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Leg ulcerations: a clinical appraisal

European Journal of Dermatology. Volume 15, Number 3, 127-32, May-June 2005, Continuing medical education

Summary

Author(s) : Jean-Marie Bonnetblanc, Service de dermatologie, CHRU Dupuytren, 2 avenue Martin Luther King, 87042 Limoges cedex France.

Summary : Leg ulcerations are frequent and often require dermatological advice. Many typical ulcerations may be recognized quite easily by inspection. Through a series of clinical examples, clinical diseases that may have ulceration of the leg as an initial presentation are illustrated. The figures of the article may be looked at first, without reading the legend, so the reader may suggest diagnostic hypotheses before discovering the true diagnosis.

Keywords : leg ulceration, diagnosis

Pictures

ARTICLE

Auteur(s) :, Jean-Marie Bonnetblanc^*

Service de dermatologie, CHRU Dupuytren, 2 avenue Martin Luther King, 87042 Limoges cedex France

accepté le 9 Février 2005

Test yourself

8 clinical cases are presented (figures 1 and 2). First, look at the picture, the age and sex of the patient to search for clinical items that evoke one or more diagnostic hypotheses. Then read the short vignette on the right and complete or shorten the list of your diagnostic hypotheses. Write down your diagnostic hypotheses and the clinical criteria that helped you reach it. The answers can be found in table 2( Table 2 ).

Leg ulcerations are frequent and often require dermatological advice [1-3]. The term “ulcer” is used when referring to a chronic ulceration. In these cases a vascular ætiology is most often encountered, leading to a diagnosis of vascular ulcer, comprising venous ulcer, arterial ulcer, mixed ulcer or necrotic angiodermatitis (hypertensive ulcer). But sometimes the dermatologist is struck by an unusual sign or symptom, or by an unusual feature of the context, leading to another cause for the ulceration. The eye of the dermatologist may be sufficient to suggest a good diagnosis, mainly based on clinical grounds. The subject of this article is to propose and illustrate clinical features for most of the causes of leg ulcerations.
Table 2 Answers to the “test yourself”

Figure 1A. Female, 68-years-old. Treated for hypertension. The ulcer appeared after minor trauma. Local pain was severe	At a glance, the necrotic livedoid periphery of the ulcer suggests necrotic angiodermatitis, periarteritis nodosa, cryoglobulinæmia or cholesterol embolism. The context highly suggests necrotic angiodermatitis
Figure 1B. Female, 67-years-old. Chronic ulcer of 2 years duration, painless, seated between the inferior and middle thirds of the leg. Arterial pulses were palpated	The bottom of the ulceration has abnormal and irregular granulation suggesting a malignancy. The site is unusual. A biopsy confirmed an epidermoid carcinoma.
Figure 1C. Female, 31-years-old. She had no vascular abnormalities. She had recently been depressed. Some papules and pustules were observed on her face.	The vegetating bottom and the inflammatory edge suggest pyoderma gangrenosum or drug-induced ulceration. Distant skin lesions and the context of depression led to halogenoderma.
Figure 1D. Female, 54-years-old. The ulceration appeared recently and rapidly extended. She had no digestive and no articular signs or symptoms	The clinical picture is typical of pyoderma gangrenosum. A mycobacterial infection must be excluded.
Figure 2A. Male, 72-years-old. He had had a chronic ulcer of more than 5 years duration, which became painful. Pressure of the edge revealed a white paste.	The problem is an ulcer that doesn’t cure. The infiltrated and sharp edge with the irregular bottom suggest a malignancy. The “white paste” at pressure is more characteristic of verrucous carcinoma.
Figure 2B. Male; 83-years-old. Six months old grafted ulcers, but ulceration recurred from the edges. He had no varicose veins. Peripheral pulses were difficult to palpate.	The recurrence from the edge of the ulcer is indicative of an infection or a malignancy. The ulcers are too round or oval for the second hypotheses. Here the faint arterial pulses are misleading in an old patient. Tuberculosis was the diagnosis.
Figure 2C. Male, 42-years-old. He presented with a chronic ulcer. It was the second relapse. Venous incompetence was present. He had not had phlebitis.	The clinical aspect is that of a venous ulcer. It occurred in a young man and had a chronic course. A Klinefelter syndrome must be searched for.
Figure 2D. Male 21-years-old. Student in France, he presented with a chronic ulcer. It was the second recurrence	This ulcer appeared in a young man with black skin. Hæmoglobin abnormalities must be investigated, particularly with the chronicity. Tropical ulcer is more phagedenic.

