Case Report: Ainhum (Spontaneous Dactylolysis) in a 65-Year-Old American Black Man JAMES T. GREENE, MD,
RUTH-MARIE E. FINCHER, MD
ABSTRACT: Ainhum is the spontaneous autoamputation of toes as a result of the formation of a constricting band. It usually affects the fifth toe bilaterally and predominantly affects blacks in tropical regions, but has been uncommonly reported in temperate regions as well, including the United States. Of the 29 cases identified in the American literature since 1960, only 6 were published in the internal medicine literature. Because of the rarity of ainhum and consequent lack of attention in the clinical literature, this condition may be relatively unknown and therefore unrecognized by practicing internists. A patient with ainhum is described and the clinical features of this rare disease are emphasized to facilitate recognition and appropriate management. KEY INDEXING TERMS: Ainhum; Spontaneous dactylolysis; Constricting band; Pseudoainhum; Z-plasty. [Am J Med Sci 1992; 303(2):118-120.]
A
inhum is a tropical disease of uncertain etiology characterized by the development of a constricting band around the fifth toe, resulting in spontaneous auto amputation, which occurs predominately in dark-skinned races. Since the index case was reported in 1881, approximately 130 cases have been reported in the temperate United States, mostly from southern regions. However, although 29 cases have been reported in the American literature since 1960, only 6 were published in the internal medicine literature, four of which were in state medical journals.1-15 Because this disease remains largely unrecognized among internists, we report a case of ainhum in an American black man to call attention to this uncommon illness and discuss the pertinent clinical features. From the Department of Medicine, Section of General Internal Medicine, l11edical College of Georgia, Augusta, Georgia. Correspondence: Ruth·Marie E. Fincher, MD, Medical College of GA, 1120 15th Street, Department of Medicine BIW-540, Augusta, Georgia 30912.
118
Case Report A 65-year-old black man, a life-long resident of South Carolina, came to the Veterans Administration Medical Center in Augusta, Georgia with complaints of a painful left fifth toe. He reported that his right fifth toe spontaneously "fell off" 3 months earlier after he sought medical attention for it at the same institution. Initially a callous developed between the fourth and fifth toes on his right foot, with a constricting band that eventually encircled the toe, resulting in autoamputation. He denied a history of trauma to his feet or prolonged periods of shoelessness. He had no history of diabetes, syphilis, leprosy, tuberculosis, lupus, or parasitic disease. Results of the physical examination included a body temperature of 37 C, a blood pressure of 159/80 mm Hg, and a pulse rate of 74 beats per minute. The pertinent physical findings were limited to the feet. The right fifth toe was absent. The left fifth toe was laterally rotated to the left and constricted by a fibrous band (Figure 1). The toe was painful when examined by palpation and there was soft-tissue swelling involving the toe and dorsum of the foot. Serum chemistry and complete blood count test results were normal. Radiographs of the left foot showed boney reabsorption and narrowing of the fifth phalanx with soft-tissue swelling (Figure 2).
Discussion
Ainhum occurs most frequently in dark-skinned races from tropical regions including Africa, Central and South America, the West Indies, India, and China. 16,17 Patients have been reported from temperate regions as well, including Europe and Russia. l l The first description of ainhum was published by da Silva Lima16 from Bahia, Brazil in 1867. The index case reported in the United States was published in 1881.6 Since then, about 130 documented cases have been reported from the United States, most of which involved blacks of African descent who lived in or originated from the South. 1,3-7,l6-18 The disease has been reported only rarely in whites. 13,19 The term ainhum has been attributed to the language of the Nagos tribe in Brazil and means "fissure." It is closely related to a term "Ayun," meaning "to saw," from the Lagos tribe in Nigeria, and therefore appears to be of African origin.6 The accepted medical term is dactylolysis spontanea, or spontaneous dactylolysis. l l Although early reports indicated a male predominance, there appears to be no sex preference overall. 20 The age at onset is usually in the fourth or fifth decade. 3 The disease occurs bilaterally in 75% of patientsY February 1992 Volume 303 Number 2
Greene and Fincher
hum may be subdivided into congenital conditions, including congenital constriction bands and other rare conditions, and acquired conditions including leprosy, scleroderma, Raynaud's disease, ergot poisoning, trauma burns, frostbite, diabetes mellitus, syringomyelia, and syphilis and other treponemal diseases.12.22-25 The genetic tendency in blacks for overproduction of fibrous tissue as a response to injury or infection has been proposed as an important causal factor for development of ainhum. 26.27 Pathologic studies seem to support this hypothesis. Histologic findings include hyperkeratosis and parakeratosis of the involved epidermis, with excessive connective tissue and collagen fibers. The constricting band itself also consists of collagen. 14 The diagnosis of ainhum is based on characteristic clinical and radiographic findings. 6 Cole28 described four clinical stages of ainhum. The first stage is marked by the formation of a groove or fissure, often in association with a hyperkeratotic lesion (corn or callous), as in our patient. The fissure or groove slowly encircles the toe and is accompanied by a variable degree of
Figure 1. Photograph demonstrating the constricting band around the base of the left fifth toe.
