Buschke-ollendorff syndrome in a grande multipara: A case report and short review of the literature

Abstract

A 36-year-old short-statured grande multipara (gravida 10, para 8) with diabetes mellitus and hyperlipidaemia was incidentally found to have Buschke-Ollendorff syndrome (osteopoikilosis and dermatofibrosis lenticularis disseminata). The pelvis and hips, followed by the knees, were the sites mainly affected by the osteopoikilosis. The lumbosacral spine was also affected. She had a single connective tissue naevus on the right thigh. Apart from two abortions, the repeated pregnancies were uneventful and all her deliveries were normal at full-term or nearly full-term. With regard to the relationship between Buschke-Ollendorff syndrome and multiparity, it was apparent that multiparity did not affect the involved weight bearing bony structures, nor did the disseminated osteopoikilosis interfere with the endurance of multiple pregnancies. Buschke-Ollendorff syndrome is known generally to have a benign course, a feature that is illustrated in this case, even when associated with the stresses of multiparity. The report also provides a short and updated review of various clinical aspects of the syndrome and its associations, some of which are of a serious nature.

Connective tissue nevi: An entity revisited

“…Only two cases of osteopoikilosis with collagenoma are reported in the literature. 3,18 To explain this finding, two hypotheses can be proposed: either an elastic abnormality was present and undetected or there is heterogeneity in the type of CTN found in BOS. A second histopathologic feature in our series was the increased number of fibroblasts in 4 cases.…”

Osteopoikilosis

“…These lesions occur frequently in the long tubular bones of the hands and feet, the pelvis and scapulae [2]. They typically spare the skull, ribs and vertebrae [3].…”

Buschke-Ollendorff Syndrome

“…14,15 Although BOS is generally considered benign, this rare disorder has also been described in patients with diabetes mellitus; otosclerosis; ocular anomalies, including cataracts; peptic ulcer; cryptorchidism; congenital spinal stenosis; short stature with or without precocious puberty; and muscle contractures. 16 However, many of these associations with BOS may be coincidental. In fact, BOS is usually asymptomatic and requires no specific therapy.…”

“…19 Skull, ribs, and vertebrae are rarely involved, a finding that helps to distinguish OPK and BOS from other disorders. 16 The bone lesions take several years to de- velop, reaching maturation at or near the time of puberty, although they are commonly detectable during late childhood. 20 Typically, they change little after puberty 21 and do not predispose to fractures.…”

“…There is no evidence to suggest an increase in morbidity or mortality in patients with OPK and BOS. 16,22 Nevertheless, documentation and appreciation of the bone lesions is important by early adult life to avoid confusion with osteoblastic skeletal metastases. 23 Two different cutaneous findings have been described in BOS.…”