- Email: [email protected]
“Puffy hand syndrome”
G Model
ARTICLE IN PRESS
BONSOI-4400; No. of Pages 3
Joint Bone Spine xxx (2016) xxx–xxx
Available online at
ScienceDirect www.sciencedirect.com
Case report
“Puffy hand syndrome” Mickaël Chouk a , Claire Vidon a , Elise Deveza b , Frank Verhoeven a , Fabien Pelletier b , Clément Prati a , Daniel Wendling a,∗ a b
Department of Rheumatology, CHRU Besanc¸on, boulevard Fleming, 25030 Besanc¸on, France Department of Dermatology, CHRU Besanc¸on, boulevard Fleming, 25030 Besanc¸on, France
a r t i c l e
i n f o
Article history: Accepted 9 April 2016 Available online xxx Keywords: Puffy hand Edema Hand Toxicomania
a b s t r a c t Intravenous drug addiction is responsible for many complications, especially cutaneous and infectious. There is a syndrome, rarely observed in rheumatology, resulting in “puffy hands”: the puffy hand syndrome. We report two cases of this condition from our rheumatologic consultation. Our two patients had intravenous drug addiction. They presented with an edema of the hands, bilateral, painless, no pitting, occurring in one of our patient during heroin intoxication, and in the other 2 years after stopping injections. In our two patients, additional investigations (biological, radiological, ultrasound) were unremarkable, which helped us, in the context, to put the diagnosis of puffy hand syndrome. The pathophysiology, still unclear, is based in part on a lymphatic toxicity of drugs and their excipients. There is no etiological treatment but elastic compression by night has improved edema of the hands in one of our patients. ´ e´ franc¸aise de rhumatologie. Published by Elsevier Masson SAS. All rights reserved. © 2016 Societ
1. Introduction
2. Case reports
Biological explorations showed no inflammation, no antinuclear antibodies; the dosage of immunoglobulins, complement and iron balance were normal. X-rays of the hands were normal. The musculoskeletal ultrasound showed no arthritis, no tenosynovitis but only subcutaneous edema (Fig. 2). In this context, the clinical and paraclinical presentation allowed us to retain the diagnosis of puffy hand syndrome. The patient reported an improvement of hand’s edema after using compression sleeves during 3 months.
2.1. Case number 1
2.2. Case number 2
A 40-year-old man was seen in rheumatology consultation for management of thoraco-lumbar osteoporotic vertebral fractures. This patient used corticosteroids (betamethasone) during 15 years, introduced by his family doctor for an edema of the two hands. This patient was a former drug addict. He used intravenous heroin during 6 years (from 17 to 23). He was a smoker too. Two years after stopping his injections, with substitution by methadone, he observed progressive development of a bilateral, painless, and no pitting edema (Fig. 1). The corticosteroid therapy was ineffective. This edema remained stable over time.
A 27-year-old woman was admitted in rheumatology consultation for bilateral hand edema. She was a former drug addict. She was a smoker and an alcoholic too. She used intravenous cocaine during 9 years (from 16 to 25) and then she stopped during 2 years. Unfortunately, she started again intravenous cocaine and nasal heroin during 6 months. This patient transiently took buprenorphine. After 5 years of toxicomania, still using cocaine, she developed a bilateral hand edema extending to the forearms, at first fluctuating and then permanent. This edema appeared during pregnancy. We observed also acrocyanosis of both hands, telangiectasia and palmar erythrosis. There was no Raynaud’s phenomenon. Biological investigations showed anti-nuclear antibodies at low titers (1/160) without specificity, no rheumatoid factor or
Swollen hands are not a rare event in rheumatology, but nevertheless may be caused by a lot of different mechanisms and etiologies. In this context, the “puffy hand” takes a special place. We report two cases of such a condition.
∗ Corresponding author. E-mail address: [email protected] (D. Wendling).
http://dx.doi.org/10.1016/j.jbspin.2016.05.001 ´ e´ franc¸aise de rhumatologie. Published by Elsevier Masson SAS. All rights reserved. 1297-319X/© 2016 Societ
Please cite this article in press as: http://dx.doi.org/10.1016/j.jbspin.2016.05.001
Chouk
M,
et
al.
“Puffy
hand
syndrome”.
