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Pachydermodactyly: A case report and discussion of the pathologic entity
The lournal of HAND SURGERY
Vathana
quite responsive to irradiation. In this case an en bloc resection was performed and iliac bone graft served as a replacement. The patient has no recurrence 2 years after operation (Fig. 5). Thanks to Dr. Suvarin Khantanaphar for his valuable advice and review.
REFERENCES 1. Dahlin DC: Bone tumor-General aspects and data on 6221 cases, ed 3. Springfield, 1978, Charles C Thomas Publisher, pp 344-6 2. Mirra 1M, Gold RH, Marcove RC: Bone tumors: diagnosis and treatment. Philadelphia, 1980, JB Lippincott Co, pp 509-16 3. Marcial-Rojas PA: Primary hemangiopericytoma of bone. Cancer 2:308, 1960
4. Stout AP: Tumor featuring pericytes: Glomus tumor and hemangiopericytoma. Lab Invest 5:217, 1956 5. McCormac LJ, Galliva WF: Hemangiopericytoma. Cancer 7:595, 1954 6. Dunlop 1: Primary hemangiopericytoma of bone-report of two cases. 1 Bone 10int Surg [Br] 55:854, 1973 7. Yang PS, Falk E: Hemangiopericytoma of bone: Review of the literature and report of a case. Acta Orthop Scand 51:903-7, 1980 8. Zimmerman KW: Der feinere bau der blutcapillaren. Z Anat Entwicklungs 68:29, 1923 9. Sage H, Salman 0: Malignant hemangiopericytoma in the area of previous ameloblastoma of the mandible. Oral Surg 26:275, 1968
Pachydermodactyly: A case report and discussion of the pathologic entity Numerous case reports have described idiopathic hypertrophy of the skin on the dorsum of the hands. Many different and confusing names have been assigned to these conditions. A case report of a patient with pachydermodactyly is presented, with a discussion of the literature, in an effort to clarify the identification, treatment, and nomenclature of idiopathic hyperkeratosis and acanthosis of the dorsum of the hands. (J HAND SURG 9A:764·6, 1984.)
Thomas B. Fleeter, M.D., Carmen Myrie, M.D., and John P. Adams, M.D.,
Washington, D. C.
In 1R93, Garrod 1 described the development of nodules on the dorsum of the proximal interphalangeal (PIP) joints of the hand. Since that time several reports 2 - 5 have appeared that detail idiopathic hypertrophic changes of the skin on the dorsum of the metacarpophalangeal (MCP) and PIP joints of the
From the Departments of Orthopaedic Surgery, Dermatology, and Pathology, The George Washington University Medical Center, Washington, D. C. Received for publication April 12, 1983; accepted in revised form June 13, 1983. Reprint requests: Thomas B. Fleeter. M.D., Department of Orthopedic Surgery, The George Washington University Medical Center, 2150 Pennsylvania Ave. N.W., Washington, DC 20037.
764
THE JOURNAL OF HAND SURGERY
hand. Various descriptive terms have been assigned to this condition, including pachyderma, knuckle pad disease, discrete keratoderma, acanthohyperkeratosis, and fibromatosis. All of these terms describe the same histologic and clinical condition of hyperkeratosis, acanthosis, and increased dermal thickness (fibroplasia) on the skin of the dorsum of the PIP and MCP joints of the fingers, causing confusion in identifying and describing this entity. A case report of this disease is presented, with a discussion to clarify the terminology, diagnosis, and treatment of this problem.
Case report A 22-year-old white man was initially seen with complaints of asymptomatic swelling of the fingers of both hands.
Vol. 9A, No.5 September 1984
Pachydermodactyly
765
Fig. 1. Before operation the hands demonstrated marked swelling of soft tissue .
Fig. 3. Compare the markedly thickened epidermis and dermis to the normal tissues in Fig . 4 . Fig. 2. Postoperative view of the hands shows normal appearance of soft tissue. There was no family history of similar problems. He noted onset of this at age 15 years when his rings and gloves became tight. It was believed that he had acromegaly because of the enlarged appearance of his hands and jaw and apparent frontal bossing . However, results of a workup for acromegaly , thyroid dysfunction, and hyperprolactinemia were negative . The soft tissue over the PIP joints of the right thumb and index, long, and ring fingers and over the left thumb and index finger was markedly increased . Each finger showed a firm intradermal, subcutaneous swelling over the dorsal and lateral aspects of the PIP joint. The skin was freely movable but thickened, scaly, and doughy in consistency. Each joint was otherwise normal (Fig. 1). Roentgenograms of the hands revealed only swelling of the soft tissue. There were no other systemic manifestations of disease. Biopsies of the involved areas revealed extensive hyperkeratosis , epidermal hyperplasia (acanthosis) , and dramatic thickening of the dermis by increased numbers of fibroblasts and collagen deposits. There also were increased numbers of eccrine glands, many of which were partially fibrosed. Biochemical analysis of the involved tissue showed
no evidence of changes consistent with Dupuytren's disease.:l The patient had subcutaneous resections over the involved PIP joints of the right hand and recovered without sequelae (Fig. 2) .
