Pharmacology and Therapeutics
AINHUM: TREATMENT WITH INTRALESIONAL STEROIDS JAMES W. ROSSITER, M.D, AND PHILIP C, ANDERSON, M,D.
ABSTRACT: Ainhum is a rare constricting band disease, usually affecting digits and resulting in spontaneous amputation. Prompt symptomatic relief and latter partial resolution of the fibrotic band followed intralesional steroid injection in the case presented.
Ainhum is a disease of dark-skinned people characterized by a groove encircling one or both small toes, resulting usually in spontaneous amputation.^ Involvement of other toes and fingers has been reported. Pain and mutilation caused by ainhum has been relieved by amputation or by reconstructive surgery.2' 3 |n addition to a review of the literature, a case of ainhum is presented. The patient's condition responded excellently to repeated intralesional injections of steroid in low doses with complete remission of pain and marked healing of the fissure around the toe. Case Report A 36-year-old Nigerian Negro man was seen in our Dermatology Outpatient Department because of pain in tbe fiftb toe of tbe rigbt foot of 3 years' duration. His private pbysician in Nigeria bad recommended surgical amputation some 8-12 months earlier, Witb the exception of tbe foot disorder, the patient had been in excellent health. He denied the presence of any similar disorder in family members. Examination of the fifth toe, right foot, revealed a groove extending from the plantar digital fold and encircling the toe to approximately 2/3 of
From the Division of Dermatofogy, University of Missouri Medical Center, Columbia, Missouri
its circumference (Eig, 1), Hyperkeratotic debris filled and distended tbe groove dorsomedially. Tbe segment of the digit distal to the fissure was bulbous and displayed normal neurological and vascular function. The toe was indurated and exquisitely tender. The remainder of the physical examination was unremarkable, Roentgenologic examination of tbe involved toe sbowed a narrow band-like constriction of tbe soft tissues of the base of tbe toe, and marked narrowing of tbe proximal pbalanx underlying the soft-tissue changes (Fig, 2), The following tests were all normal: chest film, complete blood count, SMA-12-60, sedimentation rate and complete urinalysis. Monthly intralesional injections of triamcinolone acetonide in a concentration of 5 mg/1 CC were used over a 10-montb period. Each injection consisted of 5 to 7,5 mg. Early in the course of treatment, near total ablation of tbe pain occurred. After 3 injections, resolution of tbe induration, increased pliability of tbe fibrous band, and decreased length of the fissure were noted. On bis last visit to our clinic 10 montbs later, the hyperkeratotic debris filling the groove had disappeared and the fissure was about half healed. The toe at tbat time was entirely asymptomatic.
Discussion
In 1860, Clarke^ described a disorder he called "dry gangrene" of the small toes affecting natives of the Gold Coast of Africa. This report is believed to be the first account of ainhum. The first recognized description of ainhum was reported by da Silva Lima'' from Bahia, Brazil. Cole'^ states that in 1863 Vernewil reported a disease of the toes affecting
Address for reprints: P, C, Anderson, M,D,, Division of Dermatology, University of Missouri Medical Center, Columbia, MO 65201,
379
380
INTERNATIONAL JOURNAL OF DERMATOLOGY
June 1976
Vol. 15
Many etiologic factors have been implicated in ainhum. Kean^^ gt al. suggested that the Negro may have a hereditary predisposition to the disease due to the fibrogenetic character present in some members of fhe race. Spinzig'-^ stated that this tendency was evoked by some mechanical or infectious injury. Other authors have attempted to implicate leprosy, yaws, syphilis, and fungi" as etiologic factors, but no one has isolated any organism from fhe lesions. The chigger Sarcopsytia penetrans was held as the
Fig. 1. The fissure, filled but distended with Keratin debris, accentuates the bulbous appearance of the toe.
fhe Nagos tribe in Brazil, who knevu the disease as "ainhum" or as "serrar," meaning "compression." The African (Yoruba) ferm "ainhum" or "Ayun" is derived from a word meaning "saw" or "file."7 Mafass believed that the term "ainhum" or "Ainhoum" originated from fhe Brazilian Negro patois meaning "fissure." Ofher syndromes for ainhum have been used, but the term dactylolysis spontanea is fhe mosf accurate description of the disease.2' » Ainhum has been reported from many different regions of the world, but in Nigeria, fhe presumed incidence of 2.48 per 1,000 males and 1.08 per 1,000 females is the greatest.!"' ^i Piftman,-^ in 1880, described the first case of ainhum in fhe United Sfafes in a Negro man. Subsequently, about 125 cases have been reported in the U.S., most originating in the South.
