reflections on the life of the elephant man

1
P302 Sir Ernest Graham-Little: Politician and dermatologist Barry Monk, Bedford Hospital, Bedford, AB, United Kingdom Sir Ernest Graham-Little, MD, MP, was one of the outstanding British dermatologists of the first half of the 20th century. He managed to combine his distinguished dermatology career with serving for 26 years as an independent Member of Parliament in the British House of Commons. This poster was based on the winning essay in the 2005 Samuel Zakon Prize of the History of Dermatology Society, and was shown at the 2004 meeting of the British Association of Dermatology. Commercial support: None identified. P303 Reflections on the life of the Elephant Man Melissa Barkham, MBChB, University Hospital Birmingham, Birmingham, United Kingdom; Donna Thompson, MBBS, University Hospital of North Staffordshire, Stoke on Trent, United Kingdom; Andrew Smith, MD, University Hospital of North Staffordshire, Stoke on Trent, United Kingdom The tragic story of Joseph Merrick, otherwise known as the Elephant Man, is important because it illustrates some of the difficulties patients with cosmetic disfigurement and physical disability may face. Joseph was born on August 5, 1862, in Leicester, England. By the age of 2 he had begun to develop abnormalities which became progressively worse throughout his life. His mother died when he was 11 years old, and he was unwanted by his father. Unable to earn a living through physical work, he became a sideshow ‘‘freak.’’ Dr Frederick Treves described him in his memoirs; ‘‘the most striking feature about him was his enormous and misshapen head. From the brow there projected a huge bony mass like a loaf, while from the back of his head hung a bag of spongy, fungous looking skin the surface of which was comparable to brown cauliflower. The right arm was enormous and shapeless. The other arm was remarkable by contrast, a delicately-shaped limb with a beautiful hand which any woman might have envied.’’ It is now generally accepted that Joseph had Proteus syndrome. This is a rare, sporadic, hamartomatous syndrome named after the Greek god Proteus (the polymorphous). Features include limb asymmetry, plantar hyperplasia, vascular malformations, lipomas, connective tissue and verru- cous epidermal naevi, macrocephaly, cranial hyperostosis, and hypertrophy of long bones. A propensity to malignancy has also been reported and some patients have abnormalities of the PTEN tumor suppressor gene. Although more is now under- stood about this disease, an effective treatment for patients with Proteus syndrome is yet to be found. Joseph Merrick was of normal intellect and enjoyed reading. For much of his short life (he died at the age of 27) he was taunted and ridiculed because of his disease. Treves also wrote, ‘‘...[H]ere was a man in the heyday of youth so vilely deformed that everyone he met confronted him with a look of horror and disgust. He was moreover lame and could hardly make his utterances understood. It was not until I came to know that Merrick was highly intelligent, that he possessed and acute sensibility and a romantic imagination that I realized the overwhelming tragedy of his life.’’ Society has changed a lot since then, but patients with any form of skin disease may still suffer because of an ignorance and lack of tolerance from others, which can have profound psychological effects. It is important that we deal with these issues in a sensitive and compassionate manner. Commercial support: None identified. P304 Old Testament skin Charles Phillips, MD, Brody School of Medicine at East Carolina University, Greenville, NC, United States; Christopher Scott, MD, Brody School of Medicine at East Carolina University, Greenville, NC, United States The Bible is widely regarded as one of the most important works of literature. The Old Testament establishes the background for the 3 large religions: Judaism, Christianity, and Islam. The bible is collected into books that tell tales of the birth of the world and its subsequent destruction and repopulation. The characters in the bible are rich and colorful, good and evil, young and very old, but all of them human. One tangible aspect to their humanity, and one that can be appreciated by a dermatologist, is their skin. Though by no means a dermatology textbook, the characters in the bible