Article
Vestibular Papillomatosis: A Benign Condition Mimicking Genital Warts
Vestibular papillomatosis (VP) is a benign condition of the female genitalia that may be mistaken for condyloma acuminatum (genital warts).
Buschke-Ollendorff syndrome (BOS) is a rare disease that is inherited in an autosomal-dominant fashion with high penetrance. It is characterized by osteopoikilosis associated with skin manifestations. The approximate incidence of the disease is 1:20,000, with few cases reported in the literature since 1928.1 Skeletal lesions known as osteopoikilosis are areas of increased bone density that can be seen on radiographic imaging and typically are located in the substantia spongiosa of the epiphyses and metaphyses of long bones and the pelvis. In BOS, cutaneous lesions consist of elastic or collagen nevi. Phenotypic expression of the disease is variable, and skeletal and cutaneous lesions may occur separately. Gene mutations of proteins involved in bone and connective tissue morphogenesis have been described in patients with BOS.2-5
Case Reports
Patient 1
A 17-year-old adolescent girl was referred to our hospital for evaluation of an incidental finding of osteopoikilosis that had been noted in the setting of a traumatic event. Clinical examination revealed cutaneous lesions characterized by asymptomatic, linear, stringlike and atrophic fibrotic plaques localized symmetrically on the trunk, right buttock, and right thigh (Figure 1). The lesions on the thigh were present at birth and had spread progressively to the other areas. There was no known history of inflammatory skin disease to explain the presence of the lesions, and no family history of similar signs or symptoms was reported. Histopathologic examination of a punch biopsy of a plaque from the trunk revealed increased collagen bundles associated with thick interlacing elastic fibers. The epidermis did not show any specific histologic alterations. These histopathologic features were diagnostic of a connective tissue nevus (Figure 2).
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Figure 1. An asymptomatic atrophic fibrotic plaque localized on the right thigh. |
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Figure 2. Low-power view of a punch biopsy showing a normal epidermis. Collagen bundles of the dermis were thickened and somewhat homogenized, as highlighted by the Masson stain (original magnification ×2.5). |
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Figure 3. Radiograph of the legs revealed numerous small, ovoid or round foci of sclerosis on the substantia spongiosa of the metaphyses and the epiphyses of the femur, tibia, and fibula on both sides of the body. |
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Figure 4. Multiple asymptomatic flesh-colored papules with elastic consistency on the back that were characteristic of dermatofibrosis lenticularis disseminata. |
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Figure 5. The epidermis and dermis appeared normal on histopathology; however, the elastic fibers were markedly increased in both size and number up to the deep dermis in the absence of degenerating changes (H&E, original magnification ×2.5; vascular endothelial growth factor, original magnification ×20 [inset in bottom right corner]). |
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| Figure 6. A radiograph of the left hand revealed small sporadic areas of sclerosis in the substantia spongiosa on the heads of some phalanges (white arrows). |
Radiographic imaging of the hands and feet revealed numerous small, ovoid or round foci of sclerosis that were a few millimeters in diameter and were occasionally confluent. This finding was prevalent on the carpal and tarsal bones but less evident on the phalanges and the epiphysis of the metatarsal and metacarpal bones. Full radiographic imaging of both arms and legs subsequently was obtained and showed similar lesions predominantly in the substantia spongiosa of the metaphyses and the epiphyses of the humerus, femur, tibia, and fibula bilaterally (Figure 3). Evaluation of the patient’s parents revealed that her mother had sporadic lenticular areas of increased bone density seen on radiography of the carpal and tarsal bones, particularly at the level of calcaneus, and the proximal and distal humeral epiphyses.
These lesions in our patient were consistent with an incomplete form of osteopoikilosis, which reflects the known variable expression of BOS. The radiologic findings in addition to the cutaneous lesions and the positive family history supported a diagnosis of BOS.
Patient 2
A 5-year-old boy presented with multiple congenital, asymptomatic, flesh-colored papules with elastic consistency on the left thigh, back, and pubic area that were characteristic of dermatofibrosis lenticularis disseminata (Figure 4). The patient’s 7-year-old sister had similar cutaneous lesions. The patient underwent a skin biopsy from the back that revealed thickening of collagen bundles in the dermis with an increase in elastic fibers (Figure 5). On radiographic imaging, small sporadic areas of sclerosis were noted in the substantia spongiosa of the lateral aspect of the humeral capitulum, the radial neck, the capitate, the head of the proximal phalanx of the fourth finger on the left hand, and the heads of the third and fifth middle phalanges on the left hand (Figure 6). Radiography revealed osteopoikilosis on both humeral heads in the patient’s mother. These findings in addition to the dermatologic and histopathologic features suggested a diagnosis of BOS.