 12.8 14.4 10.6 13.4 14 14.3 13.2 15.5 10.2 Coleman-Liau 13.5 13.4 17 12.8 12.7 14.4 15.5 14.2 14.5 15.5 SMOG index 8.7 9.9 10.5 8.3 10.3 10.8 10.8 9.7 11.4 9.2 Automated readability index 7.8 8.2 12 7.7 10 11.9 13.5 9.6 13.1 10 Average grade level 9.8 10.8 13.1 9.6 11.4 12.5 13.4 11.4 13.4 11.0 AAD, American Academy of Dermatology; AF, Arthritis Foundation; M, methotrexate; NBUVB, narrowband ultraviolet B; NPF, National Psoriasis Foundation; Ps, psoriasis; PsA, psoriatic arthritis.*Not analyzed.。