often have a cutaneous disease, or their skin and appendages play an important role in the story being told. The intent of this poster is to review for the interested readers some of the stories and how the skin played a role. Where possible, a diagnosis or differential diagnosis will be proffered. In the book of Genesis, Esau is the first born of Isaac and Rebekah is born ‘‘all covered with hair,’’ suggesting a diagnosis of hypertrichosis lanuginosa congenita. Moses is called upon by God to lead His people out of Israel. As part of His effort to establish his identity to Moses, God has Moses stick his hand in his cloak and as he pulls it back out it has become depigmented (‘‘leprous’’ or ‘‘aswhite as snow’’). Moses has been chosen to rescue the Israelites from slavery in Egypt. A reluctant Pharaoh, his heart having been hardened, refuses to let them go. God then invokes a series of plagues on Pharaoh and the Egyptians to convince him to let the Israelites go. Several of the plagues involve obvious or likely skin problems. The third plague was a plague of gnats (or lice in some translations), which must have involved a significant number of bites or infestations. The fourth plague was one of flies, which again may have been likely to bite. The sixth plague was primarily cutaneous and involved all citizens of Egypt being covered with boils. The Book of Job recounts the story of a godly man who was tried by Satan. Initially, all of his wealth and most of his family were taken away. Satan is then allowed to strike his flesh. He suffers a mucocuta- neous disease that causes him to waste away. Despite his affliction, Job maintains his faith and later has his health, his status and a new family restored to him. Commercial support: None identified. P305 Moritz Kaposi—Beyond his sarcoma Periasamy Balasubramaniam, MBBS, MD, Birmingham Skin Centre, Birmingham, UK, United Kingdom; Manjeet Mehmi, MBChB, Birmingham Skin Centre, Birmingham, United Kingdom; Anthony Abdullah, MD, Birmingham Skin Centre, Birmingham, United Kingdom Kaposi sarcoma (KS) is a well-known angiosarcoma associated with immunosup- pression. Its association with HIV disease in particular, has become prominent in the last 2 decades. Moritz Kaposi gave an excellent description of this specific entity more than a century ago. Kaposi was an eminent physician and a dermatologist. He was born (1837-1902) in Kaposva ´r, Hungary. Because of his Catholic faith, possibly, Kaposi changed his surname of Khun to Kaposi in reference to his birth place. He studied medicine in Vienna, Austria and attained his medical degree in 1861. His thesis, Dermatologie und Syphilis (1866) made him popular. He became an assistant to the famous professor of dermatology, Ferdinand von Hebra in 1862. In 1872, he described Kaposi sarcoma for the first time. KS is a rare cancer of the skin, which he had discovered in 5 elderly male patients; he initially named it ‘‘idiopathic multiple pigmented sarcomas.’’ His description was remarkable. ‘‘The disease leads to death, and it does so within a short period of two to three years... The disease must, from our present experience, be considered from the onset not only as incurable but also as deadly.’’ After he was appointed as professor of dermatology in 1875, he completed the Textbook of Skin Diseases in 1878 together with his mentor von Hebra. Kaposi’s main textbook Pathology and Therapy of the Skin Diseases in Lectures for Practical Physicians and Students was published in 1880. It became one of the most significant books in the history of dermatology, and was translated into several languages. Kaposi was a prolific writer and had published more than 100 articles and books. He worked with special interest in syphilis. His other works include description of dermatitits herpetiformis (with von Hebra), Kaposi’s varicel- liform eruption, and lichen scrofulosorum. Kaposi recognised discoid lupus to be an exclusively cutaneous disease and used the term lupus erythematosus disseminatus to refer to the cases with systemic involvement. In spite of the very high frequency of tuberculosis in his time, Kaposi maintained that DLE had no relation to tuberculosis. It is inspirational for modern physicians to learn about his history and his contribution to dermatology. Commercial support: None identified. AB34 JAM ACAD DERMATOL FEBRUARY 2007

