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Painful Lower Extremity Ulcerations with Hyperpigmentation

 

Am Fam Physician. 2020 Feb 1;101(3):177-178.

A 25-year-old woman presented with ulcerations of the lower extremities that developed one week earlier in the setting of hyperpigmentation over two to three years. She initially noticed multiple angular-shaped, erythematous nodules on the distal lower extremities that became hyperpigmented patches and plaques. The patient reported pain and itching in the involved areas. She had no history of diabetes mellitus or other chronic illnesses.

Physical examination revealed multiple tender, superficial ulcerations with surrounding erythema and hyperpigmentation on the medial aspect of the bilateral ankles and dorsum of her right foot (Figure 1). She also had an atrophic, white, stellate scar proximal to her right third and fourth metatarsophalangeal joints. Numerous purpuric patches and nodules were noted throughout the distal lower extremities. Her physical examination was otherwise unremarkable, and her vital signs were normal.

FIGURE 1.


FIGURE 1.

Question

Based on the patient's history and physical examination findings, which one of the following is the most likely diagnosis?

A. Chronic venous insufficiency.

B. Cutaneous leukocytoclastic vasculitis.

C. Erythema nodosum.

D. Livedoid vasculopathy.

E. Pyoderma gangrenosum.

Discussion

The correct answer is D: livedoid vasculopathy. Livedoid vasculopathy is a chronic thromboocclusive cutaneous disorder marked by recurrent skin findings affecting the lower extremities. Characteristic clinical features are the initial appearance of tender or pruritic erythematous nodules, followed by superficial ulcerations and atrophic ivory-white scars with surrounding hyperpigmentation known as atrophie blanche.1 Ankle edema and peripheral purpura or petechiae may precede skin manifestations. Physical findings are often bilateral and most commonly observed on the distal lower extremity, ankle, or dorsal foot.2 Ulcerations are often painful and may persist for months despite treatment.

Livedoid vasculopathy most commonly presents in women with bimodal peaks at 45 to 50 and 70 to 75 years of age.1 Clinical findings are caused by the occlusion of cutaneous blood vessels

Address correspondence to Erik Kumetz, MD, at erik.kumetz@fe.navy.mil. Reprints are not available from the authors.

Author disclosure: No relevant financial affiliations.

References

show all references

1. Micieli R, Alavi A. Treatment for livedoid vasculopathy: a systematic review. JAMA Dermatol. 2018;154(2):193–202....

2. Hairston BR, Davis MD, Pittelkow MR, et al. Livedoid vasculopathy. Arch Dermatol. 2006;142(11):1413–1418.

3. Alavi A, Hafner J, Dutz JP, et al. Livedoid vasculopathy: an in-depth analysis using a modified Delphi approach. J Am Acad Dermatol. 2013;69(6):1033–1042.

4. Vasudevan B, Neema S, Verma R. Livedoid vasculopathy: a review of pathogenesis and principles of management. Indian J Dermatol Venereol Leprol. 2016;82(5):478–488.

5. Polo Gascón MR, de Carvalho JF, de Souza Espinel DP, et al. Quality-of-life impairment in patients with livedoid vasculopathy. J Am Acad Dermatol. 2014;71(5):1024–1026.

6. Valencia IC, Falabella A, Kirsner RS, et al. Chronic venous insufficiency and venous leg ulceration. J Am Acad Dermatol. 2001;44(3):401–424.

7. Schwartz RA, Nervi SJ. Erythema nodosum: a sign of systemic disease. Am Fam Physician. 2007;75(5):695–700. Accessed December 12, 2019. https://www.aafp.org/afp/2007/0301/p695.html

This series is coordinated by John E. Delzell Jr., MD, MSPH, associate medical editor.

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