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Vascular diseases are the most common cutaneous manifestations of reflex sympathetic dystrophy
1050 Brief reports
J AM ACAD DERMATOL JUNE 2001
Vascular diseases are the most common cutaneous manifestations of reflex sympathetic dystrophy S. Sundaram, MD, and G. F. Webster, MD, PhD Philadelphia, Pennsylvania Reflex sympathetic dystrophy (RSD) is a chronic pain syndrome with prominent cutaneous findings. Atrophy has been considered to be the most common manifestation of the disease. We catalogued the abnormal skin conditions in RSD by means of chart review. Vascular problems were most common, followed by inflammatory diseases, infections, and atrophic diseases. Atrophic disease accounts for a minority of the skin problems seen in RSD. Most cutaneous complaints were related to vascular disease, particularly edema. (J Am Acad Dermatol 2001;44:1050-1.)
R
eflex sympathetic dystrophy (RSD, chronic regional pain syndrome) is a progressive painful syndrome characterized by hyperactivity of the sympathetic nervous system occurring after trauma. The clinical course is commonly divided into 3 stages: first (acute or hyperemic), second (dystrophic or ischemic), and third (atrophic) stage. It is currently thought that nerve injury causes tissues to be more sensitive to adrenergic catecholamines rather than an actual increase in sympathetic efferent activity as has been assumed.1 We have previously described 2 small groups of patients with cutaneous manifestations of RSD in which edema, ulcerations, and blistering were prominent.2,3 The purpose of this article is to further categorize the skin manifestations of RSD and to determine the most common presentation of this disease.
METHODS We conducted a retrospective chart review of all patients with RSD seen at the Department of Dermatology at Jefferson Medical College between 1994 and 1998. Each patient met the established criteria for diagnosis of RSD and was referred to us by their neurologist. Criteria for diagnosis of RSD are a painful progressive neuropathy after trauma or neurologic disease consisting of spontaneous pain, hyperalgesia, motor impairments, swelling, and vasodilatation.4
From the Department of Dermatology, Jefferson Medical College. Reprint requests: Guy F. Webster, MD, PhD, Suite 500, 211 S 9th St, Philadelphia, PA 19107. Copyright © 2001 by the American Academy of Dermatology, Inc. 0190-9622/2001/$35.00 + 0 16/54/114299 doi:10.1067/mjd.2001.114299
RESULTS A total of 26 patients with RSD were included in this study. The duration of RSD in these patients varied from months to years. The skin lesions observed were divided into 4 categories: vascular, inflammatory, infectious, and atrophic. Results are shown in Table I. Vascular findings were most common, with edema, usually of the leg, found in 58% of patients. Erythema was seen in 54% of patients. Other vascular lesions observed were mottling (8%), telangiectasia (8%), and nevus arraneus (4%). The second most common group of findings was inflammatory. Dermatitis (35%) was most frequently observed in this group and included contact, seborrheic, atopic, and dyshydrotic dermatitis. Also included in this category were erythematous papules (23%), hyperpigmentation (8%), and discoid lupus (8%). Infectious diseases observed in the patients with RSD included folliculitis (12%), onychomycosis (8%), and paronychia (4%). Atrophic changes included ulceration (12%), bullae (12%), alopecia (8%), and poor wound healing (4%).
DISCUSSION In our experience, the skin lesions in patients with RSD cause far more distress than is commonly appreciated. Although usually thought of as an atrophic skin disease, the majority of complaints in this series of patients were vascular in origin. Chronic edema was most frequent and the most problematic because it led to dermatitis, localized infection, and ulceration of the skin. Recent studies into the physiology of RSD have documented early changes in blood flow in the evolution of the disease in which the microcirculation loses the spinally mediated vasoconstrictor response.5-9
Brief reports 1051
J AM ACAD DERMATOL VOLUME 44, NUMBER 6
It was surprising to find 2 of 26 patients with evidence of chronic cutaneous lupus. One of the patients had lupus before RSD. The other experienced discoid scalp lesions after the diagnosis of RSD. Although not a rare disease, its incidence is lower than the 1 in 12.5 seen in this series. Because previous series have failed to report the occurrence of lupus with RSD and there is no apparent pathogenetic link between the two diseases, it is likely that the finding is coincidental. Treatment of vascular skin problems seen in RSD is frustrating and often unsuccessful. Traditional methods of edema control usually involve mechanical compression, a treatment that is intolerable to most patients with RSD. The best therapy is always to control the RSD, if possible, with medication or sympathectomy, after which the cutaneous manifestations usually spontaneously clear.2,3 In patients whose neurologic disease is unresponsive, the treatment of chronic edema and ulceration centers on good wound care and compressive therapy. Because of the exquisite sensitivity of the skin of a patient with RSD, effective compression is often intolerable without substantial analgesia. REFERENCES 1. Janig W, McLachlan EM. The role of modifications in noradrenergic peripheral pathways after nerve lesions in the generation of pain. In: Fields HL, Liebeskind JC, editors. Progress in pain research and management. Vol 1. Seattle: IASP Press; 1994. p. 101-28. 2. Webster GF, Schwartzman RJ, Jacoby RA, Knobler RL, Uitto JJ. Reflex sympathetic dystrophy: occurrence of inflammatory skin lesions in patients with stages II and III disease. Arch Dermatol 1991;127:1541-4. 3. Webster GF, Iozzo RV, Schwartzman RJ, Tahmoush AJ, Knobler RL, Jacoby RA. Reflex sympathetic dystrophy: occurrence of chronic edema and nonimmune bullous skin lesions. J Am Acad Dermatol 1993;28:29-32. 4. Schwartzman RJ, McLellan TL. Reflex sympathetic dystrophy: a review. Arch Neurol 1987;44:555-61. 5. Ribbers GM, Oosterhuis WP, van Limbeek J, deMetz M. Reflex sympathetic dystrophy: is the immune system involved? Arch Phys Med Rehab 1998;79:1549-52.
