A Simple Guide to Butterfly Skin Disease (Epidermolysis Bullosa), Diagnosis, Treatment and Related Conditions

By Kenneth Kee

This is one of the interesting disorders that I came across while reading about skin disorders.

This book describes Butterfly Skin Disease (Epidermolysis Bullosa), Diagnosis and Treatment and Related Diseases.

Epidermolysis bullosa is a rare chronic non-inflammatory skin disorder that is normally inherited.

It induces the skin to be incredibly thin and brittle.

People who are born with this disorder are often known as “butterfly children” since their diseased skin is as delicate as a butterfly’s wings.

Blistering of the skin often materializes in infancy in reaction to simply being held or handled.

Epidermolysis bullosa (EB) is a group of inherited diseases that are in which large blisters and erosion develop after the slightest trauma to the skin and mucous membranes.

These may arise anywhere on the body but most often appear at areas of friction and minor trauma such as the feet and hands.

In some types, blisters may also develop in internal organs, such as the esophagus, stomach and respiratory tract, without any obvious friction.

In severe forms of the disorder, epidermolysis bullosa can be life-threatening.

Most forms of epidermolysis bullosa are produced by a genetic mutation inherited from one or both parents that affects the production of the proteins in the skin.
Epidermolysis bullosa simplex 70%
Dystrophic epidermolysis bullosa 25%
Junctional Epidermolysis bullosa 5%
Kindler syndrome rare

There is another category of epidermolysis bullosa that is not produced by a genetic mutation.
Epidermolysis bullosa acquisita develops instead as an autoimmune disorder.

Fragile or butterfly skin that blisters and tears is frequent in all forms of epidermolysis bullosa
Pain and itching often appear with the blisters and tears.
Thick skin on the palms of the hands or soles of the feet
Rough or thick fingernails or toenails
Changes in skin color or tone
Scarring and tightening of the skin
Blisters inside the mouth and throat
Dental problems such as tooth decay
Difficulty swallowing (dysphagia)
Blisters around the eyes and nose

Normally epidermolysis bullosa blisters are seen at birth and during infancy.

Epidermolysis bullosa blisters may not appear until a toddler first starts to walk or when an older child starts new physical activities that trigger more friction on the soles of the feet

A visual inspection of the skin and presenting signs may also be sufficient to make a diagnosis.

In most cases a skin biopsy of a newly induced blister might be done to confirm the diagnosis.

Families with a history of epidermolysis bullosa may need prenatal genetic testing and genetic counseling

There is no cure for epidermolysis bullosa, no matter the type.

The treatment of butterfly skin disease is mainly preventive and supportive.
Wound care
Medicines to relieve pain and inflammation
Preventing new injuries
Avoiding complications
Rehabilitation therapy
Providing good nutrition

Surgeries that are occasionally used for this disorder are:
Widening the esophagus
Placing a feeding tube
Grafting skin
Restoring movement using surgical intervention to treat contractures
Surgical excision of squamous cell carcinoma

Possible future treatments

Doctors are studying new ways to treat epidermolysis bullosa:
Gene therapy, such as gel applied to wounds of people with dystrophic epidermolysis bullosa
Bone marrow (stem cell) transplantation
Protein replacement therapy

TABLE OF CONTENT
Introduction
Chapter 1 Butterfly Skin Disease (Epidermolysis Bullosa)
Chapter 2 Causes
Chapter 3 Symptoms
Chapter 4 Diagnosis
Chapter 5 Treatment
Chapter 6 Prognosis
Chapter 7 Skin Blisters
Chapter 8 Dermatitis Herpetiformis
Epilogue

• Language: English
• Publisher: Kenneth Kee
• Release date: Oct 17, 2022
• ISBN: 9781005696269

Kenneth Kee

Medical doctor since 1972.Started Kee Clinic in 1974 at 15 Holland Dr #03-102, relocated to 36 Holland Dr #01-10 in 2009.Did my M.Sc (Health Management ) in 1991 and Ph.D (Healthcare Administration) in 1993.Dr Kenneth Kee is still working as a family doctor at the age of 74However he has reduced his consultation hours to 3 hours in the morning and 2 hours inthe afternoon.He first started writing free blogs on medical disorders seen in the clinic in 2007 on http://kennethkee.blogspot.com.His purpose in writing these simple guides was for the health education of his patients which is also his dissertation for his Ph.D (Healthcare Administration). He then wrote an autobiography account of his journey as a medical student to family doctor on his other blog http://afamilydoctorstale.blogspot.comThis autobiography account “A Family Doctor’s Tale” was combined with his early “A Simple Guide to Medical Disorders” into a new Wordpress Blog “A Family Doctor’s Tale” on http://ken-med.com.From which many free articles from the blog was taken and put together into 1000 eBooks.He apologized for typos and spelling mistakes in his earlier books.He will endeavor to improve the writing in futures.Some people have complained that the simple guides are too simple.For their information they are made simple in order to educate the patients.The later books go into more details of medical disorders.He has published 1000 eBooks on various subjects on health, 1 autobiography of his medical journey, another on the autobiography of a Cancer survivor, 2 children stories and one how to study for his nephew and grand-daughter.The purpose of these simple guides is to educate patient on health disorders and not meant as textbooks.He does not do any night duty since 2000 ever since Dr Tan had his second stroke.His clinic is now relocated to the Buona Vista Community Centre.The 2 units of his original clinic are being demolished to make way for a new Shopping Mall.He is now doing some blogging and internet surfing (bulletin boards since the 1980's) startingwith the Apple computer and going to PC.The entire PC is upgraded by himself from XT to the present Pentium duo core.The present Intel i7 CPU is out of reach at the moment because the CPU is still expensive.He is also into DIY changing his own toilet cistern and other electric appliance.His hunger for knowledge has not abated and he is a lifelong learner.The children have all grown up and there are 2 grandchildren who are even more technically advanced than the grandfather where mobile phones are concerned.This book is taken from some of the many articles in his blog (now with 740 posts) A Family Doctor’s Tale.Dr Kee is the author of:"A Family Doctor's Tale""Life Lessons Learned From The Study And Practice Of Medicine""Case Notes From A Family Doctor"