The cause of ainhum is unknown, but is probably an acquired condition. 16 Although there is no evidence to support a simple genetic mode of transmission, the disease usually occurs in blacks and sometimes in several members of the same family.lO The fact that it can occur in patients living in climates other than tropical ones also may suggest that it is a genetic disorder. Early investigators suggested that the cause might be trauma to the small toe resulting from habitual shoelessness.3•2o However, ainhum has occurred in patients who wear shoes. 14.19 Other possible causes discussed in the earlier literature included leprosy, syphilis, yaws, and tuberculosis, which are probably coexistent rather than causative. 21 The current literature differentiates true ainhum, in which the cause is unknown, from "pseudoainhum," a term encompassing several disease entities with similar clinical presentations. PseudoainTHE AMERICAN JOURNAL OF THE MEDICAL SCIENCES
Figure 2. Radiograph of the left foot demonstrating the constricting soft tissue band around the fifth toe. Marked boney reabsorption, narrowing, and fracture of the fifth middle phalanx also are depicted.
119
Ainhum in an American Black Man
pain. II The second stage is characterized by arterial narrowing, with eventual cessation of blood flow. Bone reabsorption occurs adjacent to the groove and the toe appears bulbous. The third stage involves separation of the bones at the joint, with resultant hypermotility of the digit. Pain, often severe, usually accompanies this stage. ll The fourth stage is marked by bloodless auto amputation of the toe at the area of constriction. 2s The radiographic manifestations of ainhum initially consist of a radiolucent band constricting the base of the small toe and distal swelling and edema. lo The toe may be rotated (everted}.3 Osteoporosis and cortical thinning also are seen in the early stages. The bone gradually narrows in caliber, with resorption and eventual autoamputation. 10 The standard treatment for ainhum in Stages I through III is a surgical procedure known as Z-plasty, which was first used in the treatment of ainhum in 1959. Z-plasty combines amputation of the affected toe with release of the constricting band using a Z-shaped incision. 2 Other procedures, including longitudinal incision of the digit with proximal phalangeal resection and linear incision to interrupt the encircling groove, have been reported but generally are unsuccessful. 1 Treatment with a monthly intralesional injection of corticosteroids produced salutary results in a single case.s Summary
Ainhum is an uncommon tropical disease of blacks that sometimes is seen in the United States, particularly in the South. Physician awareness of this disorder is important for prompt recognition and appropriate management. References 1. Bergner LH, Winfield JM: Ainhum (dactylolysis spontanea), re-
view of the literature and report of a case. Am J Surg 100:480485, 1960 2. Allyn B, Leider M: Dactylolysis spontanea (ainhum), report of a case treated by the surgical procedure known as Z-plasty. JAMA 184:655-657, 1963. 3. Fetterman LE, Hardy R, Lehrer H: The clinico-roentgenologic features of ainhum. AJR 100:512-522, 1967. 4. Grossman J, Harrison HD: Ainhum (dactylolysis spontanea). NY State J Med 68:1741-1744,1968.
