Michael J. Hovan

An otherwise healthy 14-year-old girl presented to her family physician with
visible growths on the plantar and lateral aspect of both feet (Figure 1).
She was especially puzzled because the lesions disappeared in a
non-weight-bearing state (Figure 2). Comments from family and classmates,
who spoke of the prominence of the lesions, heightened her concern and
prompted her visit. The condition was otherwise asymptomatic and she
acknowledged that they had been present for several years.

Question

Although stable and asymptomatic, with which one of the following
disease states is this otherwise benign condition sometimes associated?

[ ] A. Obesity.
[ ] B. Hyperlipidemia.
[ ] C. Tophaceous gout.
[ ] D. Ehlers-Danlos syndrome.
[ ] E. Marfan syndrome.

Discussion
The answer is D: Ehlers-Danlos syndrome. Piezogenic pedal papules usually
reflect a benign condition consisting of normal fat tissue that has extruded
through the dermis. The papules usually appear on the plantar aspect of the
foot and heel and are more marked on the medial aspect; they may also occur
near the wrist and lateral borders of the hand. They are visible only when
the patient is weight bearing and immediately disappear in a
non-weight-bearing posture. Occasionally, the papules will be painful. This
has been attributed to ischemia of the herniated fatty tissue caused by
compressive compromise of its blood supply.

Piezogenic papules are most commonly diagnosed in otherwise healthy
patients.(1) Usually, they are not suggestive of systemic diseases. Because
they are typically painless, they may go unrecognized. They only impair
musculoskeletal function if they cause pain from focal ischemic effects, but
ulceration does not occur.

Sometimes the papules may be familial.(2) Rarely, they may be associated
with Ehlers-Danlos syndrome. This patient, however, did not demonstrate
hyperlaxity of joints, hyperelasticity of skin, easy bruising, or atrophic
skin changes typically seen in Ehlers-Danlos patients. Additionally, no
family members reported a history of the syndrome.

Treatment of the papules is usually unnecessary unless they cause pain.
Therapeutic measures include use of heel pads and avoidance of prolonged
standing. If these interventions fail, excision of the papules can be
performed, but this is rarely necessary.

Obesity, per se, is not associated with piezogenic pedal papules, and most
patients with piezogenic papules are otherwise healthy.

Piezogenic papules are not seen in Marfan syndrome, in which superficial
cutaneous findings are minimal. Striae may be observed over the shoulders
and buttocks. Otherwise, the skin is grossly normal. Skeletal abnormalities,
including long extremities, are observed. Classically, the hands and fingers
are described as spider-like.

Xanthomas can occur in disorders of lipid metabolism, such as familial
hyperlipidemia. High triglyceride values exceeding 1,000 mg per dL may
result in eruptive xanthomas. These are red-yellow, painless lesions that
are most prominent on the buttocks. Their appearance does not change in the
weight-bearing state.

Tophi are collections of solid urate that deposit in connective tissues in
patients with gout. Common locations include the external ear, foot, hand,
prepatellar bursa, and olecranon region. Their appearance also remains
unchanged in the non-weight-bearing state.

MICHAEL J. HOVAN, M.D.
Mayo Clinic
13737 N. 92nd St.
Scottsdale, AZ 85260

REFERENCES

(1.) van Straaten EA, van Langen IM, Oorthuys JW, Oosting J. Piezogenic
papules of the feet in healthy children and their possible relation with
connective tissue disorders. Pediatr Dermatol 1991;8:277-9.

(2.) Gibney MD, Glaser DA. Piezogenic pedal papules in two family members.
Cutis 1996;57:260-2.