- Email: [email protected]
Extensive nodular sarcoidosis in the hand
EXTENSIVE
NODULAR
SARCOIDOSIS
IN THE HAND
B. KWON, R. R. BINDRA, P. LIAKOS and R. H. GELBERMAN
From the Department of Orthopaedic Surgery, Washington University School of Medicine, Saint Louis, Missouri, USA We report an atypical case of nodular sarcoidosis involving both hands. The pattern of extensive involvement of all digits with lesions extending into the pulp spaces has not been reported previously. The diagnosis of sarcoidosis should be considered even in patients presenting with clinically uncharacteristic manifestations.
Journal of Hand Surgery (British and European Volume, 1997) 22B." 5:676 678 Although involvement of the hands in sarcoidosis is rare, with a reported incidence of 0.2%, it may precede the onset of systemic disease (Gottlieb and Wenner, 1992; James et al, 1976; Landi et al, 1983). The presentation of sarcoidosis in the hand is non-specific, can be variable and presents with digital swelling, soft tissue masses or cutaneous lesions (James et al, 1976; Pierson and Willett, 1978; Pitt et al, 1983; Sartoris et al, 1985). Untreated sarcoidosis can lead to a number of complications, from mild dysfunction to permanent damage to the pulmonary, cardiac, and nervous systems (Crystal, 1994; Gottlieb and Wenner, 1992; Tanoue, 1996). Previous reports indicate that sarcoid involvement of the soft tissues in the hand commonly presents with painless digital swelling and variable destruction of the underlying bone (Curco et al, 1995; Elgart, 1986; Gottlieb and Wenner, 1992; Sartoris et al, 1985; Vainsencher and Winkleman, 1984). We report a case of extensive bilateral hand involvement with digital soft tissue nodules extending into the pulp spaces.
Sclerotic and lytic changes were confined to the phalanges of the right long finger (Fig 2). Bone scintigraphy with 99mTechnetium-MDP excluded osseous involvement elsewhere. Magnetic resonance imaging revealed multiple nodular areas in the subcutaneous and deep tissues with decreased signal intensity on both T f a n d T2-weighted images. Routine laboratory studies were unremarkable except for a slightly elevated uric acid level at 8.8 mg/dl (normal 3.0-8.0 mg/dl). An excisional biopsy of the mass in the pulp and the mass overlying the dorsum of the proximal interpha-
CASE REPORT
A 57-year-old factory worker presented to our emergency department with an incarcerated ring on his left little finger. He provided a history of progressive, painless swelling in all his digits of several years duration and which had recently become worse. He denied symptoms of systemic illness or history of chronic disease. However, a mediastinal biopsy had been performed at another hospital, 13 years before. Past records revealed that the patient had undergone a paratracheal lymph node biopsy which showed sarcoidosis, but he did not attend for treatment at that time. Physical examination revealed nodular masses extending from the level of the middle digital creases to the pulps of all digits bilaterally. There was a duskiness over the distal phalanges. The irregular soft tissue swellings were non-tender, firm, and had well-demarcated borders. They were around 3.0 cm in their largest diameters (Fig 1). Discrete nodules were noted also overlying the dorsal aspect of the proximal interphalangeal joints of the ring and long fingers of the left hand. The active range of digital motion was limited as the soft tissue swellings precluded making a fist. Chest X-rays were unremarkable except for surgical clips in the mediastinum. X-ray studies of both hands showed multiple, asymmetrical, nodular, soft-tissue densities. There was no calcification within the masses.
Fig 1
676
Clinical photograph of the left palm demonstrating multiple soft tissue masses.
NODULAR SARCO1DOSIS
Fig 2
Posteroanterior X-ray of the right hand demonstrating multiple soft tissue densities. Note sclerotic and lytic changes in the phalanges of the long finger (inset).
