Dactylolysis spontanea là gì

An unusual cause ofulceration: ainhum(dactylolysis spontanea)Timothy Jemmott, Alethea V Foster, Michael E Edmonds

Jemmott T, Foster AV, Edmonds ME. An unusual cause of ulceration: ainhum (dactylolysis spontanea). Int Wound J2007;4:251254.

ABSTRACTWe describe a case of ainhum, a mutilating condition, usually seen in peoples of West African origin. A patientpresented at the Diabetic Foot Clinic, Kings College Hospital, with a painful infected ulcer between the fourth andfifth toe of his right foot. He was a 43-year-old Nigerian and did not have diabetes. He reported that his left fifthtoe had autoamputated some months before. Radiograph of the right fifth toe showed absorption of the proximaland intermediate phalanges, and a diagnosis of ainhum was made. Ainhum or dactylolysis spontanea is a rarecondition of unknown aetiology in which a groove or fissure of constricting tissue forms around the proximal endof the fifth toe. Eventually, the groove extends to encircle the toe, the underlying structures are absorbed and thetoe autoamputates. It may present as chronic fissuring at the base of the fifth toe or as foot ulceration. Thiscondition is seldom seen in the United Kingdom, but it is likely that this condition is underdiagnosed. It isimportant that wound care specialists be aware of the diagnosis.

Key words: Ainhum (dactylolysis spontanea) Bone absorption Constricting annular bands Foot ulcer Toe autoamputation

INTRODUCTIONWe describe a case of ainhum, a mutilating

condition, usually seen in peoples of West

African origin. The origins of the term ainhum

are unclear. It was first used in a published

account by da Silva Lima in 1867 (1). It has

been suggested that the term ainhum derives

from the Nago word (Brazil) meaning fissure

or the Yoruba word (Nigeria) meaning to saw

(1,2). Ainhum or dactylolysis spontanea is a

rare condition of unknown aetiology, in which

a groove or fissure of constricting tissue forms

around the proximal end of the fifth toe.

Eventually, the groove extends to encircle the

toe, the underlying structures are absorbed

and the toe autoamputates.

It is seldom seen in the United Kingdom (1).

CASE STUDY

HistoryA 43-year-old man from Nigeria, West Africa,

presented in the Diabetic Foot Clinic on 3

November 2005, seeking treatment for a painful

interdigital ulcer on his right foot. He had been

referred via the orthopaedic clinic from his

general Practitioner. The patient was fit and

well, with no medical history of diabetes,

ischaemic heart disease, peripheral vascular

disease or skin conditions. He is a non smoker.

The patient reported that the fifth toe of his left

foot had also developed a similar groove. This

subsequently had developed into an encircling

constricting band, and the toe autoamputated

in September 2005 (Figures 1a, 1b). It was

unclear from the history how long this process

had taken.

There was no family history of this condition.

Key Points

ainhum is a disease of blackpeople of West Africa

ainhum or dactylolsis spontaneais a rare condition of unknownaetiology in which a groove is orfissure of constricting tissueforms around the proximal endof the fifth toe

eventually, the groove extendsto encircle the toe, the under-lying structures are absorbedand the toe autoamputates

Authors: T Jemmott, DPodM, Diabetic Foot Clinic, KingsCollege Hospital, NHS Trust, Denmark Hill, London; AV Foster,BA(Hons) PGCE DPodM, Diabetic Foot Clinic, Kings CollegeHospital, NHS Trust, Denmark Hill, London; ME Edmonds, MDFRCP, Diabetic Foot Clinic, Kings College Hospital, NHS Trust,Denmark Hill, LondonAddress for correspondence: T Jemmott, Diabetic FootClinic, Kings College Hospital, NHS Trust, Denmark Hill,London. SE5 9RS, UKE-mail: [email protected]

CASE STUDY

2007 The Authors. Journal Compilation 2007 Blackwell Publishing Ltd and Medicalhelplines.com Inc International Wound Journal Vol 4 No 3 251

ExaminationHe presented with an infected ulcer in the

interdigital space at the base of the fifth toe of

his right foot and a spreading cellulitis. The

foot was hot, swollen and painful, and the

wound was discharging pus. The fissure-like

lesion was deep and encompassed approxi-

mately 50% of the circumference of the toes

medial aspect. Pedal pulses were strong and

palpable. He had no peripheral neuropathy

with a normal vibration perception threshold

of 15 volts at the apex of the first toe, bilaterally.