Basic clinical aspects of leg ulcers of vascular origin

Venous and arterial ulcers are the most frequent and they represent more than 95% of leg ulcers. Their diagnosis is often easy. They occur in middle aged patients for venous ulcers and in older patients for arterial ulcers. Venous ulcers are situated above the medial malleola, are usually large and painless. In the post-thrombotic syndrome, dermatoliposclerosis is more frequently observed and ulcers are often very large, all around the leg. An edema is also observed under and above the ulcer in a typical case. Most often, post-thrombotic ulcers are identical to venous ulcers with venous insufficiency. Arterial ulcers are painful and acral, involving the dorsum of the feet, of the toes and the areas between the toes. Necrotic areas are observed, and there may be involvement of the underlying joint or bone, provoked by the deformities observed in aged patients. Necrotic angiodermatitis (hypertensive ulcer) is typically on both legs and is extremely painful. Ulcerations extend along the pattern of a necrotic livedo ( (figure 1A) ). Plantar ulceration is distinct from leg ulcer. It shares common but also some specific aetiologies and requires complementary investigations.

Clinical features of leg ulcerations

Leg and foot ulcerations have numerous other causes. Most of the aetiologies may mimic venous or arterial ulcers but a careful analysis of dermatological signs and symptoms may be relevant to diagnose these “non vascular” causes. The expert eye of the dermatologist is often sufficient to suggest another diagnosis than that of “vascular” leg ulcer, but no study has demonstrated its sensitivity and specificity. The following items are proposed and illustrated to determine what is not a venous or an arterial ulcer (table 1)( Table 1 ).
Table 1 Items of clinical diagnosis of leg ulcerations

A-Ulceration

Site

Number

Pain

Size

Bottom

Edge

Peripheral skin

Arterial pulses

Associated signs and/or symptoms

Neurological

Bone

Distant skin

Or lack of precedent items

B-Context

Ulcer that doesn’t cure

Age

Evolution (acute, chronic, recurrence)

Geographical area

Trauma

Black skin

Sex

Treatment (iatrogenic ulceration)

First set of criteria

This is linked to the clinical aspects of the ulceration.

The site of the ulceration may be unusual and be higher on the leg. This is observed in some aetiologies such as carcinomas ( (figure 1B) ). One or few ulcerations are usually observed. A great number of ulcerations is rarely present but it may indicate infectious ulcerations such as ecthyma or atypical mycobacteria. Pain is encountered in arterial ulcer and is an argument for the diagnosis of necrotic angiodermatitis. Pain is also a criteria of infection [4]. It may reveal neurological or bone associated disorder. The size of the ulcer is probably not very discriminatory in the differential diagnosis. It may be due to rapid centrifugal evolution as in pyoderma gangrenosum, or conversely to chronic recurrent venous ulcer. Some neglected patients may present with very large ulcers. Old ulceration may evolve into carcinoma.

The bottom of the the ulcer may vary in color. A black one may reveal melanoma. A blue-black bottom is indicative of Pseudomonas infection ( (figure 3A) ), but it usually gives a blue-green color as a hallmark of bacterial colonization. The granulomas may be irregular in size as observed in squamous cell carcinoma ( (figure 1B) ). A tumor may fill all the ulceration, as in B cell lymphoma of the leg ( (figure 3B) ). Vegetating bottom may be observed in carcinoma and in halogenoderma ( (figure 1C) ). Bleeding evokes polycythemia. The edges may vary in color such as black (melanoma, necrosis [5], violet (pyoderma gangrenosum, polyarteritis nodosa), or may be undermined ( (figure 1D) ), irregular or too regular ( (figure 3C) ) or infiltrated. Pressure of the edge leads to keratin filaments in verrucous carcinoma ( (figure 2A) ). A figurated edge is observed after trauma or caustic contact ( (figure 3D) ). Round or oval edges are rather typical of tuberculosis ( (figure 2B) )[6]. The periphery of the ulcer is usually cicatricial, sclerous, red or eczematous. Atrophy is observed in white atrophy but it may be part of necrobiosis lipoidica. Purpura often evolves towards dermite ocre, but ecchymosis is observed in amyloidosis or scurvy ( (figure 4A) )[7]. Livedo may be inflammatory in periarteritis nodosa ( (figure 4B) ) or in cholesterol embolism. Pustules are suggestive of an infectious origin. Ulcerated plaques, nodosities or tumors are suggestive of malignancy or panniculitis.

In some patients with skin necrosis, palpable arterial pulses are observed in infection, necrotic angiodermatitis, or vasculitis. In old patients absence of the pulse is not always indicative of an arterial origin and may be a false negative sign ( (figure 2B) ). Attention must be drawn to skin sensitivity or pain, and to bone deformities ( (figure 4C) )[8]. It is a rule that the skin of all the patients must be examined even if the vascular origin of the ulcer is obvious. In some cases distant cutaneous signs and/or symptoms may help ( (figure 1C) ).

Second set of criteria

These are linked to the context.

The first and the most frequent sign of an atypical ulcer is an “ulcer that doesn’t cure”. This is a frequent comment. There is, however, no definite time scale to decide that such an ulcer should be investigated. This must be decided after ruling out the usual causes of delay to healing, i.e. mixed venous and arterial ulcers, associated metabolic disorder (diabetes), denutrition, particularly in older patients, unsuitable topical treatment, non compliance of the patient, or personal benefit for the patient to be followed in a clinic. In most reviews, a delay of 2 to 3 months is acceptable to diagnose a late cicatrisation.