Fig. 2. Severe narrowing of the proximal phalanx is seen underlying the soft tissue groove.
No. 5
AINHUM • Rossiter and Anderson
responsible agent by Wellman,'5 but this case too involved previous trauma. Ainhum-like constricting bands, pseudoainhums, have also been found in association with other disease entities (Table 1). Wells and Robinson'^ have proposed 4 distinct etiologic groups for differentiating true ainhum and pseudoainhum. They are: (1) those conditions which may initiate annular scarring, such as laceration, burns, frostbite; (2) true ainhum; (3) constricting bands due to other lesions which sitnulate or occur concomitantly with ainhum; and (4) congenital bands. Another view would be to consider constricting bands as either congenital or acquired. Ainhum characteristically begins as a fissure or groove coinciding with the digitoplantar fold around the involved toe."' Fissural ulceration and induration frequently are present.'' Concomitant presence of a callous has been described.^^ Pain, a common feature, is quite variable in its intensity.fi Progression of the disease may be acute (months) or the terminal bloodless amputation may take years. Browne,'" in 1961, defined 4 clinical stages: (1) a crease which deepens into a sulcus at the digitoplantar fold, (2) extension of the sulcus around the base of the toe, (3) complete encirclement of a lifeless toe, and (4) autoamputation. Ainhum is accompanied by no known systemic manifestation nor is laboratory data of any assistance.^ Roentgenographic findings ranging from narrowing of the trabeculae with bone absorption adjacent to the groove early in the disease, to complete resorption in the latter stages of the disease, are characteristic.'" The microscopic picture is that of a reactive, markedly hyperkeratotic acanthotic epidermis, overlying a slight to moderate chronically inflamed, severely fibrosed dermis and hypodermis.''^
381
Table 1. Pseudoainhums Keratodermas—keratoderma heredltarium mutilans, mal de Maleda Pityriasis rubra pilaris Pachylnychia congenita Syringomyelia Infection—syphilis, leprosy Endocrine—diabetes melitus Connective tissue disease—scleroderma, morphea, Raynaud's disease Trauma—burn, frostbite
Amputation of the involved digit at or proximal to the fissure is considered to be the treatment of choice.^ Surgical approach using Z-plasty has also been used with very limited success.' Incision of the sclerosing band has also been attempted, but without success.' More recently, Nagabhushanam-'" and Tiwary-' in separate communications have reported the use of an intralesional cortisone injection in Stage I and II ainhums, and have noted temporary relief of pain and reduction of the associated induration. Because ainhum represents a progressive fibrosing process comparable to that seen in keloids—it arises most typically in Negroes and in response to trauma— the selection of intralesional steroids as a therapeutic modality seemed promising. The precise mechanism of action of steroids on altering fibrosis most likely relates to their well-known ability to inhibit both the inflammatory response and protein synthesis in cells. This speculation was supported in our case by prompt relief of pain and induration and later by increased pliability and healing of the fibrous band. Well-controlled clinical investigation should be undertaken to clarify the longterm efficacy of intralesional steroids in the treatment of constricting bands.
382
INTERNATIONAL JOURNAL OF DERMATOLOGY
However, at present, this approach offers a viable alternative to surgical intervention and is readily available to all regions of the world. References 1. Beeson, R. B. and McDermott, W., Cecil-Loeb Textbook of Medicine, 12th ed. Philadelphia, W. B. Saunders, 1963, p. 489. 2. Rook, A., Wilkinson, D. S. and Ebline, F. J. E., Textbook of Dermatology, 2nd ed. Oxford, Blackv^/ell Scientific Publications, 1972, pp. 1479-80. 3. Brown, E. C , Bilateral ainhum treated by multiple Z-plasties. Plast. Reconstr. Surg. 23:550, 1959. 4. Clarke, R., Remarks on the topography and diseases of the Gold Coast, West Coast of Africa. Trans. Epidemiol. Soc. (London) 1:76 1863. 5. da Silva Lima, J. F.: Ainhum. Eaz. Me'd. Bahia 1:146, 1867 (cited by Cole). 6. Cole, E. J., Ainhum, an account of 54 patients with special reference to etiology and treatment. J. Bone Joint Surg. 47B:43, 1965. 7. Leider, M. and Rosenblum, M., A Dictionary of Dermaloiogical Words, Terms and Phrases, 1st ed. New York, 1968, p. 25. 8. Matas, R., The surgical peculiarities of the Negro. Trans. Am. Surg. Assn. 14:483, 1896. 9. Bergner, L. H., and Winfield, J. M., Ainhum (dactyiolysis spontanea), review of the literature and report of a case. Am. J. Surg 100480,1960.
