Heinz Lippmann disease

Findings

The radiographs of the affected extremity (Figure 1) reveal fine granular densities that represent ossifications in the subcutaneous tissue, independent of the veins, and can be further confirmed on venography. The other radiologic features are oval or cylindrical densities that may interface and coalesce. These frequently show thin rims and lucent centers, which may have a fine weblike pattern. Heavy cords and sheets may retain central lucent zones and may show a trabecular pattern (Figure 2).

Discussion

Subcutaneous osseous deposits in relation to chronic venous insufficiency were first reported by Lippman in 1960 and have subsequently been labeled as Heinz Lippmann disease.¹ Chronic venous insufficiency is defined as tissue damage caused by abnormal changes in the venous circulation. In the lower extremities, chronic venous insufficiency is frequently caused by varicosities of the long saphenous system or by pathologies in the deep venous system, including obstruction or insufficiency. Less commonly, a short saphenous system may also be involved. Other less frequent etiologies are arteriovenous fistulas and vascular malformations. Dependent edema is an important contributory factor in the pathogenesis.^1,2

Clinical manifestations of chronic venous insufficiency are chronic dermatitis and cellulitis, as well as atrophy of the skin and subcutaneous tissue with induration and brown discoloration. Late sequelae include recurrent ulceration and subcutaneous ossification.

Although Lippmann et al¹ quoted a high incidence of subcutaneous ossification, the incidence has declined over subsequent years, following earlier diagnosis and adequate treatment of varicosities and other etiologies that lead to chronic insufficiency. Hence, in the current medical era, subcutaneous ossification is a rare event and is primarily found in cases of extremely long-standing venous insufficiency. Interestingly, Lippmann et al¹ noted a higher incidence of subcutaneous ossification in females, although venous insufficiency may have higher occupational preponderance in males.

Phleboliths can occasionally be confused with subcutaneous ossified deposits. Phleboliths tend to be more discrete and present a smoother outline.³ They have a denser rim and a small central zone of lucency. Multiple phleboliths usually remain discrete, while ossifications generally tend to coalesce.³ Arterial calcifications are usually linear, showing a double-tracked appearance, and are present in the deeper soft tissues of the leg.⁴ Myositis ossificans is classically located in the muscle rather than in subcutaneous tissue and has a characteristic appearance.⁵ Rarely, other calcifications (including parasitic infestations and calcinosis) can present in a similar manner. In most cases, a clinical history and evaluation of the radiodensities will lead to the diagnosis.^1,2

CONCLUSION

The subcutaneous ossific deposits following chronic venous insufficiency can be confused with various other causes of ossification in deeper tissues or muscle. Some etiologies may require treatment and further investigation. The evaluation of different radiodensities in tandem with a typical clinical presentation helps the diagnosis.

1. Lippmann HI, Goldin RR. Subcutaneous ossification of the legs in chronic venous insufficiency. Radiology. 1960;74:279-288.
2. Dalinka MK, Melchior EL. Soft tissue calcifications in systemic disease. Bull N Y Acad Med.1980;56:539-563.
3. Koval G, Vinogradov S. X-ray semeiotics of changes in the soft tissues and bones of the lower extremities in disorders of the venous outflow [in Russian]. Vestn Rentgenol Radiol. 1989;6:41-46.
4. Lanzer P. Monckeberg media calcinosis [in German]. Z Kardiol. 1998;87:586-593.
5. Zeanah WR, Hudson TM. Myositis ossificans: Radiologic evaluation of two cases with diagnostic computed tomograms. Clin Orthop Relat Res. 1982;168:187-191.