Henri- Francois Secretan (1856-1916) and his syndrome

Pregledni rad Acta med-hist Adriat 2011;9(2);237-242 Review Article UDK: 61-05 Secretan, H-F. 616.5-005.98 HENRI-FRANÇOIS SECRETAN (1856–1916) AND HIS SYNDROME HENRI-FRANÇOIS SECRETAN (1856.–1916.) I NJEGOV SINDROM Khalid Al Aboud*, Daifullah Al Aboud** SUMMARY Henri-François Secretan (1856-1916) was a Swiss physician, who in 1901described a medical condition characterized by a hard, sometimes cyanotic oedema (Charcot’s blue oedema) on the back of one or both hands and forearms. This condition was later known as Secretan’s disease or Secretan’s syndrome. This report discusses Henri Secretan and the syndrome that bears his name. Key words: Factitious lymphedema, skin, Secretan disease Henri-François Secretan was a Swiss physician [1,2] credited for describing a rare syndrome, characterised by a hard oedema most commonly on the dorsum of the hand, that became known as Secretan’s disease or Secretan’s syndrome [3-20]. SECRETAN’S SYNDROME Secretan’s syndrome is a hyperplastic, recurring hard oedema of the hand or foot? dorsum. Its aetiology, pathology, and treatment are unknown [18]. * ** Dermatology Department, King Faisal Hospital, Makkah, Saudi Arabia. Dermatology Department, Taif University, Taif, Saudi Arabia. Address for correspondence: Khalid Al Aboud , MD, P.O Box 5440, Makkah 21955, Saudi Arabia. E-mail [email protected] 237 The disease can be caused by a hemostatic ligature or tight bandaging of the forearms or by repeated self-inflicted trauma with a hard object [6]. The syndrome has also been described on the upper and lower extremities. [19]. Secretan’s syndrome is also known as peritendinous fibrosis, posttraumatic hard oedema, and factitious lymphedema [12]. Typically it presents itself with an oedema on the dorsal surface of the hand and wrist, while the thumb is spared. The oedema is likely to be lymphatic and may be associated with pain and limited flexion of the metacarpophalangeal joints [6]. Secretan’s syndrome is an injury that is self-inflicted for secondary gain or is a conversion reaction [12]. Oedema caused by a hemostatic ligature presents itself with a clear-cut margin. In the acute stage, soft-tissue swells and can be treated with casting and psychiatric care. The chronic, hyperplastic stage occurs after repeated trauma and can lead to organizing hematoma and fibrosis of the subcutaneous and peritendonal areas. If the diagnosis is made before the progression to significant fibrosis, patients respond to conservative care with psychiatric and surgical intervention [9]. Patients with extensive limb involvement might have factitious traumatic panniculitis (FTP) [21]. In such patients, self-infliction may be less apparent. Patients with FTP can be diagnosed as having Weber-Christian disease, pancreatic fat necrosis, or superficial thrombophlebitis. The microscopic organising hematoma can be associated with amorphous polysaccharide masses that mix with iron pigment [21]. Some report similarity between Secretan’s disease and the Munchausen syndrome, as either patients are “sad and unhappy” [9]. It has been suggested that Secretan’s disease is an example of a hypersympathetic process that differs from causalgia, because there is no pain on examination and the X-rays show no osteopenia. Other authors believe that Secretan’s syndrome is a variant of Secretan’s disease consistent with posttraumatic sympathetic dystrophy [9]. Several orthopaedic and dermatological reports have recognised it as an often self-induced disease and a spontaneous, work-related hazard [9] Secretan’s disease can cause extensive disability of the hand, that can last for more than 6 months and often for years [10]. There are many theories about the aetiology of the disease, including a trauma that leads to peritendinous fibrosis, a hypersympathetic stimulus after an injury, and self-inflicted trauma [12]. 