June 1976
Vol. 15
10. Browne, S. C , Ainhum, a clinical and etiological study of 83 cases. Ann. Trop. Med. Parasit. 55:314, 1961. 11. Browne, S. G., True ainhum, its distinctive and differentiating features. J. Bone Joint Surg. 47B:52, 1965. 12. Kean, B. H., Tucker, H. A. and Miller, W. C , Ainhum: clinical summary of forty-five cases on Isthmus of Panama. Trans. Roy Soc. Trop. Med. Hyg. 39:331, 1946. 13. Spinzig, E. W., Ainhum, its occurrence in the United States with a report of three cases. Am. J. Roentgenol. 42:246, 1939. 14. Davies, J. N. P., and Hewer, J. F.: Ainhum. Trans. Roy. Soc. Trop. Med. Hyg. 35:125, 1941. 15. Wellman, F. C , Criticism of some of the theories regarding the etiology of gounclou and ainhum. JAMA 46:636, 1906. 16. Wells, T. L., and Robinson, R. C. V., Annular constrictions of the digits, presentation of an interesting example. Arch. Dermatol. Syphilol. 66:569, 1952. 17. Snider: Ainhum. Arch. Dermatol. Syphilol. 20:139, 1929. 18. Fetterman, L. E., Hardy, R., and Lehrer, H., The clinicoroentgenologic features of ainhum. Am. J. Roentgenol. 100:512, 1967. 19. Kean, B. H., and Tucker, H. A., Etiologic concepts and pathologic aspects of ainhum. Arch. Pathol. 41-42:639, 1946. 20. Nagabhushanam, P., Ainhum, review of the literature and three case reports. Ind. J. Dermatol. Venereol. 37/4:146, 1967. 21. Tiwary, P. K.: Case report on ainhum. Ind. J. Dermatol. Venereol. 38/3:110, 1968.
A microscopial discovery, not less remarkable than that of Dr. Gruby, has been made during the present year, by Dr. Simon, of Berlin. It consists in the detection within the small mass of sebaceous substance which collects in the follicles of the skin, and becomes darkened at its extremity, and to which we give the name comedo, or grub, oi an articulated animalcule of considerable size.—Wilson, E., Introductory Lecture on the Opening of the Medical Session, 1842-43. Delivered at the Middlesex Hospital, Lancet 1:49, ' 1842-43.
AINHUM: TREATMENT WITH INTRALESIONAL STEROIDS JAMES W. ROSSITER, M.D, AND PHILIP C, ANDERSON, M,D.
ABSTRACT: Ainhum is a rare constricting band disease, usually affecting digits and resulting in spontaneous amputation. Prompt symptomatic relief and latter partial resolution of the fibrotic band followed intralesional steroid injection in the case presented.
Ainhum is a disease of dark-skinned people characterized by a groove encircling one or both small toes, resulting usually in spontaneous amputation.^ Involvement of other toes and fingers has been reported. Pain and mutilation caused by ainhum has been relieved by amputation or by reconstructive surgery.2' 3 |n addition to a review of the literature, a case of ainhum is presented. The patient's condition responded excellently to repeated intralesional injections of steroid in low doses with complete remission of pain and marked healing of the fissure around the toe. Case Report A 36-year-old Nigerian Negro man was seen in our Dermatology Outpatient Department because of pain in tbe fiftb toe of tbe rigbt foot of 3 years' duration. His private pbysician in Nigeria bad recommended surgical amputation some 8-12 months earlier, Witb the exception of tbe foot disorder, the patient had been in excellent health. He denied the presence of any similar disorder in family members. Examination of the fifth toe, right foot, revealed a groove extending from the plantar digital fold and encircling the toe to approximately 2/3 of
From the Division of Dermatofogy, University of Missouri Medical Center, Columbia, Missouri
its circumference (Eig, 1), Hyperkeratotic debris filled and distended tbe groove dorsomedially. Tbe segment of the digit distal to the fissure was bulbous and displayed normal neurological and vascular function. The toe was indurated and exquisitely tender. The remainder of the physical examination was unremarkable, Roentgenologic examination of tbe involved toe sbowed a narrow band-like constriction of tbe soft tissues of the base of tbe toe, and marked narrowing of tbe proximal pbalanx underlying the soft-tissue changes (Fig, 2), The following tests were all normal: chest film, complete blood count, SMA-12-60, sedimentation rate and complete urinalysis. Monthly intralesional injections of triamcinolone acetonide in a concentration of 5 mg/1 CC were used over a 10-montb period. Each injection consisted of 5 to 7,5 mg. Early in the course of treatment, near total ablation of tbe pain occurred. After 3 injections, resolution of tbe induration, increased pliability of tbe fibrous band, and decreased length of the fissure were noted. On bis last visit to our clinic 10 montbs later, the hyperkeratotic debris filling the groove had disappeared and the fissure was about half healed. The toe at tbat time was entirely asymptomatic.