Discussion Changes of hyperkeratosis and acanthosis of the skin have been defined as pachyderma because of the greatly thickened, scaly appearance. When these changes appear on the fingers, the term "pachydermodactyly" has been applied. Pachydermodactyly was first described by Garrod 1 in 1893. There have been few mentions of it in the literature since that time . Morginson 2 described a series of patients with discrete keratodermas over the finger articulations, associated with perifollicular hyperkeratosis and seborrheic dermatitis. Because this entity is clinically different from pachydermodactyly, and because histologically it refers to hyperkeratosis and acanthosis without dermal thickening and fibrosis, it should be in a category separate from pachydermodactyly.
766
The Journal of HAND SURGERY
Fleeter et al.
thickened, and without erythema. Complaints are generally limited to cosmetic appearance or increasing hand size. Histologically the disease is marked by hyperkeratosis, acanthosis, and a thickened fibrotic dermis with increased numbers of eccrine glands entrapped in the fibrous stroma (Figs. 3 and 4). In addition to acromegaly and thyroid dysfunction, several other conditions have been associated with pachydermodactyly. These include seborrheic dermatitis, glossitis, and pachydermoperiostosis, 6-8 in which there is clubbing of the fingers and toes and periosteal reactions at the phalangeal tufts. In pachydermodactyly, the radiographic appearance of the hands is normal, whereas in pachydermoperiostosis there often is resorption of the phalangeal tufts. Both diseases are characterized by pachyderma and may involve changes in the face with thickening, furrowing, and oiliness of the forehead skin. These changes may easily be confused with those of acromegaly, and, additionally, patients with acromegaly may develop pachyderma of the hands and feet.
Summary
Fig. 4. A biopsy specimen from the normal-appearing small finger reveals no obvious changes.
Discrete keratoderma2 refers to histologic features of hyperkeratosis and acanthosis only. Fibromatosis lacks epidermal hyperkeratosis and acanthosis and demonstrates only dermal fibromatosis. Thus neither of these terms accurately represents the clinical or histologic changes that occur in pachydermodactyly. Lagier and Meinecke3 reviewed the pathology of knuckle pad disease and found the histologic changes in the fibrous tissue to be similar to those in Dupuytren's disease, along with a high correlation between the presence of knuckle pad disease and Dupuytren's disease. However, the marked increases in dermal collagen and mucopolysaccharide deposition noted in pachydermodactyly are not seen in knuckle pad disease and may represent a different entity. In pachydermodactyly the lesions are barely perceptible in their early formation but progress to approximately 5 mm in thickness and are found on the dorsum of the MCP and PIP joints. Similar changes may occur around the elbows. The lesions are circumscribed and freely movable. The skin appears scaly, markedly
Pachydermodactyly is an entity that includes hyperkeratosis and thickening of the epidermis and dermis of the skin overlying the dorsum of the MCP and PIP joints. When present, it usually is not associated with any other disease and requires treatment only for cosmetic reasons or when it interferes with the patient's hand function. However, one must be aware that pachydermodactyly may be a manifestation of underlying acromegaly. REFERENCES 1. Garrod AE: On an unusual form of nodule upon the joints of the fingers. Bartholomew's Hosp Rep 29:157-61,1893 2. Morginson WJ: Discrete keratodermas over the knuckle and finger articulations. Arch Dermatol 71:349-53, 1955 3. Lagier R, Meinecke R: Pathology of knuckle pads. Virchows Arch [Pathol Anat] 365:185-91, 1975 4. Jones HW: Two cases of knuckle pads. Br Med J 1:759, 1923 5. Sweitzer SE, Winer LH: Fibrotic nodules of the skin. Arch Dermatol Syph 25:1-5, 1945 6. Lauter SA, Vasey FB, Huttner I, Osterland CK, et al: Pachydermoperiostosis: studies on the synovium. J Rheumatol 5:85-95, 1978 7. Yu YL, Turck WPG: Pachydermoperiostosis. Postgrad Med J 57:521-4, 1981 8. Guyer PB, Brunton FJ, Wren MW, et al: Pachydermoperiostosis with aero-osteolysis. A report of five cases. J Bone Joint Surg [Br]60:219-22, 1978
Vathana
quite responsive to irradiation. In this case an en bloc resection was performed and iliac bone graft served as a replacement. The patient has no recurrence 2 years after operation (Fig. 5). Thanks to Dr. Suvarin Khantanaphar for his valuable advice and review.
