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Advances in dermatology and venereology Acta Dermato-Venereologica
Treatment of Oral Lesions in Dystrophic Epidermolysis Bullosa : A Case Series of Cord Blood Platelet Gel and Low-level Laser Therapy
Ezio SINDICI 1 , Simona ASTESANO 1 , Luigina FAZIO 2 , Antonella DRAGONETTI 2 , Mariateresa PUGLIESE 2 , Crispian SCULLY 3 , Stefano CAROSSA 1 , Roberto BROCCOLETTI 1 and Paolo G . ARDUINO 1 *
1
CIR-Dental School , Department of Surgical Sciences , University of Turin , Via Nizza 230 , 10126 Turin , 2 Torino Cord Blood Bank – Immunohematology and Transfusional Medical Service , A . O . U . Città della Salute e della Scienza , Turin , Italy , and 3 World Health Organization Collaborating Centre for Oral Health-General Health , UK . * E-mail : paologiacomo . arduino @ unito . it Accepted Aug 16 , 2016 ; Epub ahead of print Aug 18 , 2016
Inherited epidermolysis bullosa ( EB ) is a rare group of genetically heterogeneous diseases , characterized by deficiencies in the adhesion of the connective tissue to the epithelium . Junctional EB and dystrophic EB , are the most severe types . Common clinical manifestations are mechanical fragility of the skin and mucosae , with blister formation and abnormal wound healing , the severity of the lesions depending on the distinct type ( 1 – 3 ). The oral cavity is frequently involved , but , although bullae and erosions are common , there is only one published study of therapy , reporting that sucralfate suspension can reduce the development and duration of blisters and ulcers , reduce the associated pain , and improve indices of gingival inflammation ( 4 ).
Autologous and allogeneic platelet ( PLT ) preparations , in particular a blood component termed “ platelet gel ”, traditionally obtained from adult blood platelets , are rich in regenerative growth factors , which are valu able for the treatment of chronic wounds ( 5 ). Recent findings on multiple biological properties of human umbilical cord blood ( CB ), and its high level of viral safety , prompted one group to produce PLT gel from CB ( cord blood platelet gel ; CBPG ) ( 6 ), and another to detail its effectiveness for the treatment of EB skin lesions ( 7 ).
Low-level laser therapy ( LLLT ) has potential biostimulating effects , improving wound healing , and is a possible treatment for autoimmune oral erosive lesions , with a notable analgesic effect ( 8 ).
We therefore performed a pilot evaluation of the efficacy and safety of CBPG with LLLT for the treatment of inherited EB oral mucosal lesions , over a 3-day treatment period ( one application each day ).
PATIENTS AND METHODS
Patients with dystrophic EB and symptomatic oral lesions were prospectively selected between June and October 2015 . The ethics review board of the “ Azienda Ospedaliera Città della Salute e della Scienza of Turin ”, Turin , Italy , approved the study ( protocol number 0089210 _ CS / 585 / 09-2015 ). Participants with long-standing ulcerations were selected .
CB units not fulfilling the criteria for banking for transplant purposes were processed within 48 h of collection to obtain CBPG according to the “ Italian Cord Blood Platelet Gel project ” protocol ( 9 ) ( for complete details see Appendix S1 1 ).
Morphological changes were measured with a 15-mm periodontal probe ( PCPUNC15 : Hu-Friedy ® , Chicago , IL , USA ).
The symptom score ( for reporting pain ) was recorded using a visual analogue scale ( VAS ); patients were requested to mark the scale at each visit , before and after the laser session .
The primary outcome measures were lesions ’ size and pain ; secondary outcome measure was adverse effects . All data were collected during a 6-month follow-up period ( at 1 ( T4 ), 4 ( T5 ), 12 ( T6 ) and 24 ( T7 ) weeks after the end of the therapy ).
Wilcoxon ’ s signed rank was used to calculate the differences of the outcome data . p-values ≤ 0.05 were considered statistically significant . SPSS for Windows ( version 11 , SPSS Inc ., Chicago , IL , USA ) statistical software was utilized .
RESULTS
Seven patients ( 4 males ) with dystrophic EB were included . The mean age at baseline was 19.8 years ( range 8 – 34 ). Nineteen oral lesions were treated ; the buccal mucosa was the most common site ( 37 %) ( Fig . 1 ), fol-
1 https :// www . medicaljournals . se / acta / content / abstract / 10.2340 / 00015555-2512
Fig . 1 . Oral lesions in dystrophic epidermolysis bullosa . Case 3 : A right buccal mucosal lesion at ( a ) T1 _ day1 , ( b ) T2 _ day2 , ( c ) T3 _ day3 , ( d ) T4 _ day7 , and ( e ) T7 _ 24 weeks after end of therapy .
This is an open access article under the CC BY-NC license . www . medicaljournals . se / acta Journal Compilation © 2017 Acta Dermato-Venereologica . doi : 10.2340 / 00015555-2512 Acta Derm Venereol 2017 ; 97 : 383 – 384