- Email: [email protected]
Fingertip necrosis as a sign of carpal tunnel syndrome
J AM ACAD DERMATOL VOLUME 68, NUMBER 2
the primary structure of TTR have been discovered and the majority linked with amyloidosis.4 Only 1 known variant (Val122del) has previously been reported in a Hispanic patient (Ecuadorian).4,5 Essentially all plasma TTR is synthesized in the liver. Liver transplantation currently offers the best outcome for long-term survival by stopping production of the abnormal protein.4 However, transplantation only halts progression and does not reverse the disease, underlining the need for timely diagnosis. This case demonstrates the importance of considering TA in patients with chronic polyneuropathy and of diagnostic approaches such as a simple random oral mucosa biopsy and TTR immunostaining in the early stages of the disease. Mhair S. Dekmezian,a Jaime A. Tschen, MD,b and Jeong Hee Cho-Vega, MD, PhDb Baylor College of Medicinea and St Joseph Dermatopathology,b Houston, Texas Funding sources: None. Conflicts of interest: None declared. Correspondence to: Jeong Hee Cho-Vega, MD, PhD, St Joseph Dermatopathology, 6909 Greenbrier St, Houston, TX 77030 E-mail: [email protected] REFERENCES 1. Plante-Bordeneuve V, Ferreira A, Lalu T, Zaros C, Lacroix C, Adams D, et al. Diagnostic pitfalls in sporadic transthyretin familial amyloid polyneuropathy (TTR-FAP). Neurology 2007;69:693-8. 2. Outteryck O, Stojkovic T, Launay D, Meignie-Ramon B, Vermersch P. Periorbital ecchymoses are not pathognomonic of the light-chain type of amyloidosis. Acta Derm Venereol 2007;87:544-5. 3. Robbins J. Thyroxine-binding proteins. Prog Clin Biol Res 1976;5:331-55. 4. Benson MD, Kincaid JC. The molecular biology and clinical features of amyloid neuropathy. Muscle Nerve 2007;36:411-23. 5. Uemichi T, Liepnieks JJ, Benson MD. A trinucleotide deletion in the transthyretin gene (delta V 122) in a kindred with familial amyloidotic polyneuropathy. Neurology 1997;48:1667-70. http://dx.doi.org/10.1016/j.jaad.2012.07.026
Fingertip necrosis as a sign of carpal tunnel syndrome To the Editor: Two women with bilateral, spontaneous finger ulceration presented to our dermatology clinic. The suspected referring diagnosis was sclerodactyly as a result of scleroderma. The clinical history revealed sensory and motor disturbances of the upper limbs. Neither patient had a previous diagnosis of neuropathy.
Letters e51
Fig 1. Carpal tunnel syndrome. Patient 2: hyperkeratosis, ulceration, and dystrophic changes on second and third fingers of left hand.
An 82-year-old retired housewife in otherwise good health had a 2-year history of ulceration and crusting on the tips of the index and middle fingers of both hands (more evident on the left side). Nail dystrophy and signs of cutaneous atrophy of the fingertips were also present. A complete blood cell count with differential, serum autoantibodies, and Doppler ultrasound imaging produced unremarkable findings. Hand radiography showed minimal osteoarthrosis of the interphalangeal joints without signs of acral osteolysis. Because the patient reported reduced sensation and decreased functional capacity of some of her fingers, we performed a Phalen test ( paresthesia and pain in the fingers by full flexion of the wrist for 60 seconds), which was strongly positive. This finding and a positive Tinel sign ( paresthesia and pain in the fingers induced by percussion over the volar surface of the wrist) led us to suspect carpal tunnel syndrome (CTS). This diagnosis was confirmed by electromyography, which revealed severe, bilateral median nerve damage with motor and sensory deficits. The patient underwent emergency decompression surgery. Five months later, the neuropathic symptoms had resolved and the ulcerations had healed completely with scar formation. A 70-year-old retired schoolteacher presented with a 4-month history of hyperkeratosis and ulcerative, dystrophic lesions on the second and third fingers of the left hand (Fig 1). The patient reported an increasingly diminished grasping ability associated with hand pain, most often at night. She had hypertension treated with angiotensin-converting enzyme inhibitors. A complete blood cell count with differential, serum autoantibodies, Doppler ultrasound imaging, and hand radiographs produced unremarkable findings. Tinel sign and Phalen test revealed positive results. Electromyography confirmed bilateral CTS. The motor deficit resolved after decompression surgery and the skin necrosis healed within 4 months. Typical symptoms of CTS include sensory disturbances (hypoesthesia and paresthesia) and motor
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FEBRUARY 2013