120
5. Dunbar WG, Hodges TO: Dactylolysis spontanea (Ainhum). Virginia Med Month 97:438-441, 1970. 6. Baerg RH: Ainhum (dactylolysis spontanea): A review of the literature and a report of two cases. JAPA 61:44-54, 1971. 7. Stephenson L: Ainhum (Dactylosis Spontanea): Report of a case. N Carolina Med J 36:404-405, 1975. 8. Rossiter JW, Anderson PC: Ainhum: Treatment with intralesional steroids. Int J Dermatol15:379-382, 1976. 9. DiGiovanni JE, Fallat LM: Ainhum: Report of a case. JAPA 67: 401-405, 1977. 10. Mendelson DS, Chan KF, Song IS: Spontaneous dactyloiysis with pain in a 58-year·old American Black man. JAMA 246: 1591-1592, 1981. 11. Rausher H, Birrer RB, Aronstein M, Geiger AJ, Sande HA: Ainhum; Dactylolysis spontanea. NY State J Med 81:1779-1881, 1981. 12. Orlando CA, Roth A, Stern AR: Ainhum (dactylolysis spontanea): A literature review and case report. JAPA 71:487-490, 1981. 13. Bertoli CL, Stassi J, Rifkin MD: Ainhum, an unusual presentation involving the second toe in a white male. Shel Radiolll: 133-135, 1984. 14. Kerhishnik W, O'Donnell E, Wenig JA, McCarthy DJ: The surgical pathology of ainhum (dactylolysis spontanea). J Foot Surg 25:95-123, 1986. 15. Genakos JJ, Cocores JA, Terris A: Ainhum (Dactylolysis Spontanea) report of a bilateral case and literature review. J Amer Podiatr Med Assoc 76:676-680, 1986. 16. Spinzig EW: Ainhum: Its occurrence in the United States with a report of three cases. AJR 42:246-263, 1939. 17. Burch GE, Hale AR: A plethysmographic study of the toe of a patient with ainhum. Arch Intern Med 100:113-124, 1957. 18. Young C: Ainhum or dactylosis spontanea. South Med J 41:2931,1948. 19. Tye M: Ainhum. NEJM 234:152-154, 1946. 20. Browne SG: True Ainhum: Its distinctive and differentiating features. J Bone Joint Surg (Br) 47:52-55, 1965. 21. Dent DM, Fataar S, Rose AG: Ainhum and angiodysplasia. Lancet 2(8243): 396-397, 1981. 22. McLaurin CI: Psoriasis presenting with pseudoainhum. J Am Acad DermatoI7:130-132, 1982. 23. Schamroth JM: Mutilating keratoderma. Int J Dermatol25:249251,1986. 24. Christopher AP, Grattan CEH, Cowan MA: Pseudoainhum and erythropoietic protoporphyria. Br J Dermatol118:1l3-116, 1988. 25. Somasundaram V, Wahab AJ, Shobana S, Premalatha S, Abdul Razack EM, Muthuswamy TC: Pseudoainhum in Clouston's disease.lnt J Dermatol29:225-226, 1990. 26. Browne SG: Ainhum. Int J DermatoI15:348-350, 1976. 27. Kean BH, Tucker H: Etiologic concepts and pathologic aspects of ainhum. Arch Pathol 41:639-644, 1946. 28. Cole GJ: Ainhum; An account of fifty-four patients with special reference to etiology and treatment. J Bone Joint Surg (Br) 47: 43-51, 1965.
February 1992 Volume 303 Number 2
RUTH-MARIE E. FINCHER, MD
ABSTRACT: Ainhum is the spontaneous autoamputation of toes as a result of the formation of a constricting band. It usually affects the fifth toe bilaterally and predominantly affects blacks in tropical regions, but has been uncommonly reported in temperate regions as well, including the United States. Of the 29 cases identified in the American literature since 1960, only 6 were published in the internal medicine literature. Because of the rarity of ainhum and consequent lack of attention in the clinical literature, this condition may be relatively unknown and therefore unrecognized by practicing internists. A patient with ainhum is described and the clinical features of this rare disease are emphasized to facilitate recognition and appropriate management. KEY INDEXING TERMS: Ainhum; Spontaneous dactylolysis; Constricting band; Pseudoainhum; Z-plasty. [Am J Med Sci 1992; 303(2):118-120.]
A
inhum is a tropical disease of uncertain etiology characterized by the development of a constricting band around the fifth toe, resulting in spontaneous auto amputation, which occurs predominately in dark-skinned races. Since the index case was reported in 1881, approximately 130 cases have been reported in the temperate United States, mostly from southern regions. However, although 29 cases have been reported in the American literature since 1960, only 6 were published in the internal medicine literature, four of which were in state medical journals.1-15 Because this disease remains largely unrecognized among internists, we report a case of ainhum in an American black man to call attention to this uncommon illness and discuss the pertinent clinical features. From the Department of Medicine, Section of General Internal Medicine, l11edical College of Georgia, Augusta, Georgia. Correspondence: Ruth·Marie E. Fincher, MD, Medical College of GA, 1120 15th Street, Department of Medicine BIW-540, Augusta, Georgia 30912.
118
Case Report A 65-year-old black man, a life-long resident of South Carolina, came to the Veterans Administration Medical Center in Augusta, Georgia with complaints of a painful left fifth toe. He reported that his right fifth toe spontaneously "fell off" 3 months earlier after he sought medical attention for it at the same institution. Initially a callous developed between the fourth and fifth toes on his right foot, with a constricting band that eventually encircled the toe, resulting in autoamputation. He denied a history of trauma to his feet or prolonged periods of shoelessness. He had no history of diabetes, syphilis, leprosy, tuberculosis, lupus, or parasitic disease. Results of the physical examination included a body temperature of 37 C, a blood pressure of 159/80 mm Hg, and a pulse rate of 74 beats per minute. The pertinent physical findings were limited to the feet. The right fifth toe was absent. The left fifth toe was laterally rotated to the left and constricted by a fibrous band (Figure 1). The toe was painful when examined by palpation and there was soft-tissue swelling involving the toe and dorsum of the foot. Serum chemistry and complete blood count test results were normal. Radiographs of the left foot showed boney reabsorption and narrowing of the fifth phalanx with soft-tissue swelling (Figure 2).