langeal joint of the right long finger was carried out. Intraoperatively, the masses appeared yellow-brown, solid and fibrous, intimately adherent to and poorly demarcated from the overlying skin and deeper soft tissues including the extensor tendon expansion. A debulking of the masses was performed preserving neurovascular structures and the extensor tendon. The operative wounds healed without incident. Histological examination of biopsy tissue revealed non-caseating granulomata surrounding deep dermal and subcutaneous tissue. There were no polarizable foreign bodies. PAS and acid-fast stains were negative, as were routine bacterial and fungal cultures. These findings, in conjunction with the prior biopsy report of sarcoid involvement in perihilar lymph nodes, confirmed the diagnosis of sarcoidosis. DISCUSSION Sarcoidosis is a systemic illness which typically presents
677
with involvement of perihilar lymph nodes, pulmonary parenchyma, skin and eye structures (Crystal, 1994; Elgart, 1986; Tanoue, 1996; Tanoue et al, 1993). Sarcoid nodules are a rare manifestation of the disease, involving the subcutaneous soft tissues in 2.2 to 6% of cases (Maycock et al, 1963; Vainsencher and Winkleman, 1984). Previous reports indicate that these nodules are most commonly located in the palm or in the digits at the level of the proximal and middle phalanges (Curco et al, 1995; Gottlieb and Wenner, 1992; Scott and Scott, 1984). Extensive nodular sarcoidosis involving multiple digits with lesions extending to the finger tips as seen in this patient has not been reported previously. In the clinical presentation of nodular swellings in the hands, other diagnoses should be considered. Although pigmented villonodular synovitis can present similarly with a firm and painless nodule, the multiplicity of digits involved in this case excluded this diagnosis (Jones et al, 1969; Moore et al, 1984). Neurofibromata presenting in multiple digits are a manifestation of von Recklinghausen's disease which can be diagnosed by its characteristic stigmata (Idler, 1995; Rinaldi, 1983). Tuberculosis usually presents with single digital involvement, without discrete nodular swellings and in the setting of systemic signs and symptoms (Robins, 1967). Although amyloidosis associated with chronic diseases can present with similar swellings, they are localized to flexor tendons and their sheaths (Hallett, 1982; Kurer et al, 1991). Once sarcoidosis has been confirmed, the degree of involvement of other organs, especially the lungs and eyes, should be sought. Pulmonary function studies and an ophthalmological examination are warranted as interstitial lung disease with progressive pulmonary fibrosis and impaired vision or blindness cause significant morbidity (Crystal, 1994; Tanoue, 1996). Subcutaneous sarcoid nodules rarely cause major problems unless organ dysfunction results from architectural distortion due to extensive granulomatous involvement (Gross et al, 1971). The overall prognosis of sarcoidosis is, however, good, with spontaneous remissions occurring in 50% of patients. Low dose, intermittent glucocorticoid therapy and rarely cytotoxic drug therapy are used in refractory cases (Crystal, 1994; Tanoue, 1996). Although sarcoidosis commonly presents with characteristic signs and symptoms, unusual manifestations should not preclude its diagnosis. Nodular sarcoidosis involving the hands is rare, but the diagnosis should be considered when presented with atypical soft-tissue swelling in the hands. REFERENCES Crystal RG. Sarcoidosis. In: Isselbacher KJ, Braunwald E, Wilson JD, Martin JB, Fauci AS, Kasper DL (Eds), Harrison's principles of internal medicine, 13th edn, New York, McGraw-Hill 1994:1679 1684. Curco N, Pagerols X, Vives P (1995). Subcutaneous sarcoidosis with dactylitis. Clinical and Experimental Dermatology, 20:434 435. Elgart ML (1986). Cutaneous sarcoidosis: definitions and types of lesions. Clinics in Dermatology, 4: 3541.
678 Gottlieb DC, Wenner SM (1992). Soft tissue sarcoidosis of the hand: a case report. Journal of H a n d Surgery, 17B: 23~231. Gross MD, Andriacchi F, Gordon R, Maddox D (1971). Nodular subcutaneous sarcoidosis. Archives of Dermatology, 113: 1442-I443. Hallett J (1982). Tendon tethering in the carpal tunnel due to amyloidosis in Bence-Jones myelomatosis. Journal of Bone and Joint Surgery, 64B: 357-360. Idler RS (I 995). Benign and malignant nerve tumors. Hand Clinics, 11: 203~09. James DG, Neville E, Castairs LS (1976). Bone and joint sarcoidosis. Seminars in Arthritis and Rheumatism, 6: 53-81. Jones FE, Soule EH, Coventry MB (1969). Fibrous xanthoma of synovium (giant-cell tumor of tendon sheath, pigmented nodular synovitis). Journal of Bone and Joint Surgery, 51A: 76 86. Kurer MHJ, Baillod RA, Mad gwick JCA (1991 ). Musculoskeletal manifestations of amyloidosis. Journal of Bone and Joint Surgery, 73B: 271 276. Landi A, Brooks D, De Santis G (1983). Sarcoidosis of the hand - - report of two cases. Journal of H a n d Surgery, 8:197 200. Maycock RL, Bertrand P, Morrison CC, Scott J H (1963). Manifestations of sarcoidosis. American Journal of Medicine, 35: 67-89. Moore JR, Weiland A J, Curtis R M (1984). Localized nodular tenosynovitis: Experience with 115 cases. Journal of H a n d Surgery, 9A: 4 1 2 4 17. Pierson D J, Willett ES (1978). Sarcoidosis presenting with finger pain. Journal of the American Medical Association, 239: 2023~024.