deficits in the median nerve area. The entrapment neuropathy can be primary, as in the case of our patients, or secondary to other causes such as connective tissue disease, in particular scleroderma.1 Skin lesions such as finger erythema, nail dystrophy, and blisters have been described in patients with severe damage to the motor, sensory, and autonomic fibers of the median nerve.1-3 The pathogenetic hypothesis is that fingertip necrosis is caused by a transitory alteration of autonomic innervation associated with impaired arterial vascularization as a result of a lesion in the vasa nervorum. A diminished pain threshold because of the sensory deficit may also contribute by allowing increased exposure to physical or thermal microtrauma.1,2,4 A rare ulcerative, mutilating variant of CTS associated with sclerodactyly and acro-osteolysis has been described.1-3,5 This radiographic sign was absent in our patients. These 2 cases highlight an advanced stage of CTS as a cause of fingertip necrosis. Federica Scarfı, MD, Meena Arunachalam, MD, Massimiliano Galeone, MD, Andrea Bassi, MD, and Elisa Difonzo, MD Department of Critical Care Medicine and Surgery, Division of Dermatology, University of Florence, Italy Funding sources: None. Conflicts of interest: None declared. Correspondence to: Federica Scarfi, MD, Department of Critical Care Medicine and Surgery, Division of Dermatology, University of Florence, Piazza dell’Indipendenza, 11, 50129 Florence, Italy E-mail: [email protected]
REFERENCES 1. Romanı J, Puig L, de Miguel G, de Moragas JM. Carpal tunnel syndrome presenting as sclerodactylia, nail dystrophy and acroosteolysis in a 60-year-old woman. Dermatology 1997;195:159-61. 2. Aratari E, Regesta G, Rebora A. Carpal tunnel syndrome appearing with prominent skin symptoms. Arch Dermatol 1984;120:517-9. 3. Natale M, Spennato P, Bocchetti A, Fratta M, Savarese L, Rotondo M. Ulcerative and mutilating variant of carpal tunnel syndrome. Acta Neurochir 2005;147:905-8. 4. L eger O, Lavall e F. Carpal tunnel syndrome revealed by digital ulcerations caused by arterial vasospasm. Chir Main 2005;24:39-41. 5. Tosti A, Morelli R, D’Alessandro R, Bassi F. Carpal tunnel syndrome presenting with ischemic skin lesions, acroosteolysis, and nail changes. J Am Acad Dermatol 1993;29:287-90. http://dx.doi.org/10.1016/j.jaad.2012.07.028
Agminated blue nevi in a patient with dermatomyositis To the Editor: Blue nevi most commonly present as solitary blue papules. Rarely, multiple blue nevi may occur in an individual as discrete and widely disseminated nevi, or may be grouped in a single ‘‘agminated’’ cluster.1 Because agminated blue nevi may be difficult to distinguish clinically from metastatic melanoma, a biopsy and histopathologic examination are necessary for the diagnosis.2 We report a case of a solitary blue nevus evolving into agminated blue nevi on the forearm of a patient with dermatomyositis. A 31-year-old Chinese woman with dermatomyositis controlled on prednisone 10 mg daily and azathioprine 50 mg daily, presented to us with numerous asymptomatic bluish pigmented lesions on her left forearm. The largest of the lesions had developed in her early teens but had remained relatively unchanged for years. In the last year, several smaller, pigmented lesions had appeared adjacent to the original lesion. The patient had been given the diagnosis of dermatomyositis at the age of 24 years; investigations at that time had revealed no evidence of an underlying malignancy. There was no family history of malignant melanoma. Clinical examination revealed a 5- 3 4-mm, wellcircumscribed, dome-shaped, blue-black papule on the back of the patient’s left forearm, and 5 less wellcircumscribed, blue-black macules, 1 to 2 mm in size, clustered around the primary lesion (Fig 1). An elliptical excision that included all pigmented lesions and a 2-mm margin of surrounding normalappearing skin was performed. The histopathologic examination revealed identical changes throughout the entire specimen. Foci of common blue nevi were seen microscopically (Fig 2). Based on the clinical and histopathological findings, the diagnosis of agminated blue nevi was made. Blue nevi are ectopic collections of dermal melanocytes that are thought to form when the melanocytes fail to complete their migration from the neural crest to the dermoepidermal junction.3 When numerous blue nevi present in a well-circumscribed area smaller than 10 cm, the term ‘‘agminated blue nevi’’ is used.4 These lesions may be congenital or acquired. The word ‘‘agminated’’ refers to being gathered in a group and is derived from the Latin word ‘‘agminis’’ meaning an army, column, or troop. Previous reports have referred to agminated blue nevi as plaque-type blue nevus5 or eruptive blue nevi.2 We agree with Velez et al4 that ‘‘agminated’’ is the preferred descriptive term. Although the pathogenesis of agminated blue nevi is unknown, a case has been reported in which a cluster of blue nevi appeared after a severe