REFERENCES 1. Dahlin DC: Bone tumor-General aspects and data on 6221 cases, ed 3. Springfield, 1978, Charles C Thomas Publisher, pp 344-6 2. Mirra 1M, Gold RH, Marcove RC: Bone tumors: diagnosis and treatment. Philadelphia, 1980, JB Lippincott Co, pp 509-16 3. Marcial-Rojas PA: Primary hemangiopericytoma of bone. Cancer 2:308, 1960
4. Stout AP: Tumor featuring pericytes: Glomus tumor and hemangiopericytoma. Lab Invest 5:217, 1956 5. McCormac LJ, Galliva WF: Hemangiopericytoma. Cancer 7:595, 1954 6. Dunlop 1: Primary hemangiopericytoma of bone-report of two cases. 1 Bone 10int Surg [Br] 55:854, 1973 7. Yang PS, Falk E: Hemangiopericytoma of bone: Review of the literature and report of a case. Acta Orthop Scand 51:903-7, 1980 8. Zimmerman KW: Der feinere bau der blutcapillaren. Z Anat Entwicklungs 68:29, 1923 9. Sage H, Salman 0: Malignant hemangiopericytoma in the area of previous ameloblastoma of the mandible. Oral Surg 26:275, 1968
Pachydermodactyly: A case report and discussion of the pathologic entity Numerous case reports have described idiopathic hypertrophy of the skin on the dorsum of the hands. Many different and confusing names have been assigned to these conditions. A case report of a patient with pachydermodactyly is presented, with a discussion of the literature, in an effort to clarify the identification, treatment, and nomenclature of idiopathic hyperkeratosis and acanthosis of the dorsum of the hands. (J HAND SURG 9A:764·6, 1984.)
Thomas B. Fleeter, M.D., Carmen Myrie, M.D., and John P. Adams, M.D.,
Washington, D. C.
In 1R93, Garrod 1 described the development of nodules on the dorsum of the proximal interphalangeal (PIP) joints of the hand. Since that time several reports 2 - 5 have appeared that detail idiopathic hypertrophic changes of the skin on the dorsum of the metacarpophalangeal (MCP) and PIP joints of the
From the Departments of Orthopaedic Surgery, Dermatology, and Pathology, The George Washington University Medical Center, Washington, D. C. Received for publication April 12, 1983; accepted in revised form June 13, 1983. Reprint requests: Thomas B. Fleeter. M.D., Department of Orthopedic Surgery, The George Washington University Medical Center, 2150 Pennsylvania Ave. N.W., Washington, DC 20037.
764
THE JOURNAL OF HAND SURGERY
hand. Various descriptive terms have been assigned to this condition, including pachyderma, knuckle pad disease, discrete keratoderma, acanthohyperkeratosis, and fibromatosis. All of these terms describe the same histologic and clinical condition of hyperkeratosis, acanthosis, and increased dermal thickness (fibroplasia) on the skin of the dorsum of the PIP and MCP joints of the fingers, causing confusion in identifying and describing this entity. A case report of this disease is presented, with a discussion to clarify the terminology, diagnosis, and treatment of this problem.
Case report A 22-year-old white man was initially seen with complaints of asymptomatic swelling of the fingers of both hands.
Vol. 9A, No.5 September 1984
Pachydermodactyly
765
Fig. 1. Before operation the hands demonstrated marked swelling of soft tissue .
Fig. 3. Compare the markedly thickened epidermis and dermis to the normal tissues in Fig . 4 . Fig. 2. Postoperative view of the hands shows normal appearance of soft tissue. There was no family history of similar problems. He noted onset of this at age 15 years when his rings and gloves became tight. It was believed that he had acromegaly because of the enlarged appearance of his hands and jaw and apparent frontal bossing . However, results of a workup for acromegaly , thyroid dysfunction, and hyperprolactinemia were negative . The soft tissue over the PIP joints of the right thumb and index, long, and ring fingers and over the left thumb and index finger was markedly increased . Each finger showed a firm intradermal, subcutaneous swelling over the dorsal and lateral aspects of the PIP joint. The skin was freely movable but thickened, scaly, and doughy in consistency. Each joint was otherwise normal (Fig. 1). Roentgenograms of the hands revealed only swelling of the soft tissue. There were no other systemic manifestations of disease. Biopsies of the involved areas revealed extensive hyperkeratosis , epidermal hyperplasia (acanthosis) , and dramatic thickening of the dermis by increased numbers of fibroblasts and collagen deposits. There also were increased numbers of eccrine glands, many of which were partially fibrosed. Biochemical analysis of the involved tissue showed
no evidence of changes consistent with Dupuytren's disease.:l The patient had subcutaneous resections over the involved PIP joints of the right hand and recovered without sequelae (Fig. 2) .