T H E J O U R N A L OF H A N D SURGERY VOL. 22B No. 5 O C T O B E R 1997 Pitt P, Hamilton EB, Innes EH, Morley KD, Monk BE, Hughes G R (1983). Sarcoid dactylitis. Annals of the Rheumatic Diseases, 42:634 639. Rinaldi E (1983). Neurilemomas and neurofibromas of the upper limb. Journal of H a n d Surgery, 8: 590-593. Robins R H C (1967). Tuberculosis of the wrist and hand. British Journal of Surgery, 54:211-218. Sartoris DJ, Resnick D, Resnick C, Yaghmai I (1985). Musculoskeletal manifestations of sarcoidosis. Seminars in Roentgenology, 20:375 386. Scott TH, Scott M A (1984). Sarcoidosis with nodular lesions of the palm and sole. Archives of Dermatology, 120: 1239-1240. Tanoue LT (1996). Systemic sarcoidosis. Resident and Staff Physician; 42: 39~47. Tanoue LT, Zitnik R, Elias JA. Systemic sarcoidosis. In: Baum G L, Wolinsky E (Eds), Textbook ~?fpulrnonary diseases, 5th edn, Boston, Little, Brown, 1993:745 774. Vainsencher D, Winkehnann R K (1984). Subcutaneous sarcoidosis. Archives of Dermatology 120:1028 1031.
Received: 7 November 1996 Accepted after revision: 17 February 1997 Dr R. H. Gelberman, Department of Orthopaedic Surgery,Washington University School of Medicine, One Barnes Hospital Plaza, Suite 11300,Saint Louis, Missouri 63110, USA. © 1997The British Society for Surgery of the Hand
NODULAR
SARCOIDOSIS
IN THE HAND
B. KWON, R. R. BINDRA, P. LIAKOS and R. H. GELBERMAN
From the Department of Orthopaedic Surgery, Washington University School of Medicine, Saint Louis, Missouri, USA We report an atypical case of nodular sarcoidosis involving both hands. The pattern of extensive involvement of all digits with lesions extending into the pulp spaces has not been reported previously. The diagnosis of sarcoidosis should be considered even in patients presenting with clinically uncharacteristic manifestations.
Journal of Hand Surgery (British and European Volume, 1997) 22B." 5:676 678 Although involvement of the hands in sarcoidosis is rare, with a reported incidence of 0.2%, it may precede the onset of systemic disease (Gottlieb and Wenner, 1992; James et al, 1976; Landi et al, 1983). The presentation of sarcoidosis in the hand is non-specific, can be variable and presents with digital swelling, soft tissue masses or cutaneous lesions (James et al, 1976; Pierson and Willett, 1978; Pitt et al, 1983; Sartoris et al, 1985). Untreated sarcoidosis can lead to a number of complications, from mild dysfunction to permanent damage to the pulmonary, cardiac, and nervous systems (Crystal, 1994; Gottlieb and Wenner, 1992; Tanoue, 1996). Previous reports indicate that sarcoid involvement of the soft tissues in the hand commonly presents with painless digital swelling and variable destruction of the underlying bone (Curco et al, 1995; Elgart, 1986; Gottlieb and Wenner, 1992; Sartoris et al, 1985; Vainsencher and Winkleman, 1984). We report a case of extensive bilateral hand involvement with digital soft tissue nodules extending into the pulp spaces.