the primary structure of TTR have been discovered and the majority linked with amyloidosis.4 Only 1 known variant (Val122del) has previously been reported in a Hispanic patient (Ecuadorian).4,5 Essentially all plasma TTR is synthesized in the liver. Liver transplantation currently offers the best outcome for long-term survival by stopping production of the abnormal protein.4 However, transplantation only halts progression and does not reverse the disease, underlining the need for timely diagnosis. This case demonstrates the importance of considering TA in patients with chronic polyneuropathy and of diagnostic approaches such as a simple random oral mucosa biopsy and TTR immunostaining in the early stages of the disease. Mhair S. Dekmezian,a Jaime A. Tschen, MD,b and Jeong Hee Cho-Vega, MD, PhDb Baylor College of Medicinea and St Joseph Dermatopathology,b Houston, Texas Funding sources: None. Conflicts of interest: None declared. Correspondence to: Jeong Hee Cho-Vega, MD, PhD, St Joseph Dermatopathology, 6909 Greenbrier St, Houston, TX 77030 E-mail: [email protected] REFERENCES 1. Plante-Bordeneuve V, Ferreira A, Lalu T, Zaros C, Lacroix C, Adams D, et al. Diagnostic pitfalls in sporadic transthyretin familial amyloid polyneuropathy (TTR-FAP). Neurology 2007;69:693-8. 2. Outteryck O, Stojkovic T, Launay D, Meignie-Ramon B, Vermersch P. Periorbital ecchymoses are not pathognomonic of the light-chain type of amyloidosis. Acta Derm Venereol 2007;87:544-5. 3. Robbins J. Thyroxine-binding proteins. Prog Clin Biol Res 1976;5:331-55. 4. Benson MD, Kincaid JC. The molecular biology and clinical features of amyloid neuropathy. Muscle Nerve 2007;36:411-23. 5. Uemichi T, Liepnieks JJ, Benson MD. A trinucleotide deletion in the transthyretin gene (delta V 122) in a kindred with familial amyloidotic polyneuropathy. Neurology 1997;48:1667-70. http://dx.doi.org/10.1016/j.jaad.2012.07.026
Fingertip necrosis as a sign of carpal tunnel syndrome To the Editor: Two women with bilateral, spontaneous finger ulceration presented to our dermatology clinic. The suspected referring diagnosis was sclerodactyly as a result of scleroderma. The clinical history revealed sensory and motor disturbances of the upper limbs. Neither patient had a previous diagnosis of neuropathy.
Letters e51
Fig 1. Carpal tunnel syndrome. Patient 2: hyperkeratosis, ulceration, and dystrophic changes on second and third fingers of left hand.
An 82-year-old retired housewife in otherwise good health had a 2-year history of ulceration and crusting on the tips of the index and middle fingers of both hands (more evident on the left side). Nail dystrophy and signs of cutaneous atrophy of the fingertips were also present. A complete blood cell count with differential, serum autoantibodies, and Doppler ultrasound imaging produced unremarkable findings. Hand radiography showed minimal osteoarthrosis of the interphalangeal joints without signs of acral osteolysis. Because the patient reported reduced sensation and decreased functional capacity of some of her fingers, we performed a Phalen test ( paresthesia and pain in the fingers by full flexion of the wrist for 60 seconds), which was strongly positive. This finding and a positive Tinel sign ( paresthesia and pain in the fingers induced by percussion over the volar surface of the wrist) led us to suspect carpal tunnel syndrome (CTS). This diagnosis was confirmed by electromyography, which revealed severe, bilateral median nerve damage with motor and sensory deficits. The patient underwent emergency decompression surgery. Five months later, the neuropathic symptoms had resolved and the ulcerations had healed completely with scar formation. A 70-year-old retired schoolteacher presented with a 4-month history of hyperkeratosis and ulcerative, dystrophic lesions on the second and third fingers of the left hand (Fig 1). The patient reported an increasingly diminished grasping ability associated with hand pain, most often at night. She had hypertension treated with angiotensin-converting enzyme inhibitors. A complete blood cell count with differential, serum autoantibodies, Doppler ultrasound imaging, and hand radiographs produced unremarkable findings. Tinel sign and Phalen test revealed positive results. Electromyography confirmed bilateral CTS. The motor deficit resolved after decompression surgery and the skin necrosis healed within 4 months. Typical symptoms of CTS include sensory disturbances (hypoesthesia and paresthesia) and motor
J AM ACAD DERMATOL
e52 Letters
FEBRUARY 2013