Book preview

A

Simple

Guide

To

Butterfly Skin Disease

(Epidermolysis Bullosa),

Diagnosis,

Treatment

And

Related Conditions

By

Dr Kenneth Kee

M.B.,B.S. (Singapore)

Ph.D (Healthcare Administration)

Copyright Kenneth Kee 2022 Smashwords Edition

Published by Kenneth Kee at Smashwords.com

Dedication

This book is dedicated

To my wife Dorothy

And my children

Carolyn, Grace

And Kelvin

This book describes Butterfly Skin Disease (Epidermolysis Bullosa), Diagnosis and Treatment and Related Diseases which is seen in some of my patients in my Family Clinic.

(What You Need to Treat Butterfly Skin Disease)

This e-Book is licensed for your personal enjoyment only. This eBook may not be re-sold or given away to other people. If you would like to share this book with another person, please purchase an additional copy for each reader.

If you’re reading this book and did not purchase it, or it was not purchased for your use only, then please return to Smashwords.com and purchase your own copy.

Thank you for respecting the hard work of this author.

Introduction

I have been writing medical articles for my blog: http://kennethkee.blogspot.com (A Simple Guide to Medical Disorder) for the benefit of my patients since 2007.

My purpose in writing these simple guides was for the health education of my patients.

Health Education was also my dissertation for my Ph.D (Healthcare Administration).

I then wrote an autobiography account of my journey as a medical student to family doctor on my other blog: http://afamilydoctorstale.blogspot.com.

This autobiography account A Family Doctor’s Tale was combined with my early A Simple Guide to Medical Disorders into a new Wordpress Blog A Family Doctor’s Tale on http://kenkee481.wordpress.com.

From which many free articles from the blog was taken and put together into 800 eBooks.

Some people have complained that the simple guides are too simple.

For their information they are made simple in order to educate the patients.

The later books go into more details of medical disorders.

The first chapter is always from my earlier blogs which unfortunately tends to have typos and spelling mistakes.

Since 2013, I have tried to improve my spelling and writing.

As I tried to bring the patient the latest information about a disorder or illness by reading the latest journals both online and offline, I find that I am learning more and improving on my own medical knowledge in diagnosis and treatment for my patients.

My diagnosis and treatment capability has improved tremendously from my continued education.

Just by writing all these simple guides I find that I have learned a lot from your reviews (good or bad), criticism and advice.

I am sorry for the repetitions in these simple guides as the second chapters onwards have new information as compared to my first chapter taken from my blog.

I also find repetition definitely help me and maybe some readers to remember the facts in the books more easily.

I apologize if these repetitions are irritating to some readers.

Chapter 1

What is butterfly skin disease (epidermolysis bullosa)?

Epidermolysis bullosa is a rare chronic non-inflammatory skin disorder that is normally inherited.

It induces the skin to be incredibly thin and brittle.

People who are born with this disorder are often known as butterfly children since their diseased skin is as delicate as a butterfly’s wings.

Having to live with a disorder such as epidermolysis bullosa may be difficult and it starts at birth for most.

Blistering of the skin often materializes in infancy in reaction to simply being held or handled.

Epidermolysis bullosa (EB) is a group of inherited diseases that are in which large blisters and erosion develop after the slightest trauma to the skin and mucous membranes.

These may arise anywhere on the body but most often appear at areas of friction and minor trauma such as the feet and hands.

In some subtypes, blisters may also develop in internal organs, such as the esophagus, stomach and respiratory tract, without any obvious friction.

In severe forms of the disorder, epidermolysis bullosa can be life-threatening.

There is no cure for this disease but there are many methods to treat and prevent wounds and infection.

The success of the treatment is dependent on the severity of the disorder and how many areas of the body are affected.

Epidermolysis bullosa patients have been called butterfly skin children or crystal skin children.

Types of butterfly skin disease

There are many forms of butterfly skin disease.

They are classified by the underlying cause and the parts of the body that are affected.

Most forms of epidermolysis bullosa are produced by a genetic mutation inherited from one or both parents that affects the production of the proteins in the skin.

Forms of epidermolysis bullosa that have a genetic cause are separated into 4 categories:

1. Epidermolysis bullosa simplex

2. Junctional Epidermolysis bullosa

3. Dystrophic epidermolysis bullosa

4. Kindler syndrome

There is another category of epidermolysis bullosa that is not produced by a genetic mutation.

5. Epidermolysis bullosa acquisita develops instead as an autoimmune disorder.

Simple blister epidermolysis or Epidermolysis bullosa simplex

This is the most frequent form of epidermolysis with 70% of people having this type but it is still very infrequent, involving between 1 in 30,000 to 1 in 50,000 people.

It normally starts at birth or in early childhood but can become worse in the adult years, too.