Joint
Bone
Spine
(2016),
G Model BONSOI-4400; No. of Pages 3
ARTICLE IN PRESS M. Chouk et al. / Joint Bone Spine xxx (2016) xxx–xxx
2
3. Discussion
Fig. 1. Clinical presentation of puffy hands.
anti-citrullinated peptide antibodies, the complement and the protein electrophoresis were normal. Hand’s X-rays and musculoskeletal ultrasound were normal (no arthritis, no tenosynovitis). Ultrasound study of upper limbs vessels was normal too. In this context, the clinical and paraclinical presentation allowed us to put the diagnosis of puffy hand syndrome. Compression sleeves were prescribed to the patient, but we do not know if it improved edema.
Fig. 2. Ultrasonographic evaluation: absence of synovitis or tenosynovitis.
Please cite this article in press as: http://dx.doi.org/10.1016/j.jbspin.2016.05.001
Chouk
M,
et
The puffy hand syndrome was first described in 1965 by Abeles among inmates of a prison in New York [1]. The frequency of this syndrome is difficult to assess because of the small number of cases reported in the literature but is estimated between 7 and 16% of drug addicts [2]. The pathophysiology is not completely understood, but different mechanisms are involved [3]. It is an obstruction and chronic destruction of the lymphatic network especially by the toxicity of heroin or drainage of impurities (quinine, talc, flour. . .). Buprenorphine therapy may also be subject to misuse. This poorly soluble molecule can participate in the lymphatic destruction [4]. Venous disease was initially described [1] but refuted by Neviaser et al., suggesting this is rather a consequence of drug addiction that a real substratum of pathophysiology [5]. Poor asepsis during injection can also be responsible for infectious complications, destroying lymphatic network and venules with septic micro-thrombi [6]. The lymphatic network destruction has been demonstrated by lymphoscintigraphy in some cases [4–7], but this exam is not feasible in daily practice and is not essential for diagnosis. According to Andresz et al. [8], the main risk factors for developing puffy hand syndrome are female gender, injecting in the feet or hands especially subcutaneously, and the lack of tourniquet use. Clinically, puffy hand syndrome is characterized by an edema of the hands that can extend to the forearm with sometimes loss of visibility of tendons and veins on the dorsum of both hands, this may also affect the feet. This edema is not pitting and remains painless. It can be at first asymmetric (in this case more to the non-dominant member) and intermittent, becoming permanent and symmetrical over months. It can be associated with vascular acrosyndromes [3]. This syndrome can appear during (observation number 2) or after (case number 1) intravenous drug addiction [3]. Other complications of intravenous drug use should be removed before to put the diagnosis of puffy hand syndrome. In addition to dermatological etiologies, such as subcutaneous abscesses, pyo-myositis, necrotising fasciitis [9,10], and also vascular etiologies (hematomas, aneurysms and mycotic pseudoaneurysm, septic superficial or deep vein thrombosis, arteriovenous fistulas) [10], or purely musculoskeletal etiologies may be raised. These are arthritis (associated or not with infective endocarditis) and septic tenosynovitis. Finally, we should remember that this lesion may become infected secondarily [11]. In our two drug addicts, as in all patients with bilateral edema of hands, differential diagnosis can be discussed. We should rule out rheumatic diseases, inflammatory (RS3PE, rheumatoid arthritis, polymyalgia rheumatica), metabolic (gout, chondrocalcinosis), complex regional pain syndrome (espacially in the inflammatory phase), systemic diseases (scleroderma or CREST, Sjögren syndrome), angioneurotic edemas, infectious diseases (bacterial, mycobacterial, viral [hepatitis] or parasitic [filariasis]). Organ deficiencies can be suspected (heart failure, nephrotic syndrome, cirrhosis), especially in the forms affecting hands and feet. Finally arterial or vein diseases (superficial or deep vein thrombosis) or purely lymphatic (compression because neoplastic disease, sentinel node removal, congenital hypoplasia of the lymphatic network [Milroy’s disease, Meige’s disease] but predominantly in the lower limbs) and traumatics pathologies (metacarpal or carpal bones fractures) should be eliminated. There is no specific treatment for puffy hand syndrome, but an elastic compression can improve lymphedema. Prevention of skin infections and protection against cold (vascular acrosyndromes) are also important measures to be considered [4]. The puffy hand syndrome is a rare entity, usually observed by dermatologists, addiction specialists and vascular doctors. However, this condition can also be met in rheumatology, because of a al.
“Puffy
hand
syndrome”.