238 Dr. Henri-François Secretan (1856–1916) (http://www.secretan.info/) Secretan’s disease must be distinguished from occupational injuries, such as those seen in fishermen, that are caused by sea urchin spines and tight cuffs of wet suits. It must also be distinguished from other types of acute and chronic oedema, such as lymphatic aplasia, recurrent erysipelas, deep thrombophlebitis, angioedema, filariasis, venous obstruction, postsurgical disturbances, and carcinoma [6]. The diagnosis should be made based on a complete history, focusing on any reports that suggest selfinduced repetitive contusions [9]. A psychosocial inquiry must be completed to detect secondary gains or underlying psychiatric issues [9]. Magnetic resonance imaging can reveal a tendon oedema in combination with diffuse peritendonous fibrosis, extending to the fascia of the dorsal interosseous muscles [18]. The syndrome is best treated with conservative care and psychiatric counselling [9,12]. Splinting and active exercise constitute the best initial treatment. Only after months of such conservative management should one resort to surgery [8]. It has been suggested that patients with this syndrome suffer from varying forms of emotional disturbances and require a non-confrontational approach with appropriate psychological support [9]. Some groups recommend early surgical intervention as the preferred modality to mini- 239 mize disability, while others believe that surgical interventions can result in continuation of the syndrome and progression to disability [9]. HENRI-FRANÇOIS SECRETAN (1856-1916) Henri-François Secretan was born in Neuchâtel, Switzerland on 22 February 1856 [1]. His father, Charles, was a respected philosopher [1]. At the age of ten, his family moved to Lausanne. Secretan was a student at Collège Galliard and took up the study of literature. Later he studied law and medicine at Geneva, Pisa, and Paris universities, where he took his medical degree [2]. He travelled to Italy, France, Spain, Germany, Austria, England, and Algeria and settled down in Lausanne in 1885 to practice medicine. As a doctor for insurance companies, he saw patients daily—workers who were victims of workplace accidents [2]. Secretan was active in a new field of medicine, because accident insurance had just been established in Switzerand. He devoted himself to treating patients who suffered from work-related injuries, establishing a polyclinic for accident victims [1]. He preferred conservative management and opposed surgery, opining that “nature is the best doctor’’ [1]. Secretan’s prescribed treatments were of a soothing nature. He promoted cleanliness, recommending frequent warm and cold water baths. Secretan bandaged wounds with clean, boiled cloth and was suspicious of irritating ointments and foul-smelling powders that were popular at the time1. He read Latin and Greek literature and spoke several languages, including Italian - because many of his patients were Italians who worked in Switzerland [1] - and Spanish [2]. He became a specialist in medical accidents and wrote several books, including Society and Morality in 1897 and L’assurance contre les accidents, observations chirurgicales et professionnelles (Insurance against Accidents: Surgical and Professional Notes) , published in 1906 in the three national languages of Switzerland and printed in thousands of copies [2]. In this book, he described his experiences with and opinions on work-related injuries such as back injuries and hernias [1]. The book contains his medical articles, including “Oedème dur et hyperplasie traumatique du métacarpe dorsal’’ (“Hard oedema and traumatic hyperplasia of the dorsal metacarpus”)12. This article was originally 240 published in the June 1901 issue of Revue Medicale de la Suisse Romande [1], in which he reported several cases of woody swelling of the dorsum of the hand that occurred at work and resolved on its own [9]. He commented that this condition was frequent; he witnessed as many as 12 cases a year. Secretan was adamant that those who developed the woody swelling on the back of their hands or, in one