deficits in the median nerve area. The entrapment neuropathy can be primary, as in the case of our patients, or secondary to other causes such as connective tissue disease, in particular scleroderma.1 Skin lesions such as finger erythema, nail dystrophy, and blisters have been described in patients with severe damage to the motor, sensory, and autonomic fibers of the median nerve.1-3 The pathogenetic hypothesis is that fingertip necrosis is caused by a transitory alteration of autonomic innervation associated with impaired arterial vascularization as a result of a lesion in the vasa nervorum. A diminished pain threshold because of the sensory deficit may also contribute by allowing increased exposure to physical or thermal microtrauma.1,2,4 A rare ulcerative, mutilating variant of CTS associated with sclerodactyly and acro-osteolysis has been described.1-3,5 This radiographic sign was absent in our patients. These 2 cases highlight an advanced stage of CTS as a cause of fingertip necrosis. Federica Scarfı, MD, Meena Arunachalam, MD, Massimiliano Galeone, MD, Andrea Bassi, MD, and Elisa Difonzo, MD Department of Critical Care Medicine and Surgery, Division of Dermatology, University of Florence, Italy Funding sources: None. Conflicts of interest: None declared. Correspondence to: Federica Scarfi, MD, Department of Critical Care Medicine and Surgery, Division of Dermatology, University of Florence, Piazza dell’Indipendenza, 11, 50129 Florence, Italy E-mail: [email protected]
REFERENCES 1. Romanı J, Puig L, de Miguel G, de Moragas JM. Carpal tunnel syndrome presenting as sclerodactylia, nail dystrophy and acroosteolysis in a 60-year-old woman. Dermatology 1997;195:159-61. 2. Aratari E, Regesta G, Rebora A. Carpal tunnel syndrome appearing with prominent skin symptoms. Arch Dermatol 1984;120:517-9. 3. Natale M, Spennato P, Bocchetti A, Fratta M, Savarese L, Rotondo M. Ulcerative and mutilating variant of carpal tunnel syndrome. Acta Neurochir 2005;147:905-8. 4. L eger O, Lavall e F. Carpal tunnel syndrome revealed by digital ulcerations caused by arterial vasospasm. Chir Main 2005;24:39-41. 5. Tosti A, Morelli R, D’Alessandro R, Bassi F. Carpal tunnel syndrome presenting with ischemic skin lesions, acroosteolysis, and nail changes. J Am Acad Dermatol 1993;29:287-90. http://dx.doi.org/10.1016/j.jaad.2012.07.028
Agminated blue nevi in a patient with dermatomyositis To the Editor: Blue nevi most commonly present as solitary blue papules. Rarely, multiple blue nevi may occur in an individual as discrete and widely disseminated nevi, or may be grouped in a single ‘‘agminated’’ cluster.1 Because agminated blue nevi may be difficult to distinguish clinically from metastatic melanoma, a biopsy and histopathologic examination are necessary for the diagnosis.2 We report a case of a solitary blue nevus evolving into agminated blue nevi on the forearm of a patient with dermatomyositis. A 31-year-old Chinese woman with dermatomyositis controlled on prednisone 10 mg daily and azathioprine 50 mg daily, presented to us with numerous asymptomatic bluish pigmented lesions on her left forearm. The largest of the lesions had developed in her early teens but had remained relatively unchanged for years. In the last year, several smaller, pigmented lesions had appeared adjacent to the original lesion. The patient had been given the diagnosis of dermatomyositis at the age of 24 years; investigations at that time had revealed no evidence of an underlying malignancy. There was no family history of malignant melanoma. Clinical examination revealed a 5- 3 4-mm, wellcircumscribed, dome-shaped, blue-black papule on the back of the patient’s left forearm, and 5 less wellcircumscribed, blue-black macules, 1 to 2 mm in size, clustered around the primary lesion (Fig 1). An elliptical excision that included all pigmented lesions and a 2-mm margin of surrounding normalappearing skin was performed. The histopathologic examination revealed identical changes throughout the entire specimen. Foci of common blue nevi were seen microscopically (Fig 2). Based on the clinical and histopathological findings, the diagnosis of agminated blue nevi was made. Blue nevi are ectopic collections of dermal melanocytes that are thought to form when the melanocytes fail to complete their migration from the neural crest to the dermoepidermal junction.3 When numerous blue nevi present in a well-circumscribed area smaller than 10 cm, the term ‘‘agminated blue nevi’’ is used.4 These lesions may be congenital or acquired. The word ‘‘agminated’’ refers to being gathered in a group and is derived from the Latin word ‘‘agminis’’ meaning an army, column, or troop. Previous reports have referred to agminated blue nevi as plaque-type blue nevus5 or eruptive blue nevi.2 We agree with Velez et al4 that ‘‘agminated’’ is the preferred descriptive term. Although the pathogenesis of agminated blue nevi is unknown, a case has been reported in which a cluster of blue nevi appeared after a severe