Leg ulcers frequently appear after 40 years. A young age may be observed in some venous ulcers, but a diagnosis of Klinefelter syndrome ( (figure 2C) ), and, rarely, other genetic syndromes, must be evoked. Venous insufficiency is present in Klinefelter syndrome. It has been hypothezised that hypogonadism is associated with leg ulcers but the proof is indirect. In younger patients (before the age of 20), enzymatic defects should be investigated, particularly prolidase deficiency, with which a dysmorphy is associated. Other enzymatic defects have been described, possibly responsive to corticosteroids. In black patients, abnormal haemoglobin must be investigated, particularly drepanocytosis ( (figure 2D) )[9].

Leg ulcers frequently have a chronic and disabling course with recurrence. Rapidly evolving ulceration is typical of pyoderma gangrenosum, but it is also observed in other vasculitis and in infections.

Another fact to consider is linked to the geographical area where the patient is examined or is coming from. Tropical areas shared specific ætiologies, mainly leprae, Buruli ulcers, and filariosis.

Trauma is a cause of ulceration, whatever the site injured. However a minor trauma may be the cause of an unusually large ulceration. In children, epidermolysis bullosa or Ehlers Danlos disease are diagnosed in such situations, and in adults, skin fragility secondary to long term treatment with corticosteroids ( (figure 4D) ).

Black skinned patients may have leg ulceration linked to geographical area, as well more frequent haemoglobin genetic disorders.

Sex is probably a weak criteria, but a young male patient with a leg ulcer must have a caryotype investigation to search for Klinefelter syndrome ( (figure 2D) ).

Finally, a iatrogenic cause must be searched for, such as immunosuppressants (hydroxyurea, methotrexate, …) or other (barbiturates, …) [10].

Discussion

When a new patient presents with a leg ulcer, the most frequent types of ulcer come first to mind i.e. of vascular origin. Some atypical clinical signs or symptoms may alert, such as the age of the patient or the age of the start of leg ulceration. Other clinical items linked to the ulcer may also draw attention and the classification proposed here may help the clinician to identify another infrequent or rare cause. The clinical aspect of the ulceration is best observed by the eye of the clinician, which is the first instrument in suggesting new hypothesis.

In the list of clinical items, a sign or a symptom may be present on its own or associated with other signs and/or symptoms, as demonstrated by many of the figures. An increasing number of signs and/or symptoms might lead to more or less diagnostic hypotheses, but this has not been measured.

Finally, with this scheme, various diseases may be diagnosed. Although infrequent or rare diseases probably represent less than 5% of leg ulcerations, some of them are important to diagnose early, particularly infections and malignancies. Treponematoses, mycobacterial or deep fungal infections may be difficult to identify and a biopsy culture is often relevant. If carcinomas are easier to recognize, verrucous carcinoma is often diagnosed after a long period of follow-up. Pyoderma gangrenosum is more easily recognized if rapid peripheral skin destruction with its violaceous hue is present, but it is sometimes more chronic.

This scheme, which is often used in an unconscious way in a hypothetico-deductive process in the clinical assessment of leg ulceration, may help clinicians involved in the care of leg ulcers. These typical figures of many aetiologies of leg ulcerations may help in diagnosing atypical aspects or rare diseases.

References

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2 Westerhoff W. Leg ulcers. Diagnosis and treatment. Amsterdam: Elsevier, 1993.

3 Hafner J, Ramelet AA, Schmeller W, Brunner U. Management of leg ulcers. Current Problems in Dermatology. Basel: Karger, 1999.

4 Douglas WS, Simpson NB. Guidelines for the management of chronic venous leg ulceration. Report of a multidisciplinary workshop. Br J Dermatol 1995; 132: 446-52.

5 Combemale P, Amiral J, Estival JL, Dupin M, Chouvet B, Berruyer M. Cutaneous necrosis revealing the coexistence of an antiphospholipid syndrome with acquired protein S deficiency, factor V Leiden and hyperhomocysteinemia. Eur J Dermatol 2002; 12: 278-82.

6 Cecchi R, Giomi A, Innocenti F. Scrofuloderma (tuberculosis colliquativa cutis) of the left foot. Eur J Dermatol 1998; 8: 67-8.

7 Boulinguez S, Bouyssou-Gauthier ML, De Vençay P, Bédane C, Bonnetblanc JM. Scorbut révélé par un purpura ecchymotique et des ulcères hémorragiques des membres inférieurs. Ann Dermatol Venereol 2000; 127: 510-2.

8 Mansour J, Descamps V, Hayem G, Crickx B. Placard inflammatoire ulcéré de jambe. Ann Dermatol Venereol 2004; 131: 835-6.

9 Delpuget-Bertin N, Bédane C, Bouyssou-Gauthier ML, Vo Thi NH, Bonnetblanc JM. Cas pour diagnostic. Ann Dermatol Venereol 1998; 125: 735-6.

10 Friedrich S, Raff K, Landthaler M, Karrer S. Cutaneous ulcerations on hands and heels secondary to long-term hydroxyurea treatment. Eur J Dermatol 2004; 14: 343-6.