Discussion
In 1860, Clarke^ described a disorder he called "dry gangrene" of the small toes affecting natives of the Gold Coast of Africa. This report is believed to be the first account of ainhum. The first recognized description of ainhum was reported by da Silva Lima'' from Bahia, Brazil. Cole'^ states that in 1863 Vernewil reported a disease of the toes affecting
Address for reprints: P, C, Anderson, M,D,, Division of Dermatology, University of Missouri Medical Center, Columbia, MO 65201,
379
380
INTERNATIONAL JOURNAL OF DERMATOLOGY
June 1976
Vol. 15
Many etiologic factors have been implicated in ainhum. Kean^^ gt al. suggested that the Negro may have a hereditary predisposition to the disease due to the fibrogenetic character present in some members of fhe race. Spinzig'-^ stated that this tendency was evoked by some mechanical or infectious injury. Other authors have attempted to implicate leprosy, yaws, syphilis, and fungi" as etiologic factors, but no one has isolated any organism from fhe lesions. The chigger Sarcopsytia penetrans was held as the
Fig. 1. The fissure, filled but distended with Keratin debris, accentuates the bulbous appearance of the toe.
fhe Nagos tribe in Brazil, who knevu the disease as "ainhum" or as "serrar," meaning "compression." The African (Yoruba) ferm "ainhum" or "Ayun" is derived from a word meaning "saw" or "file."7 Mafass believed that the term "ainhum" or "Ainhoum" originated from fhe Brazilian Negro patois meaning "fissure." Ofher syndromes for ainhum have been used, but the term dactylolysis spontanea is fhe mosf accurate description of the disease.2' » Ainhum has been reported from many different regions of the world, but in Nigeria, fhe presumed incidence of 2.48 per 1,000 males and 1.08 per 1,000 females is the greatest.!"' ^i Piftman,-^ in 1880, described the first case of ainhum in fhe United Sfafes in a Negro man. Subsequently, about 125 cases have been reported in the U.S., most originating in the South.
Fig. 2. Severe narrowing of the proximal phalanx is seen underlying the soft tissue groove.
No. 5
AINHUM • Rossiter and Anderson
responsible agent by Wellman,'5 but this case too involved previous trauma. Ainhum-like constricting bands, pseudoainhums, have also been found in association with other disease entities (Table 1). Wells and Robinson'^ have proposed 4 distinct etiologic groups for differentiating true ainhum and pseudoainhum. They are: (1) those conditions which may initiate annular scarring, such as laceration, burns, frostbite; (2) true ainhum; (3) constricting bands due to other lesions which sitnulate or occur concomitantly with ainhum; and (4) congenital bands. Another view would be to consider constricting bands as either congenital or acquired. Ainhum characteristically begins as a fissure or groove coinciding with the digitoplantar fold around the involved toe."' Fissural ulceration and induration frequently are present.'' Concomitant presence of a callous has been described.^^ Pain, a common feature, is quite variable in its intensity.fi Progression of the disease may be acute (months) or the terminal bloodless amputation may take years. Browne,'" in 1961, defined 4 clinical stages: (1) a crease which deepens into a sulcus at the digitoplantar fold, (2) extension of the sulcus around the base of the toe, (3) complete encirclement of a lifeless toe, and (4) autoamputation. Ainhum is accompanied by no known systemic manifestation nor is laboratory data of any assistance.^ Roentgenographic findings ranging from narrowing of the trabeculae with bone absorption adjacent to the groove early in the disease, to complete resorption in the latter stages of the disease, are characteristic.'" The microscopic picture is that of a reactive, markedly hyperkeratotic acanthotic epidermis, overlying a slight to moderate chronically inflamed, severely fibrosed dermis and hypodermis.''^
381
Table 1. Pseudoainhums Keratodermas—keratoderma heredltarium mutilans, mal de Maleda Pityriasis rubra pilaris Pachylnychia congenita Syringomyelia Infection—syphilis, leprosy Endocrine—diabetes melitus Connective tissue disease—scleroderma, morphea, Raynaud's disease Trauma—burn, frostbite
Amputation of the involved digit at or proximal to the fissure is considered to be the treatment of choice.^ Surgical approach using Z-plasty has also been used with very limited success.' Incision of the sclerosing band has also been attempted, but without success.' More recently, Nagabhushanam-'" and Tiwary-' in separate communications have reported the use of an intralesional cortisone injection in Stage I and II ainhums, and have noted temporary relief of pain and reduction of the associated induration. Because ainhum represents a progressive fibrosing process comparable to that seen in keloids—it arises most typically in Negroes and in response to trauma— the selection of intralesional steroids as a therapeutic modality seemed promising. The precise mechanism of action of steroids on altering fibrosis most likely relates to their well-known ability to inhibit both the inflammatory response and protein synthesis in cells. This speculation was supported in our case by prompt relief of pain and induration and later by increased pliability and healing of the fibrous band. Well-controlled clinical investigation should be undertaken to clarify the longterm efficacy of intralesional steroids in the treatment of constricting bands.