Joint
Bone
Spine
(2016),
G Model BONSOI-4400; No. of Pages 3
ARTICLE IN PRESS M. Chouk et al. / Joint Bone Spine xxx (2016) xxx–xxx
functional impairment of the hands or sometimes of an unexpected iatrogenic consequence, as in one of our cases. Disclosure of interest The authors declare that they have no competing interest. References [1] Abeles H. Puffy-hand sign of drug addiction. N Engl J Med 1965;273:1167. [2] Ryan JJ, Hoopes JE, Jabaley ME. Drug injection injuries of the hands and forearms in addicts. Plast Reconstr Surg 1974;53:445–51. [3] Arrault M, Gaouar F, Vignes S. [Puffy hand syndrome]. Rev Med Intern 2009;30:460–4. [4] Simonnet N, Marcantoni N, Simonnet L, et al. [Puffy hand in long-term intravenous drug users]. J Mal Vasc 2004;29:201–4.
Please cite this article in press as: http://dx.doi.org/10.1016/j.jbspin.2016.05.001
Chouk
M,
et
3
[5] Neviaser RJ, Butterfield WC, Wieche DR. The puffy hand of drug addiction: a study of the pathogenesis. J Bone Joint Surg Am 1972;54:629–33. [6] Delage M, Samimi M, Lebidre E, et al. [“Puffy hands” syndrome]. Presse Med 2009;38:153–5. [7] Messikh R, Pelletier F, Bizouard N, et al. [Puffy hand syndrome due to drug addiction. Chronic lymphoedema and long-term intravenous drug addiction]. Ann Dermatol Venereol 2009;136:756–8. [8] Andresz V, Marcantoni N, Binder F, et al. Puffy hand syndrome due to drug addiction: a case-control study of the pathogenesis. Addiction 2006;101:1347–51. [9] Del Giudice P. Cutaneous complications of intravenous drug abuse. Br J Dermatol 2004;150:1–10. [10] Theodorou SJ, Theodorou DJ, Resnick D. Imaging findings of complications affecting the upper extremity in intravenous drug users: featured cases. Emerg Radiol 2008;15:227–39. [11] Amode R, Bilan P, Sin C, et al. Puffy hand syndrome revealed by a severe staphylococcal skin infection. Case Rep Dermatol Med 2013;2013: 376060.
al.
“Puffy
hand
syndrome”.
Joint
Bone
Spine
(2016),
ARTICLE IN PRESS
BONSOI-4400; No. of Pages 3
Joint Bone Spine xxx (2016) xxx–xxx
Available online at
ScienceDirect www.sciencedirect.com
Case report
“Puffy hand syndrome” Mickaël Chouk a , Claire Vidon a , Elise Deveza b , Frank Verhoeven a , Fabien Pelletier b , Clément Prati a , Daniel Wendling a,∗ a b
Department of Rheumatology, CHRU Besanc¸on, boulevard Fleming, 25030 Besanc¸on, France Department of Dermatology, CHRU Besanc¸on, boulevard Fleming, 25030 Besanc¸on, France
a r t i c l e
i n f o
Article history: Accepted 9 April 2016 Available online xxx Keywords: Puffy hand Edema Hand Toxicomania
a b s t r a c t Intravenous drug addiction is responsible for many complications, especially cutaneous and infectious. There is a syndrome, rarely observed in rheumatology, resulting in “puffy hands”: the puffy hand syndrome. We report two cases of this condition from our rheumatologic consultation. Our two patients had intravenous drug addiction. They presented with an edema of the hands, bilateral, painless, no pitting, occurring in one of our patient during heroin intoxication, and in the other 2 years after stopping injections. In our two patients, additional investigations (biological, radiological, ultrasound) were unremarkable, which helped us, in the context, to put the diagnosis of puffy hand syndrome. The pathophysiology, still unclear, is based in part on a lymphatic toxicity of drugs and their excipients. There is no etiological treatment but elastic compression by night has improved edema of the hands in one of our patients. ´ e´ franc¸aise de rhumatologie. Published by Elsevier Masson SAS. All rights reserved. © 2016 Societ
1. Introduction
2. Case reports
Biological explorations showed no inflammation, no antinuclear antibodies; the dosage of immunoglobulins, complement and iron balance were normal. X-rays of the hands were normal. The musculoskeletal ultrasound showed no arthritis, no tenosynovitis but only subcutaneous edema (Fig. 2). In this context, the clinical and paraclinical presentation allowed us to retain the diagnosis of puffy hand syndrome. The patient reported an improvement of hand’s edema after using compression sleeves during 3 months.