Discussion Changes of hyperkeratosis and acanthosis of the skin have been defined as pachyderma because of the greatly thickened, scaly appearance. When these changes appear on the fingers, the term "pachydermodactyly" has been applied. Pachydermodactyly was first described by Garrod 1 in 1893. There have been few mentions of it in the literature since that time . Morginson 2 described a series of patients with discrete keratodermas over the finger articulations, associated with perifollicular hyperkeratosis and seborrheic dermatitis. Because this entity is clinically different from pachydermodactyly, and because histologically it refers to hyperkeratosis and acanthosis without dermal thickening and fibrosis, it should be in a category separate from pachydermodactyly.
766
The Journal of HAND SURGERY
Fleeter et al.
thickened, and without erythema. Complaints are generally limited to cosmetic appearance or increasing hand size. Histologically the disease is marked by hyperkeratosis, acanthosis, and a thickened fibrotic dermis with increased numbers of eccrine glands entrapped in the fibrous stroma (Figs. 3 and 4). In addition to acromegaly and thyroid dysfunction, several other conditions have been associated with pachydermodactyly. These include seborrheic dermatitis, glossitis, and pachydermoperiostosis, 6-8 in which there is clubbing of the fingers and toes and periosteal reactions at the phalangeal tufts. In pachydermodactyly, the radiographic appearance of the hands is normal, whereas in pachydermoperiostosis there often is resorption of the phalangeal tufts. Both diseases are characterized by pachyderma and may involve changes in the face with thickening, furrowing, and oiliness of the forehead skin. These changes may easily be confused with those of acromegaly, and, additionally, patients with acromegaly may develop pachyderma of the hands and feet.
Summary
Fig. 4. A biopsy specimen from the normal-appearing small finger reveals no obvious changes.
Discrete keratoderma2 refers to histologic features of hyperkeratosis and acanthosis only. Fibromatosis lacks epidermal hyperkeratosis and acanthosis and demonstrates only dermal fibromatosis. Thus neither of these terms accurately represents the clinical or histologic changes that occur in pachydermodactyly. Lagier and Meinecke3 reviewed the pathology of knuckle pad disease and found the histologic changes in the fibrous tissue to be similar to those in Dupuytren's disease, along with a high correlation between the presence of knuckle pad disease and Dupuytren's disease. However, the marked increases in dermal collagen and mucopolysaccharide deposition noted in pachydermodactyly are not seen in knuckle pad disease and may represent a different entity. In pachydermodactyly the lesions are barely perceptible in their early formation but progress to approximately 5 mm in thickness and are found on the dorsum of the MCP and PIP joints. Similar changes may occur around the elbows. The lesions are circumscribed and freely movable. The skin appears scaly, markedly
Pachydermodactyly is an entity that includes hyperkeratosis and thickening of the epidermis and dermis of the skin overlying the dorsum of the MCP and PIP joints. When present, it usually is not associated with any other disease and requires treatment only for cosmetic reasons or when it interferes with the patient's hand function. However, one must be aware that pachydermodactyly may be a manifestation of underlying acromegaly. REFERENCES 1. Garrod AE: On an unusual form of nodule upon the joints of the fingers. Bartholomew's Hosp Rep 29:157-61,1893 2. Morginson WJ: Discrete keratodermas over the knuckle and finger articulations. Arch Dermatol 71:349-53, 1955 3. Lagier R, Meinecke R: Pathology of knuckle pads. Virchows Arch [Pathol Anat] 365:185-91, 1975 4. Jones HW: Two cases of knuckle pads. Br Med J 1:759, 1923 5. Sweitzer SE, Winer LH: Fibrotic nodules of the skin. Arch Dermatol Syph 25:1-5, 1945 6. Lauter SA, Vasey FB, Huttner I, Osterland CK, et al: Pachydermoperiostosis: studies on the synovium. J Rheumatol 5:85-95, 1978 7. Yu YL, Turck WPG: Pachydermoperiostosis. Postgrad Med J 57:521-4, 1981 8. Guyer PB, Brunton FJ, Wren MW, et al: Pachydermoperiostosis with aero-osteolysis. A report of five cases. J Bone Joint Surg [Br]60:219-22, 1978