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Page 1: Reflections on the life of the Elephant Man

P302Sir Ernest Graham-Little: Politician and dermatologist

Barry Monk, Bedford Hospital, Bedford, AB, United Kingdom

Sir Ernest Graham-Little, MD, MP, was one of the outstanding British dermatologistsof the first half of the 20th century. He managed to combine his distinguisheddermatology career with serving for 26 years as an independent Member ofParliament in the British House of Commons. This poster was based on the winningessay in the 2005 Samuel Zakon Prize of the History of Dermatology Society, and wasshown at the 2004 meeting of the British Association of Dermatology.

Commercial support: None identified.

P304Old Testament skin

Charles Phillips, MD, Brody School of Medicine at East Carolina University,Greenville, NC, United States; Christopher Scott, MD, Brody School of Medicineat East Carolina University, Greenville, NC, United States

The Bible is widely regarded as one of the most important works of literature.The Old Testament establishes the background for the 3 large religions: Judaism,Christianity, and Islam. The bible is collected into books that tell tales of the birth ofthe world and its subsequent destruction and repopulation. The characters in thebible are rich and colorful, good and evil, young and very old, but all of them human.One tangible aspect to their humanity, and one that can be appreciated by adermatologist, is their skin. Though by no means a dermatology textbook, thecharacters in the bible often have a cutaneous disease, or their skin and appendagesplay an important role in the story being told. The intent of this poster is to reviewfor the interested readers some of the stories and how the skin played a role. Wherepossible, a diagnosis or differential diagnosis will be proffered. In the book ofGenesis, Esau is the first born of Isaac and Rebekah is born ‘‘all covered with hair,’’suggesting a diagnosis of hypertrichosis lanuginosa congenita. Moses is called uponby God to lead His people out of Israel. As part of His effort to establish his identity toMoses, God has Moses stick his hand in his cloak and as he pulls it back out it hasbecome depigmented (‘‘leprous’’ or ‘‘as white as snow’’). Moses has been chosen torescue the Israelites from slavery in Egypt. A reluctant Pharaoh, his heart havingbeen hardened, refuses to let them go. God then invokes a series of plagues onPharaoh and the Egyptians to convince him to let the Israelites go. Several of theplagues involve obvious or likely skin problems. The third plague was a plague ofgnats (or lice in some translations), which must have involved a significant numberof bites or infestations. The fourth plague was one of flies, which again may havebeen likely to bite. The sixth plague was primarily cutaneous and involved allcitizens of Egypt being covered with boils. The Book of Job recounts the story of agodly man who was tried by Satan. Initially, all of his wealth and most of his familywere taken away. Satan is then allowed to strike his flesh. He suffers a mucocuta-neous disease that causes him to waste away. Despite his affliction, Job maintains hisfaith and later has his health, his status and a new family restored to him.

Commercial support: None identified.

P305Moritz Kaposi—Beyond his sarcoma

Periasamy Balasubramaniam, MBBS, MD, Birmingham Skin Centre, Birmingham,UK, United Kingdom; Manjeet Mehmi, MBChB, Birmingham Skin Centre,Birmingham, United Kingdom; Anthony Abdullah, MD, Birmingham SkinCentre, Birmingham, United Kingdom