2.1. Case number 1
2.2. Case number 2
A 40-year-old man was seen in rheumatology consultation for management of thoraco-lumbar osteoporotic vertebral fractures. This patient used corticosteroids (betamethasone) during 15 years, introduced by his family doctor for an edema of the two hands. This patient was a former drug addict. He used intravenous heroin during 6 years (from 17 to 23). He was a smoker too. Two years after stopping his injections, with substitution by methadone, he observed progressive development of a bilateral, painless, and no pitting edema (Fig. 1). The corticosteroid therapy was ineffective. This edema remained stable over time.
A 27-year-old woman was admitted in rheumatology consultation for bilateral hand edema. She was a former drug addict. She was a smoker and an alcoholic too. She used intravenous cocaine during 9 years (from 16 to 25) and then she stopped during 2 years. Unfortunately, she started again intravenous cocaine and nasal heroin during 6 months. This patient transiently took buprenorphine. After 5 years of toxicomania, still using cocaine, she developed a bilateral hand edema extending to the forearms, at first fluctuating and then permanent. This edema appeared during pregnancy. We observed also acrocyanosis of both hands, telangiectasia and palmar erythrosis. There was no Raynaud’s phenomenon. Biological investigations showed anti-nuclear antibodies at low titers (1/160) without specificity, no rheumatoid factor or
Swollen hands are not a rare event in rheumatology, but nevertheless may be caused by a lot of different mechanisms and etiologies. In this context, the “puffy hand” takes a special place. We report two cases of such a condition.
∗ Corresponding author. E-mail address: [email protected] (D. Wendling).
http://dx.doi.org/10.1016/j.jbspin.2016.05.001 ´ e´ franc¸aise de rhumatologie. Published by Elsevier Masson SAS. All rights reserved. 1297-319X/© 2016 Societ
Please cite this article in press as: http://dx.doi.org/10.1016/j.jbspin.2016.05.001
Chouk
M,
et
al.
“Puffy
hand
syndrome”.
Joint
Bone
Spine
(2016),
G Model BONSOI-4400; No. of Pages 3
ARTICLE IN PRESS M. Chouk et al. / Joint Bone Spine xxx (2016) xxx–xxx
2
3. Discussion
Fig. 1. Clinical presentation of puffy hands.
anti-citrullinated peptide antibodies, the complement and the protein electrophoresis were normal. Hand’s X-rays and musculoskeletal ultrasound were normal (no arthritis, no tenosynovitis). Ultrasound study of upper limbs vessels was normal too. In this context, the clinical and paraclinical presentation allowed us to put the diagnosis of puffy hand syndrome. Compression sleeves were prescribed to the patient, but we do not know if it improved edema.
Fig. 2. Ultrasonographic evaluation: absence of synovitis or tenosynovitis.
Please cite this article in press as: http://dx.doi.org/10.1016/j.jbspin.2016.05.001
Chouk
M,
et
The puffy hand syndrome was first described in 1965 by Abeles among inmates of a prison in New York [1]. The frequency of this syndrome is difficult to assess because of the small number of cases reported in the literature but is estimated between 7 and 16% of drug addicts [2]. The pathophysiology is not completely understood, but different mechanisms are involved [3]. It is an obstruction and chronic destruction of the lymphatic network especially by the toxicity of heroin or drainage of impurities (quinine, talc, flour. . .). Buprenorphine therapy may also be subject to misuse. This poorly soluble molecule can participate in the lymphatic destruction [4]. Venous disease was initially described [1] but refuted by Neviaser et al., suggesting this is rather a consequence of drug addiction that a real substratum of pathophysiology [5]. Poor asepsis during injection can also be responsible for infectious complications, destroying lymphatic network and venules with septic micro-thrombi [6]. The lymphatic network destruction has been demonstrated by lymphoscintigraphy in some cases [4–7], but this exam is not feasible in daily practice and is not essential for diagnosis. According to Andresz et al. [8], the main risk factors for developing puffy hand syndrome are female gender, injecting in the feet or hands especially subcutaneously, and the lack of tourniquet use. Clinically, puffy hand syndrome is characterized by an edema of the hands that can extend to the forearm with sometimes loss of visibility of tendons and