382
INTERNATIONAL JOURNAL OF DERMATOLOGY
However, at present, this approach offers a viable alternative to surgical intervention and is readily available to all regions of the world. References 1. Beeson, R. B. and McDermott, W., Cecil-Loeb Textbook of Medicine, 12th ed. Philadelphia, W. B. Saunders, 1963, p. 489. 2. Rook, A., Wilkinson, D. S. and Ebline, F. J. E., Textbook of Dermatology, 2nd ed. Oxford, Blackv^/ell Scientific Publications, 1972, pp. 1479-80. 3. Brown, E. C , Bilateral ainhum treated by multiple Z-plasties. Plast. Reconstr. Surg. 23:550, 1959. 4. Clarke, R., Remarks on the topography and diseases of the Gold Coast, West Coast of Africa. Trans. Epidemiol. Soc. (London) 1:76 1863. 5. da Silva Lima, J. F.: Ainhum. Eaz. Me'd. Bahia 1:146, 1867 (cited by Cole). 6. Cole, E. J., Ainhum, an account of 54 patients with special reference to etiology and treatment. J. Bone Joint Surg. 47B:43, 1965. 7. Leider, M. and Rosenblum, M., A Dictionary of Dermaloiogical Words, Terms and Phrases, 1st ed. New York, 1968, p. 25. 8. Matas, R., The surgical peculiarities of the Negro. Trans. Am. Surg. Assn. 14:483, 1896. 9. Bergner, L. H., and Winfield, J. M., Ainhum (dactyiolysis spontanea), review of the literature and report of a case. Am. J. Surg 100480,1960.
June 1976
Vol. 15
10. Browne, S. C , Ainhum, a clinical and etiological study of 83 cases. Ann. Trop. Med. Parasit. 55:314, 1961. 11. Browne, S. G., True ainhum, its distinctive and differentiating features. J. Bone Joint Surg. 47B:52, 1965. 12. Kean, B. H., Tucker, H. A. and Miller, W. C , Ainhum: clinical summary of forty-five cases on Isthmus of Panama. Trans. Roy Soc. Trop. Med. Hyg. 39:331, 1946. 13. Spinzig, E. W., Ainhum, its occurrence in the United States with a report of three cases. Am. J. Roentgenol. 42:246, 1939. 14. Davies, J. N. P., and Hewer, J. F.: Ainhum. Trans. Roy. Soc. Trop. Med. Hyg. 35:125, 1941. 15. Wellman, F. C , Criticism of some of the theories regarding the etiology of gounclou and ainhum. JAMA 46:636, 1906. 16. Wells, T. L., and Robinson, R. C. V., Annular constrictions of the digits, presentation of an interesting example. Arch. Dermatol. Syphilol. 66:569, 1952. 17. Snider: Ainhum. Arch. Dermatol. Syphilol. 20:139, 1929. 18. Fetterman, L. E., Hardy, R., and Lehrer, H., The clinicoroentgenologic features of ainhum. Am. J. Roentgenol. 100:512, 1967. 19. Kean, B. H., and Tucker, H. A., Etiologic concepts and pathologic aspects of ainhum. Arch. Pathol. 41-42:639, 1946. 20. Nagabhushanam, P., Ainhum, review of the literature and three case reports. Ind. J. Dermatol. Venereol. 37/4:146, 1967. 21. Tiwary, P. K.: Case report on ainhum. Ind. J. Dermatol. Venereol. 38/3:110, 1968.
A microscopial discovery, not less remarkable than that of Dr. Gruby, has been made during the present year, by Dr. Simon, of Berlin. It consists in the detection within the small mass of sebaceous substance which collects in the follicles of the skin, and becomes darkened at its extremity, and to which we give the name comedo, or grub, oi an articulated animalcule of considerable size.—Wilson, E., Introductory Lecture on the Opening of the Medical Session, 1842-43. Delivered at the Middlesex Hospital, Lancet 1:49, ' 1842-43.