Discussion
Ainhum occurs most frequently in dark-skinned races from tropical regions including Africa, Central and South America, the West Indies, India, and China. 16,17 Patients have been reported from temperate regions as well, including Europe and Russia. l l The first description of ainhum was published by da Silva Lima16 from Bahia, Brazil in 1867. The index case reported in the United States was published in 1881.6 Since then, about 130 documented cases have been reported from the United States, most of which involved blacks of African descent who lived in or originated from the South. 1,3-7,l6-18 The disease has been reported only rarely in whites. 13,19 The term ainhum has been attributed to the language of the Nagos tribe in Brazil and means "fissure." It is closely related to a term "Ayun," meaning "to saw," from the Lagos tribe in Nigeria, and therefore appears to be of African origin.6 The accepted medical term is dactylolysis spontanea, or spontaneous dactylolysis. l l Although early reports indicated a male predominance, there appears to be no sex preference overall. 20 The age at onset is usually in the fourth or fifth decade. 3 The disease occurs bilaterally in 75% of patientsY February 1992 Volume 303 Number 2
Greene and Fincher
hum may be subdivided into congenital conditions, including congenital constriction bands and other rare conditions, and acquired conditions including leprosy, scleroderma, Raynaud's disease, ergot poisoning, trauma burns, frostbite, diabetes mellitus, syringomyelia, and syphilis and other treponemal diseases.12.22-25 The genetic tendency in blacks for overproduction of fibrous tissue as a response to injury or infection has been proposed as an important causal factor for development of ainhum. 26.27 Pathologic studies seem to support this hypothesis. Histologic findings include hyperkeratosis and parakeratosis of the involved epidermis, with excessive connective tissue and collagen fibers. The constricting band itself also consists of collagen. 14 The diagnosis of ainhum is based on characteristic clinical and radiographic findings. 6 Cole28 described four clinical stages of ainhum. The first stage is marked by the formation of a groove or fissure, often in association with a hyperkeratotic lesion (corn or callous), as in our patient. The fissure or groove slowly encircles the toe and is accompanied by a variable degree of
Figure 1. Photograph demonstrating the constricting band around the base of the left fifth toe.
The cause of ainhum is unknown, but is probably an acquired condition. 16 Although there is no evidence to support a simple genetic mode of transmission, the disease usually occurs in blacks and sometimes in several members of the same family.lO The fact that it can occur in patients living in climates other than tropical ones also may suggest that it is a genetic disorder. Early investigators suggested that the cause might be trauma to the small toe resulting from habitual shoelessness.3•2o However, ainhum has occurred in patients who wear shoes. 14.19 Other possible causes discussed in the earlier literature included leprosy, syphilis, yaws, and tuberculosis, which are probably coexistent rather than causative. 21 The current literature differentiates true ainhum, in which the cause is unknown, from "pseudoainhum," a term encompassing several disease entities with similar clinical presentations. PseudoainTHE AMERICAN JOURNAL OF THE MEDICAL SCIENCES
Figure 2. Radiograph of the left foot demonstrating the constricting soft tissue band around the fifth toe. Marked boney reabsorption, narrowing, and fracture of the fifth middle phalanx also are depicted.