Table I. Skin findings in 26 patients with reflex sympathetic dystrophy Skin findings
Vascular Edema Erythema Mottling Telangiectasia Nevus aranaeus Inflammatory Dermatitis Erythematous papules Hyperpigmentation Discoid lupus Infectious Folliculitis Onychomycosis Paronychia Atrophic Cutaneous atrophy Ulceration Bullae Alopecia Thin nails Poor wound healing
No. of patients
% of patients
15 14 2 2 1
58 54 8 8 4
9 6 2 2
35 23 8 8
3 2 1
12 8 4
6 3 3 2 2 1
23 12 12 8 8 4
6. Perl ER. Causalgia and reflex sympathetic dystrophy revisited. In: Boivie J, Hansson P, Lindblom U, editors. Touch, temperature, and pain in health and disease. Progress in pain research and management. Vol 3. Seattle: IASP Press; 1994. p. 231-48. 7. Wasner G, Heckmann K, Maier C, Baron R. Vascular abnormalities in acute reflex sympathetic dystrophy (CRPS I): complete inhibition of sympathetic nerve activity with recovery. Arch Neurol 1999;56:613-20. 8. Kurvers HA. Reflex sympathetic dystrophy: facts and hypotheses. Vasc Med 1998;3:207-14. 9. Schwartzman RJ. Explaining reflex sympathetic dystrophy. Arch Neurol 1999;56:521-2.
J AM ACAD DERMATOL JUNE 2001
Vascular diseases are the most common cutaneous manifestations of reflex sympathetic dystrophy S. Sundaram, MD, and G. F. Webster, MD, PhD Philadelphia, Pennsylvania Reflex sympathetic dystrophy (RSD) is a chronic pain syndrome with prominent cutaneous findings. Atrophy has been considered to be the most common manifestation of the disease. We catalogued the abnormal skin conditions in RSD by means of chart review. Vascular problems were most common, followed by inflammatory diseases, infections, and atrophic diseases. Atrophic disease accounts for a minority of the skin problems seen in RSD. Most cutaneous complaints were related to vascular disease, particularly edema. (J Am Acad Dermatol 2001;44:1050-1.)
R
eflex sympathetic dystrophy (RSD, chronic regional pain syndrome) is a progressive painful syndrome characterized by hyperactivity of the sympathetic nervous system occurring after trauma. The clinical course is commonly divided into 3 stages: first (acute or hyperemic), second (dystrophic or ischemic), and third (atrophic) stage. It is currently thought that nerve injury causes tissues to be more sensitive to adrenergic catecholamines rather than an actual increase in sympathetic efferent activity as has been assumed.1 We have previously described 2 small groups of patients with cutaneous manifestations of RSD in which edema, ulcerations, and blistering were prominent.2,3 The purpose of this article is to further categorize the skin manifestations of RSD and to determine the most common presentation of this disease.
METHODS We conducted a retrospective chart review of all patients with RSD seen at the Department of Dermatology at Jefferson Medical College between 1994 and 1998. Each patient met the established criteria for diagnosis of RSD and was referred to us by their neurologist. Criteria for diagnosis of RSD are a painful progressive neuropathy after trauma or neurologic disease consisting of spontaneous pain, hyperalgesia, motor impairments, swelling, and vasodilatation.4
From the Department of Dermatology, Jefferson Medical College. Reprint requests: Guy F. Webster, MD, PhD, Suite 500, 211 S 9th St, Philadelphia, PA 19107. Copyright © 2001 by the American Academy of Dermatology, Inc. 0190-9622/2001/$35.00 + 0 16/54/114299 doi:10.1067/mjd.2001.114299
RESULTS A total of 26 patients with RSD were included in this study. The duration of RSD in these patients varied from months to years. The skin lesions observed were divided into 4 categories: vascular, inflammatory, infectious, and atrophic. Results are shown in Table I. Vascular findings were most common, with edema, usually of the leg, found in 58% of patients. Erythema was seen in 54% of patients. Other vascular lesions observed were mottling (8%), telangiectasia (8%), and nevus arraneus (4%). The second most common group of findings was inflammatory. Dermatitis (35%) was most frequently observed in this group and included contact, seborrheic, atopic, and dyshydrotic dermatitis. Also included in this category were erythematous papules (23%), hyperpigmentation (8%), and discoid lupus (8%). Infectious diseases observed in the patients with RSD included folliculitis (12%), onychomycosis (8%), and paronychia (4%). Atrophic changes included ulceration (12%), bullae (12%), alopecia (8%), and poor wound healing (4%).