InvestigationA radiograph of the right foot showed absorp-

tion of the bones of the proximal and interme-

diate phalanges and constriction of the soft

tissues of the fifth toe (Figure 2a).

Treatment and progressIn view of the severity of our patients foot

infection, we treated him aggressively with the

following antibiotics: ceftriaxone 1 g IM o.d.,

amoxycillin 500 mg t.d.s., flucloxacillin 500 mg

q.d.s. and metronidazole 400 mg t.d.s. orally. A

swab subsequently grew ++mixed anaerobes

sensitive to metronidazole.

By the 10 November 2005, the pain had di-

minished, and the wound was improving. A

further swab was taken, which subsequently

grew anaerobic streptococcus sensitive to

metronidazole. He was given a further fol-

low-up appointment for 2 weeks time, but

he failed to attend.

The patient attended the clinic again at our

request in March 2006. He said he did not

come to his previous appointment because his

toe had healed and was no longer painful. At

this time, the foot was intact, but there was

a groove at the base of the fifth toe (Figure 2b),

where the previous tissue deficit had epithe-

lialised. The lateral circumference of the toe

appeared to be normal, but the toe was

becoming slightly bulbous (Figure 3). He was

advised to return to clinic if he suffered from

further ulceration or infection of the toe. It was

also explained to him that prognosis for the toe

was poor; the toe would eventually autoam-

putate as the left fifth toe had done, but that

the condition was self-limiting.

DISCUSSIONThis report describes an uncommon cause of

ulceration in a UK foot clinic. It is a rareFigure 1. (A) Autoamputated fifth toe of left foot. (B)

Radiograph of autoamputated left fifth toe.

Figure 2a. (A) Radiograph showing absorption of proximal

and intermediate phalangeal bones of right fifth toe. (B) Groove

in the medial aspect of right fifth toe.

Key Points

in view of the severity of thepatients foot infection, wetreated him aggressively withantibiotics

the patient was advised thatthe right fifth toe was bulbousand would eventually auto-amputate as the left foot haddone and was asked to returnto the clinic if he suffered fromfurther ulceration and infectionof the toe

Cause of ulceration: ainhum

252 2007 The Authors. Journal Compilation 2007 Blackwell Publishing Ltd and Medicalhelplines.com Inc

condition of unknown aetiology, and it is

important for wound care specialists to be

aware of it.

Our patient was unclear in his history of

how long the condition had taken to develop.

The course of the disease is variable, and it has

been suggested that extension of the groove to

completely encircle the toe can take between 3

months and over 20 years (2). The time taken

for autoamputation to occur, however, is on

average about 5 years (2). The natural history of

ainhum can be described in four stages (24):

stage 1a groove or fissure forms on the

medial plantar aspect of the base of the fifth

toe; stage 2over time the groove extends to

encircle the toe with a band of constricting

tissue, the groove gets deeper and often ulcer-

ates, the toe may become bulbous; stage 3the

constricting band encircling the toe compresses

the soft tissue and bone beneath it, the bones

are absorbed, and eventually fracture; stage

4ultimately, the toe autoamputates. In our

patient, the disease was bilateral. Ainhum is

normally bilateral. In a series of 100 patients,

75%haddisease in both feet (4). It is usually seen

in men and women between the ages of 30 and

50. Early studies of ainhum had a male pre-

ponderance as it was likely males were inclined

to seek treatment more often than females (4).

Our patient also presented with severe pain

and infection. In a previous series, pain was

a severe complication in 44 out of 45 patients

requiring amputation of the affected toe (5).