instance, on the dorsum of the foot, were not hysterical or clever malingerers. During his career, he had ample opportunity to observe workmen who took advantage of being insured1. He saw patients exacerbate their wounds by encircling their limbs with cords tightly, attempting to cause oedema of an extremity [1]. His precise observations led him to identify this type of a bruise, which has since been called Secretan’s disease. Secretan’s writings were not limited to medicine [1]; he also published works of a more general nature: Les conditions de la science (The State of Science, 1892), La Société et la Morale (Society and Morality, 1897), La population et les moeurs (Population and Customs, 1915), and La propagande chrétienne et les persécutions (Christian Propaganda and Persecution, 1915) [2]. Henri-François Secretan died on March 5, 1916 after several months of illness. REFERENCES 1. Romm S. Henri-Francois Secretan. Hand Clin. 1986 Aug; 2(3):453-6. 2. Secretan B.Secretan, Histoire d’une famille lausannoise de 1400 nos jours, published by the Editions du Val de Faye in Lausanne, 2003., pp. 101-2. Availabale at ; http://www.secretan.info/ 3. Thiers H, Fayolle J, Roullet J, Berger C. [Apropos of a case of Secretan’s disease]. Bull Soc Fr Dermatol Syphiligr. 1965 May-Jun; 72(3):294-6. 4. Angelini G, Meneghini CL, Vena GA. Secretan’s syndrome: an artefact oedema of the hand. Contact Dermatitis. 1982 Sep; 8(5):345-6. 5. Jo/ rgensen J, Gammeltoft M, Schmidt H. Factitious lymphoedema, Secretan’s syndrome. Acta Derm Venereol. 1983; 63(3):271-3. 6. Angelini G. Occupational dermatitis artefacta. In: Kanerva L, Elsner P, Wahlberg JE, Maibach HI, ed. Handbook of occupational dermatology. Heidelberg: Springer-Verlag; 2000., pp. 141-8. 7. Bureau H, Decaillet JM, Magalon G. [Does Secretan‘s syndrome exist?]. Ann Chir. 1978 Nov; 32(9):571-4. 241 8. Saferin EH, Posch JL. Secretan‘s disease: Post-traumatic hard edema of the dorsum of the hand. Plast Reconstr Surg. 1976 Dec; 58(6):703-7. 9. Ramos JA, Bush D, Harrington TM. Secretan‘s syndrome. Orthopedics. 2007 Mar; 30(3):239-40. 10. Bureau H, Decaillet JM, Magalon G. [Secretan‘s disease (author‘s transl)]. Sem Hop. 1979 Mar 8-15; 55(9-10):449-52. 11. Reading G. Secretan‘s syndrome: hard edema of the dorsum of the hand. Plast Reconstr Surg. 1980 Feb; 65(2):182-7. 12. Moretta DN, Cooley RD Jr. Secrétan‘s disease: a unique case report and literature review. Am J Orthop (Belle Mead NJ). 2002 Sep;31(9):524-7. 13. Thiers H, Fayolle J, Roullet J, Berger C. [Apropos of a case of Secretan‘s disease]. Lyon Med. 1965 Nov 14;214(46):1030-4. 14. Fleming JP. Secretan‘s disease. Can J Surg. 1966 Jan;9(1):78-80. 15. Grobmyer AJ 3rd, Bruner JM, Dragstedt LR 2nd. Closed lymphangioplasty in Secretan‘s disease. Arch Surg. 1968 Jul;97(1):81-3. 16. Fleming JP. Secretan‘s disease. Plast Reconstr Surg. 1977 Oct;60(4):617-8. 17. Van Der Elst E. [Dorsal edema of the hand, so-called Secretan‘s edema]. Z Unfallmed Berufskr. 1960 Jun 15;53:112-21. 18. Whitney TM, Jones NF. Magnetic resonance imaging findings in Secretan‘s disease. J Hand Surg Am. 1995 May; 20(3):464-6. 19. Abnousi F, Chou LB. Secretan‘s disease of the foot: a case report and review. Foot Ankle Int. 2008 Feb; 29(2):248-50. 20. Verdan C. [So-called Secrétan hard edema of the back of the hand]. Helv Chir Acta. 1987 Jun;54(1-2):187-92. 21. Winkelmann RK, Barker SM. Factitial traumatic panniculitis. J Am Acad Dermatol. 1985 Dec;13(6):988-94. SAŽETAK Henri-François Secretan (1856.–1916.) švicarski je liječnik koji je 1901. opisao bolest za koju je svojstven tvrdi, a katkad i cijanotični edem (tzv. Charcotov edem) na jednoj ili objema nadlanicma i podlakticma. Ovo je stanje psolije postalo poznato kao Secretanova bolest ili sindrom. U ovome se članku raspravlja o Henriju Secretanu i sindromu koji nosi njegovo ime. Ključne riječi: lažni limfedem, koža, Secretanova bolest Acknowledgments The authors wish to thank Bernard Secretan for providing assistance in preparing this manuscript. 242