DISCUSSION In our experience, the skin lesions in patients with RSD cause far more distress than is commonly appreciated. Although usually thought of as an atrophic skin disease, the majority of complaints in this series of patients were vascular in origin. Chronic edema was most frequent and the most problematic because it led to dermatitis, localized infection, and ulceration of the skin. Recent studies into the physiology of RSD have documented early changes in blood flow in the evolution of the disease in which the microcirculation loses the spinally mediated vasoconstrictor response.5-9
Brief reports 1051
J AM ACAD DERMATOL VOLUME 44, NUMBER 6
It was surprising to find 2 of 26 patients with evidence of chronic cutaneous lupus. One of the patients had lupus before RSD. The other experienced discoid scalp lesions after the diagnosis of RSD. Although not a rare disease, its incidence is lower than the 1 in 12.5 seen in this series. Because previous series have failed to report the occurrence of lupus with RSD and there is no apparent pathogenetic link between the two diseases, it is likely that the finding is coincidental. Treatment of vascular skin problems seen in RSD is frustrating and often unsuccessful. Traditional methods of edema control usually involve mechanical compression, a treatment that is intolerable to most patients with RSD. The best therapy is always to control the RSD, if possible, with medication or sympathectomy, after which the cutaneous manifestations usually spontaneously clear.2,3 In patients whose neurologic disease is unresponsive, the treatment of chronic edema and ulceration centers on good wound care and compressive therapy. Because of the exquisite sensitivity of the skin of a patient with RSD, effective compression is often intolerable without substantial analgesia. REFERENCES 1. Janig W, McLachlan EM. The role of modifications in noradrenergic peripheral pathways after nerve lesions in the generation of pain. In: Fields HL, Liebeskind JC, editors. Progress in pain research and management. Vol 1. Seattle: IASP Press; 1994. p. 101-28. 2. Webster GF, Schwartzman RJ, Jacoby RA, Knobler RL, Uitto JJ. Reflex sympathetic dystrophy: occurrence of inflammatory skin lesions in patients with stages II and III disease. Arch Dermatol 1991;127:1541-4. 3. Webster GF, Iozzo RV, Schwartzman RJ, Tahmoush AJ, Knobler RL, Jacoby RA. Reflex sympathetic dystrophy: occurrence of chronic edema and nonimmune bullous skin lesions. J Am Acad Dermatol 1993;28:29-32. 4. Schwartzman RJ, McLellan TL. Reflex sympathetic dystrophy: a review. Arch Neurol 1987;44:555-61. 5. Ribbers GM, Oosterhuis WP, van Limbeek J, deMetz M. Reflex sympathetic dystrophy: is the immune system involved? Arch Phys Med Rehab 1998;79:1549-52.
Table I. Skin findings in 26 patients with reflex sympathetic dystrophy Skin findings
Vascular Edema Erythema Mottling Telangiectasia Nevus aranaeus Inflammatory Dermatitis Erythematous papules Hyperpigmentation Discoid lupus Infectious Folliculitis Onychomycosis Paronychia Atrophic Cutaneous atrophy Ulceration Bullae Alopecia Thin nails Poor wound healing
No. of patients
% of patients
15 14 2 2 1
58 54 8 8 4
9 6 2 2
35 23 8 8
3 2 1
12 8 4
6 3 3 2 2 1
23 12 12 8 8 4
6. Perl ER. Causalgia and reflex sympathetic dystrophy revisited. In: Boivie J, Hansson P, Lindblom U, editors. Touch, temperature, and pain in health and disease. Progress in pain research and management. Vol 3. Seattle: IASP Press; 1994. p. 231-48. 7. Wasner G, Heckmann K, Maier C, Baron R. Vascular abnormalities in acute reflex sympathetic dystrophy (CRPS I): complete inhibition of sympathetic nerve activity with recovery. Arch Neurol 1999;56:613-20. 8. Kurvers HA. Reflex sympathetic dystrophy: facts and hypotheses. Vasc Med 1998;3:207-14. 9. Schwartzman RJ. Explaining reflex sympathetic dystrophy. Arch Neurol 1999;56:521-2.