Generally, ainhum is painless in the absence of

secondary infection, although there are some

reports that suggest there may be some pain in

the early stages due to compression of the nerve

by the constricting band (2). Chronic fissuring,

ulceration and infection are well-described

complications of this condition and are the

cause of pain, which often brings the patient to

the practitioner (25). It is important to treat

any infection aggressively as in our case.

Radiographs of our case showed absorption

of the bones of the proximal and intermediate

phalanges and constriction of the soft tissue at

the base of the fifth toe. These changes are in

agreement with the reports in the literature.

These radiographic changes have been used

for the purposes of diagnosis. A study of 6000

radiographs used a staging system based on

the one devised by Cole to diagnose 102 cases

of ainhum between 1977 and 1999 (6). Angi-

ography of four patients with ainhum showed

the posterior tibial artery stopped at the ankle,

and the plantar arch and its branches were

absent, and concluded that a vascular abnor-

mality may be a contributory factor in the

aetiology of ainhum (7). However, dorsalis

pedis and posterior tibial pulses were palpable

in our patient.

Histology was not obtained in our case, but

previous studies have shown changes in the

dermis and epidermis. A study of histological

changes showed hyperkeratosis and acantho-

sis of the epidermis. Lymphocytes (T-cells and

plasma cell) and fibroblasts were present in the

dermis and were there in, probably, a response

to tissue damage and the chronic inflammation

of the wound-healing process (8). The con-

stricting band was composed of collagen,

which was laid down at right angles to the

ligamentous and capsular structures of the toe.

This band of fibrosed tissue contracts as it

would in a scar, so forming a ligature around

the toe, causing additional localised anoxia

and necrosis that restarts the wound repair

process leading to further fibrosis. The events

starting the process, which cause the constrict-

ing band to form, are not known.

Our patient was a 43-year-old Nigerian. da

Silva Lima described ainhum as a disease

particular to Ethiopian peoples in 1867 (2).

Since then, it has been described in many

races, but ainhum is usually seen in people of

tropical African origin and their descendants

in North America, Panama, Brazil and the

West Indies. In Africa, the incidence of

ainhum is between 02% and 20% of thepopulation. A study by Cole (2) in 1965 in

Ibadan Nigeria notes an incidence as high as

22%. He inspected the feet of a series of 1000patients who consented to a foot examina-

tion, and 22 cases of ainhum were identified

(2). A larger study in 1961 in the Belgium

Key Points

generally, ainhum is painless inthe absence of secondary infec-tion, although there are somereports that suggest there maybe some pain in the earlystages due to compression ofthe nerve by the constrictingband

Figure 3. Right fifth toe becoming slightly bulbous in and

groove in the medial aspect.

Cause of ulceration: ainhum

2007 The Authors. Journal Compilation 2007 Blackwell Publishing Ltd and Medicalhelplines.com Inc 253

Congo of 45 000 subjects reported an inci-

dence of 02% (3,4). In Panama in 1946, therewas an incidence of only 0015%; the subjectsof this study were all of West Indian origin.

There are many causes of foot ulceration

with clinical presentations similar to the one

that we have described. Hence, there are many

potential differential diagnoses for ainhum,

e.g. leprosy, syphilis, yaws (framboesia), dia-

betes, spina bifida, torque syndromes, where

a fibre or a hair becomes bound around a toe,

or pseudoainhum. Pseudoainhum is a term

given to congenital amniotic bands and other

diseases (9), e.g. scleroderma, morphea, lamel-

lar ichthyosis, chronic psoriasis, keratoderma

hereditarium mutilans, discoid lupus erythe-

matosus, Raynauds syndrome, in which con-

stricting annular bands occur. These conditions

are not usually limited to just the fifth toes or

peoples of tropical African descent.