veins on the dorsum of both hands, this may also affect the feet. This edema is not pitting and remains painless. It can be at first asymmetric (in this case more to the non-dominant member) and intermittent, becoming permanent and symmetrical over months. It can be associated with vascular acrosyndromes [3]. This syndrome can appear during (observation number 2) or after (case number 1) intravenous drug addiction [3]. Other complications of intravenous drug use should be removed before to put the diagnosis of puffy hand syndrome. In addition to dermatological etiologies, such as subcutaneous abscesses, pyo-myositis, necrotising fasciitis [9,10], and also vascular etiologies (hematomas, aneurysms and mycotic pseudoaneurysm, septic superficial or deep vein thrombosis, arteriovenous fistulas) [10], or purely musculoskeletal etiologies may be raised. These are arthritis (associated or not with infective endocarditis) and septic tenosynovitis. Finally, we should remember that this lesion may become infected secondarily [11]. In our two drug addicts, as in all patients with bilateral edema of hands, differential diagnosis can be discussed. We should rule out rheumatic diseases, inflammatory (RS3PE, rheumatoid arthritis, polymyalgia rheumatica), metabolic (gout, chondrocalcinosis), complex regional pain syndrome (espacially in the inflammatory phase), systemic diseases (scleroderma or CREST, Sjögren syndrome), angioneurotic edemas, infectious diseases (bacterial, mycobacterial, viral [hepatitis] or parasitic [filariasis]). Organ deficiencies can be suspected (heart failure, nephrotic syndrome, cirrhosis), especially in the forms affecting hands and feet. Finally arterial or vein diseases (superficial or deep vein thrombosis) or purely lymphatic (compression because neoplastic disease, sentinel node removal, congenital hypoplasia of the lymphatic network [Milroy’s disease, Meige’s disease] but predominantly in the lower limbs) and traumatics pathologies (metacarpal or carpal bones fractures) should be eliminated. There is no specific treatment for puffy hand syndrome, but an elastic compression can improve lymphedema. Prevention of skin infections and protection against cold (vascular acrosyndromes) are also important measures to be considered [4]. The puffy hand syndrome is a rare entity, usually observed by dermatologists, addiction specialists and vascular doctors. However, this condition can also be met in rheumatology, because of a al.
“Puffy
hand
syndrome”.
Joint
Bone
Spine
(2016),
G Model BONSOI-4400; No. of Pages 3
ARTICLE IN PRESS M. Chouk et al. / Joint Bone Spine xxx (2016) xxx–xxx
functional impairment of the hands or sometimes of an unexpected iatrogenic consequence, as in one of our cases. Disclosure of interest The authors declare that they have no competing interest. References [1] Abeles H. Puffy-hand sign of drug addiction. N Engl J Med 1965;273:1167. [2] Ryan JJ, Hoopes JE, Jabaley ME. Drug injection injuries of the hands and forearms in addicts. Plast Reconstr Surg 1974;53:445–51. [3] Arrault M, Gaouar F, Vignes S. [Puffy hand syndrome]. Rev Med Intern 2009;30:460–4. [4] Simonnet N, Marcantoni N, Simonnet L, et al. [Puffy hand in long-term intravenous drug users]. J Mal Vasc 2004;29:201–4.
Please cite this article in press as: http://dx.doi.org/10.1016/j.jbspin.2016.05.001
Chouk
M,
et
3
[5] Neviaser RJ, Butterfield WC, Wieche DR. The puffy hand of drug addiction: a study of the pathogenesis. J Bone Joint Surg Am 1972;54:629–33. [6] Delage M, Samimi M, Lebidre E, et al. [“Puffy hands” syndrome]. Presse Med 2009;38:153–5. [7] Messikh R, Pelletier F, Bizouard N, et al. [Puffy hand syndrome due to drug addiction. Chronic lymphoedema and long-term intravenous drug addiction]. Ann Dermatol Venereol 2009;136:756–8. [8] Andresz V, Marcantoni N, Binder F, et al. Puffy hand syndrome due to drug addiction: a case-control study of the pathogenesis. Addiction 2006;101:1347–51. [9] Del Giudice P. Cutaneous complications of intravenous drug abuse. Br J Dermatol 2004;150:1–10. [10] Theodorou SJ, Theodorou DJ, Resnick D. Imaging findings of complications affecting the upper extremity in intravenous drug users: featured cases. Emerg Radiol 2008;15:227–39. [11] Amode R, Bilan P, Sin C, et al. Puffy hand syndrome revealed by a severe staphylococcal skin infection. Case Rep Dermatol Med 2013;2013: 376060.
al.
“Puffy
hand
syndrome”.
Joint
Bone
Spine
(2016),