Sclerotic and lytic changes were confined to the phalanges of the right long finger (Fig 2). Bone scintigraphy with 99mTechnetium-MDP excluded osseous involvement elsewhere. Magnetic resonance imaging revealed multiple nodular areas in the subcutaneous and deep tissues with decreased signal intensity on both T f a n d T2-weighted images. Routine laboratory studies were unremarkable except for a slightly elevated uric acid level at 8.8 mg/dl (normal 3.0-8.0 mg/dl). An excisional biopsy of the mass in the pulp and the mass overlying the dorsum of the proximal interpha-
CASE REPORT
A 57-year-old factory worker presented to our emergency department with an incarcerated ring on his left little finger. He provided a history of progressive, painless swelling in all his digits of several years duration and which had recently become worse. He denied symptoms of systemic illness or history of chronic disease. However, a mediastinal biopsy had been performed at another hospital, 13 years before. Past records revealed that the patient had undergone a paratracheal lymph node biopsy which showed sarcoidosis, but he did not attend for treatment at that time. Physical examination revealed nodular masses extending from the level of the middle digital creases to the pulps of all digits bilaterally. There was a duskiness over the distal phalanges. The irregular soft tissue swellings were non-tender, firm, and had well-demarcated borders. They were around 3.0 cm in their largest diameters (Fig 1). Discrete nodules were noted also overlying the dorsal aspect of the proximal interphalangeal joints of the ring and long fingers of the left hand. The active range of digital motion was limited as the soft tissue swellings precluded making a fist. Chest X-rays were unremarkable except for surgical clips in the mediastinum. X-ray studies of both hands showed multiple, asymmetrical, nodular, soft-tissue densities. There was no calcification within the masses.
Fig 1
676
Clinical photograph of the left palm demonstrating multiple soft tissue masses.
NODULAR SARCO1DOSIS
Fig 2
Posteroanterior X-ray of the right hand demonstrating multiple soft tissue densities. Note sclerotic and lytic changes in the phalanges of the long finger (inset).
langeal joint of the right long finger was carried out. Intraoperatively, the masses appeared yellow-brown, solid and fibrous, intimately adherent to and poorly demarcated from the overlying skin and deeper soft tissues including the extensor tendon expansion. A debulking of the masses was performed preserving neurovascular structures and the extensor tendon. The operative wounds healed without incident. Histological examination of biopsy tissue revealed non-caseating granulomata surrounding deep dermal and subcutaneous tissue. There were no polarizable foreign bodies. PAS and acid-fast stains were negative, as were routine bacterial and fungal cultures. These findings, in conjunction with the prior biopsy report of sarcoid involvement in perihilar lymph nodes, confirmed the diagnosis of sarcoidosis. DISCUSSION Sarcoidosis is a systemic illness which typically presents
677
with involvement of perihilar lymph nodes, pulmonary parenchyma, skin and eye structures (Crystal, 1994; Elgart, 1986; Tanoue, 1996; Tanoue et al, 1993). Sarcoid nodules are a rare manifestation of the disease, involving the subcutaneous soft tissues in 2.2 to 6% of cases (Maycock et al, 1963; Vainsencher and Winkleman, 1984). Previous reports indicate that these nodules are most commonly located in the palm or in the digits at the level of the proximal and middle phalanges (Curco et al, 1995; Gottlieb and Wenner, 1992; Scott and Scott, 1984). Extensive nodular sarcoidosis involving multiple digits with lesions extending to the finger tips as seen in this patient has not been reported previously. In the clinical presentation of nodular swellings in the hands, other diagnoses should be considered. Although pigmented villonodular synovitis can present similarly with a firm and painless nodule, the multiplicity of digits involved in this case excluded this diagnosis (Jones et al, 1969; Moore et al, 1984). Neurofibromata presenting in multiple digits are a manifestation of von Recklinghausen's disease which can be diagnosed by its characteristic stigmata (Idler, 1995; Rinaldi, 1983). Tuberculosis usually presents with single digital involvement, without discrete nodular swellings and in the setting of systemic signs and symptoms (Robins, 1967). Although amyloidosis associated with chronic diseases can present with similar swellings, they are localized to flexor tendons and their sheaths (Hallett, 1982; Kurer et al, 1991). Once sarcoidosis has been confirmed, the degree of involvement of other organs, especially the lungs and eyes, should be sought. Pulmonary function studies and an ophthalmological examination are warranted as interstitial lung disease with progressive pulmonary fibrosis and impaired vision or blindness cause significant morbidity (Crystal, 1994; Tanoue, 1996). Subcutaneous sarcoid nodules rarely cause major problems unless organ dysfunction results from architectural distortion due to extensive granulomatous involvement (Gross et al, 1971). The overall prognosis of sarcoidosis is, however, good, with spontaneous remissions occurring in 50% of patients. Low dose, intermittent glucocorticoid therapy and rarely cytotoxic drug therapy are used in refractory cases (Crystal, 1994; Tanoue, 1996). Although sarcoidosis commonly presents with characteristic signs and symptoms, unusual manifestations should not preclude its diagnosis. Nodular sarcoidosis involving the hands is rare, but the diagnosis should be considered when presented with atypical soft-tissue swelling in the hands. REFERENCES Crystal RG. Sarcoidosis. In: Isselbacher KJ, Braunwald E, Wilson JD, Martin JB, Fauci AS, Kasper DL (Eds), Harrison's principles of internal medicine, 13th edn, New York, McGraw-Hill 1994:1679 1684. Curco N, Pagerols X, Vives P (1995). Subcutaneous sarcoidosis with dactylitis. Clinical and Experimental Dermatology, 20:434 435. Elgart ML (1986). Cutaneous sarcoidosis: definitions and types of lesions. Clinics in Dermatology, 4: 3541.