We treated our patient for his wound infec-

tion with antibiotics that gave him relief from

his symptoms. Several treatments have been

suggested for ainhum: local injection with

corticosteroids (10), topical application of

salicylic acid preparations (8) and surgical

excision of the groove with a Z-plasty pro-

cedure (2). These treatments attempt to disrupt

the formation of the fibrous band before the

bone becomes involved. There is, however, no

evidence to suggest that any of these treat-

ments will halt the progression of the disease

once the bone is involved as it was in our case.

Amputation of the toe is also a well-described

treatment for this condition (15).

A literature search on PubMed elicited 115

references, of these, only 20 were published in

the last 10 years. Fourteen of these papers were

about pseudoainhum, one was about an atypical

case of ainhum in the big toe of a Caucasian

Italian man (11), and the remaining five

concerned ainhum. There were no reports or

papers from the United Kingdom in the last 10

years. Reports of ainhum are rare in the United

Kingdom. In total, we found only three cases

reported in United Kingdom: a Negro in

Bristol 1941 (12); a Nigerian in Manchester

in 1957 (1) and a case in a black man in London

1993 (13). Auckland et al. (1) argued in 1957

that due to the increase in immigration of Afro-

Caribbean people, ainhum would be seen

much more frequently in the United Kingdom.

However, there has not been an increase in

cases reported in the literature. It is not

possible to determine the prevalence of ain-

hum in the United Kingdom from the litera-

ture, but it is likely that this condition is

underdiagnosed, and it is important for

wound care specialists to be aware of the

diagnosis. Once aware of the condition, it is

straightforward to diagnose it, and to inform

and explain to the patient its natural history

and self-limiting nature.

REFERENCES1 Auckland G, Ball J, Griffiths DL. Ainhum. J Bone

Joint Surg Br 1957;39-B:5139.

2 Cole GJ. Ainhum: an account of fifty-four patients

with special reference to etiology and treatment.

J Bone Joint Surg Br 1965;47:4351.

3 Brown SG. A clinical and etiological study of 83

cases. Ann Trop Med Parasitol 1961;55:3149.

4 Brown SG. True Ainhum: its distinctive and differ-

entiating features. J Bone Joint Surg Br 1965;47:525.

5 Kean BH, Tucker HA, Miller WC. Ainhum: A clinical

summary of forty-five cases on the Isthmus of

Panama. Trans R Soc Trop Med Hyg 1946;39:3314.

6 Daccarett M, Espinosa G, Rahimi F, Eckerman CM,

Wayne-Bruton S, Couture M, Rosenblum J.

Ainhum (dactylolysis spontanea): a radiological

survey of 6000 patients. J Foot Ankle Surg 2002;

41:3728.

7 Dent DM, Fataar S, Rose AG. Ainhum and angio-

dysplasia. Lancet 1981;2:3967.

8 Kerhisnik W, ODonnell E, Wenig JA, McCarthy DJ.

The surgical pathology of ainhum (dactylolysis

spontanea). J Foot Surg 1986;25:95123.

9 Graham RM, James MP. Pseudo-ainhum, angiodys-

plasia and focal acral hyperkeratosis. J R Soc Med

1985;78 Suppl. 11:135.

10 Rossiter JW, Anderson PC. Ainhum: treatment with

intralesional steroids. Int J Dermatol 1976;15:37982.

11 Olivieri I, Piccirillo A, Scarano E, Ricciuti F,

Padula A, Molfese V. Dactylolysis spontanea or

ainhum involving the big toe. J Rheumatol 2005;

32:24379.

12 Damir JNP, Hewer TF. Ainhum: A report of a case in

England. Trans R Soc Trop Med Iyg 1941;35:125.

13 Hunt M, Glucksman EE. Ainhum presenting at the

accident and emergency department. Arch Emerg

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Key Points

this condition is underdiag-nosed, and it is important forthe wound care specialist to beaware of the diagnosis and toexplain to the patient itsnatural history and self limitingorigin

Cause of ulceration: ainhum

254 2007 The Authors. Journal Compilation 2007 Blackwell Publishing Ltd and Medicalhelplines.com Inc