119
Ainhum in an American Black Man
pain. II The second stage is characterized by arterial narrowing, with eventual cessation of blood flow. Bone reabsorption occurs adjacent to the groove and the toe appears bulbous. The third stage involves separation of the bones at the joint, with resultant hypermotility of the digit. Pain, often severe, usually accompanies this stage. ll The fourth stage is marked by bloodless auto amputation of the toe at the area of constriction. 2s The radiographic manifestations of ainhum initially consist of a radiolucent band constricting the base of the small toe and distal swelling and edema. lo The toe may be rotated (everted}.3 Osteoporosis and cortical thinning also are seen in the early stages. The bone gradually narrows in caliber, with resorption and eventual autoamputation. 10 The standard treatment for ainhum in Stages I through III is a surgical procedure known as Z-plasty, which was first used in the treatment of ainhum in 1959. Z-plasty combines amputation of the affected toe with release of the constricting band using a Z-shaped incision. 2 Other procedures, including longitudinal incision of the digit with proximal phalangeal resection and linear incision to interrupt the encircling groove, have been reported but generally are unsuccessful. 1 Treatment with a monthly intralesional injection of corticosteroids produced salutary results in a single case.s Summary
Ainhum is an uncommon tropical disease of blacks that sometimes is seen in the United States, particularly in the South. Physician awareness of this disorder is important for prompt recognition and appropriate management. References 1. Bergner LH, Winfield JM: Ainhum (dactylolysis spontanea), re-
view of the literature and report of a case. Am J Surg 100:480485, 1960 2. Allyn B, Leider M: Dactylolysis spontanea (ainhum), report of a case treated by the surgical procedure known as Z-plasty. JAMA 184:655-657, 1963. 3. Fetterman LE, Hardy R, Lehrer H: The clinico-roentgenologic features of ainhum. AJR 100:512-522, 1967. 4. Grossman J, Harrison HD: Ainhum (dactylolysis spontanea). NY State J Med 68:1741-1744,1968.
120
5. Dunbar WG, Hodges TO: Dactylolysis spontanea (Ainhum). Virginia Med Month 97:438-441, 1970. 6. Baerg RH: Ainhum (dactylolysis spontanea): A review of the literature and a report of two cases. JAPA 61:44-54, 1971. 7. Stephenson L: Ainhum (Dactylosis Spontanea): Report of a case. N Carolina Med J 36:404-405, 1975. 8. Rossiter JW, Anderson PC: Ainhum: Treatment with intralesional steroids. Int J Dermatol15:379-382, 1976. 9. DiGiovanni JE, Fallat LM: Ainhum: Report of a case. JAPA 67: 401-405, 1977. 10. Mendelson DS, Chan KF, Song IS: Spontaneous dactyloiysis with pain in a 58-year·old American Black man. JAMA 246: 1591-1592, 1981. 11. Rausher H, Birrer RB, Aronstein M, Geiger AJ, Sande HA: Ainhum; Dactylolysis spontanea. NY State J Med 81:1779-1881, 1981. 12. Orlando CA, Roth A, Stern AR: Ainhum (dactylolysis spontanea): A literature review and case report. JAPA 71:487-490, 1981. 13. Bertoli CL, Stassi J, Rifkin MD: Ainhum, an unusual presentation involving the second toe in a white male. Shel Radiolll: 133-135, 1984. 14. Kerhishnik W, O'Donnell E, Wenig JA, McCarthy DJ: The surgical pathology of ainhum (dactylolysis spontanea). J Foot Surg 25:95-123, 1986. 15. Genakos JJ, Cocores JA, Terris A: Ainhum (Dactylolysis Spontanea) report of a bilateral case and literature review. J Amer Podiatr Med Assoc 76:676-680, 1986. 16. Spinzig EW: Ainhum: Its occurrence in the United States with a report of three cases. AJR 42:246-263, 1939. 17. Burch GE, Hale AR: A plethysmographic study of the toe of a patient with ainhum. Arch Intern Med 100:113-124, 1957. 18. Young C: Ainhum or dactylosis spontanea. South Med J 41:2931,1948. 19. Tye M: Ainhum. NEJM 234:152-154, 1946. 20. Browne SG: True Ainhum: Its distinctive and differentiating features. J Bone Joint Surg (Br) 47:52-55, 1965. 21. Dent DM, Fataar S, Rose AG: Ainhum and angiodysplasia. Lancet 2(8243): 396-397, 1981. 22. McLaurin CI: Psoriasis presenting with pseudoainhum. J Am Acad DermatoI7:130-132, 1982. 23. Schamroth JM: Mutilating keratoderma. Int J Dermatol25:249251,1986. 24. Christopher AP, Grattan CEH, Cowan MA: Pseudoainhum and erythropoietic protoporphyria. Br J Dermatol118:1l3-116, 1988. 25. Somasundaram V, Wahab AJ, Shobana S, Premalatha S, Abdul Razack EM, Muthuswamy TC: Pseudoainhum in Clouston's disease.lnt J Dermatol29:225-226, 1990. 26. Browne SG: Ainhum. Int J DermatoI15:348-350, 1976. 27. Kean BH, Tucker H: Etiologic concepts and pathologic aspects of ainhum. Arch Pathol 41:639-644, 1946. 28. Cole GJ: Ainhum; An account of fifty-four patients with special reference to etiology and treatment. J Bone Joint Surg (Br) 47: 43-51, 1965.
February 1992 Volume 303 Number 2
![[Ainhum (spontaneous dactylolysis): about a clinical case].](/assets/img/hold.png)