Kaposi sarcoma (KS) is a well-known angiosarcoma associated with immunosup-pression. Its association with HIV disease in particular, has become prominent in thelast 2 decades. Moritz Kaposi gave an excellent description of this specific entitymore than a century ago. Kaposi was an eminent physician and a dermatologist. Hewas born (1837-1902) in Kaposvar, Hungary. Because of his Catholic faith, possibly,Kaposi changed his surname of Khun to Kaposi in reference to his birth place. Hestudied medicine in Vienna, Austria and attained his medical degree in 1861. Histhesis, Dermatologie und Syphilis (1866) made him popular. He became anassistant to the famous professor of dermatology, Ferdinand von Hebra in 1862. In1872, he described Kaposi sarcoma for the first time. KS is a rare cancer of the skin,which he had discovered in 5 elderly male patients; he initially named it ‘‘idiopathicmultiple pigmented sarcomas.’’ His description was remarkable. ‘‘The disease leadsto death, and it does so within a short period of two to three years. . . The diseasemust, from our present experience, be considered from the onset not only asincurable but also as deadly.’’ After he was appointed as professor of dermatology in1875, he completed the Textbook of Skin Diseases in 1878 together with his mentorvon Hebra. Kaposi’s main textbook Pathology and Therapy of the Skin Diseases inLectures for Practical Physicians and Students was published in 1880. It becameone of the most significant books in the history of dermatology, and was translatedinto several languages. Kaposi was a prolific writer and had published more than 100articles and books. He worked with special interest in syphilis. His other worksinclude description of dermatitits herpetiformis (with von Hebra), Kaposi’s varicel-liform eruption, and lichen scrofulosorum. Kaposi recognised discoid lupus to be anexclusively cutaneous disease and used the term lupus erythematosus disseminatusto refer to the cases with systemic involvement. In spite of the very high frequencyof tuberculosis in his time, Kaposi maintained that DLE had no relation totuberculosis. It is inspirational for modern physicians to learn about his historyand his contribution to dermatology.

Commercial support: None identified.

P303Reflections on the life of the Elephant Man

Melissa Barkham, MBChB, University Hospital Birmingham, Birmingham, UnitedKingdom; Donna Thompson, MBBS, University Hospital of North Staffordshire,Stoke on Trent, United Kingdom; Andrew Smith, MD, University Hospital ofNorth Staffordshire, Stoke on Trent, United Kingdom

The tragic story of Joseph Merrick, otherwise known as the Elephant Man, isimportant because it illustrates some of the difficulties patients with cosmeticdisfigurement and physical disability may face. Joseph was born on August 5, 1862,in Leicester, England. By the age of 2 he had begun to develop abnormalities whichbecame progressively worse throughout his life. His mother died when he was 11years old, and he was unwanted by his father. Unable to earn a living throughphysical work, he became a sideshow ‘‘freak.’’ Dr Frederick Treves described him inhis memoirs; ‘‘the most striking feature about him was his enormous and misshapenhead. From the brow there projected a huge bony mass like a loaf, while from theback of his head hung a bag of spongy, fungous looking skin the surface of which wascomparable to brown cauliflower. The right arm was enormous and shapeless. Theother arm was remarkable by contrast, a delicately-shaped limb with a beautiful handwhich any woman might have envied.’’ It is now generally accepted that Joseph hadProteus syndrome. This is a rare, sporadic, hamartomatous syndrome named afterthe Greek god Proteus (the polymorphous). Features include limb asymmetry,plantar hyperplasia, vascular malformations, lipomas, connective tissue and verru-cous epidermal naevi, macrocephaly, cranial hyperostosis, and hypertrophy of longbones. A propensity to malignancy has also been reported and some patients haveabnormalities of the PTEN tumor suppressor gene. Although more is now under-stood about this disease, an effective treatment for patients with Proteus syndromeis yet to be found. Joseph Merrick was of normal intellect and enjoyed reading. Formuch of his short life (he died at the age of 27) he was taunted and ridiculed becauseof his disease. Treves also wrote, ‘‘. . .[H]ere was a man in the heyday of youth sovilely deformed that everyone he met confronted him with a look of horror anddisgust. He was moreover lame and could hardly make his utterances understood. Itwas not until I came to know that Merrick was highly intelligent, that he possessedand acute sensibility and a romantic imagination that I realized the overwhelmingtragedy of his life.’’ Society has changed a lot since then, but patients with any form ofskin disease may still suffer because of an ignorance and lack of tolerance fromothers, which can have profound psychological effects. It is important that we dealwith these issues in a sensitive and compassionate manner.

Commercial support: None identified.

AB34 J AM ACAD DERMATOL FEBRUARY 2007