678 Gottlieb DC, Wenner SM (1992). Soft tissue sarcoidosis of the hand: a case report. Journal of H a n d Surgery, 17B: 23~231. Gross MD, Andriacchi F, Gordon R, Maddox D (1971). Nodular subcutaneous sarcoidosis. Archives of Dermatology, 113: 1442-I443. Hallett J (1982). Tendon tethering in the carpal tunnel due to amyloidosis in Bence-Jones myelomatosis. Journal of Bone and Joint Surgery, 64B: 357-360. Idler RS (I 995). Benign and malignant nerve tumors. Hand Clinics, 11: 203~09. James DG, Neville E, Castairs LS (1976). Bone and joint sarcoidosis. Seminars in Arthritis and Rheumatism, 6: 53-81. Jones FE, Soule EH, Coventry MB (1969). Fibrous xanthoma of synovium (giant-cell tumor of tendon sheath, pigmented nodular synovitis). Journal of Bone and Joint Surgery, 51A: 76 86. Kurer MHJ, Baillod RA, Mad gwick JCA (1991 ). Musculoskeletal manifestations of amyloidosis. Journal of Bone and Joint Surgery, 73B: 271 276. Landi A, Brooks D, De Santis G (1983). Sarcoidosis of the hand - - report of two cases. Journal of H a n d Surgery, 8:197 200. Maycock RL, Bertrand P, Morrison CC, Scott J H (1963). Manifestations of sarcoidosis. American Journal of Medicine, 35: 67-89. Moore JR, Weiland A J, Curtis R M (1984). Localized nodular tenosynovitis: Experience with 115 cases. Journal of H a n d Surgery, 9A: 4 1 2 4 17. Pierson D J, Willett ES (1978). Sarcoidosis presenting with finger pain. Journal of the American Medical Association, 239: 2023~024.
T H E J O U R N A L OF H A N D SURGERY VOL. 22B No. 5 O C T O B E R 1997 Pitt P, Hamilton EB, Innes EH, Morley KD, Monk BE, Hughes G R (1983). Sarcoid dactylitis. Annals of the Rheumatic Diseases, 42:634 639. Rinaldi E (1983). Neurilemomas and neurofibromas of the upper limb. Journal of H a n d Surgery, 8: 590-593. Robins R H C (1967). Tuberculosis of the wrist and hand. British Journal of Surgery, 54:211-218. Sartoris DJ, Resnick D, Resnick C, Yaghmai I (1985). Musculoskeletal manifestations of sarcoidosis. Seminars in Roentgenology, 20:375 386. Scott TH, Scott M A (1984). Sarcoidosis with nodular lesions of the palm and sole. Archives of Dermatology, 120: 1239-1240. Tanoue LT (1996). Systemic sarcoidosis. Resident and Staff Physician; 42: 39~47. Tanoue LT, Zitnik R, Elias JA. Systemic sarcoidosis. In: Baum G L, Wolinsky E (Eds), Textbook ~?fpulrnonary diseases, 5th edn, Boston, Little, Brown, 1993:745 774. Vainsencher D, Winkehnann R K (1984). Subcutaneous sarcoidosis. Archives of Dermatology 120:1028 1031.
Received: 7 November 1996 Accepted after revision: 17 February 1997 Dr R. H. Gelberman, Department of Orthopaedic Surgery,Washington University School of Medicine, One Barnes Hospital Plaza, Suite 11300,Saint Louis, Missouri 63110, USA. © 1997The British Society for Surgery of the Hand