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| CASE REPORT |
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| Year : 2022 | Volume
: 14
| Issue : 1 | Page : 102-104 |
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Dripping candle wax lesion of the hand
Leon Alexander
Department of Surgery, Division of Plastic Surgery, Sheikh Khalifa Medical City, Abu Dhabi, UAE
| Date of Submission | 22-Apr-2021 |
| Date of Acceptance | 03-Apr-2022 |
| Date of Web Publication | 15-Jun-2022 |
Correspondence Address:
Dr. Leon Alexander
Department of Surgery, Division of Plastic Surgery, Sheikh Khalifa Medical City, Abu Dhabi
UAE
 Source of Support: None, Conflict of Interest: None  | Check |
DOI: 10.4103/jotr.jotr_30_21
It is essential to consider melorheostosis in the differential diagnosis for patients presenting with soft-tissue swelling, joint contractures, and “dripping candle wax” like hyperostotic lesions on X-rays. Melorheostosis is a sporadic disease with an uncertain etiology. It is often diagnosed incidentally and can affect the bones of the vertebrae, trunk, and upper and lower extremities. It can present with compressive symptoms due to soft-tissue swelling and fibrosis. On the hand, symptoms of median nerve compression or trigger finger may occur. The treatment of this condition is primarily conservative, and surgery is reserved for patients with severe and disabling symptoms.
Keywords: Bone tumor, contracture, dripping candle wax, hand tumor, melorheostosis, nerve compression
How to cite this article:
Alexander L. Dripping candle wax lesion of the hand. J Orthop Traumatol Rehabil 2022;14:102-4 |
| Introduction | |  |
Melorheostosis is a rare, benign condition characterized by hyperostosis of cortical bone and soft-tissue contractures. It mainly involves the periosteum and endosteum of the diaphysis of cortical bone and, depending on the extent of involvement, can be monostotic, polyostotic, or monomelic.[1],[2] There have been several reports detailing its occurrence in the lower extremity but very few describing it in the hand.[3] The exact etiology of this condition is not clear. Still, two theories have been put forward – “Classic theory” based on the embryonic infection of sensory nerve inducing bony changes in the corresponding sclerotome and the “theory of mosaicism.”[2],[3] We describe this rare lesion in an unusual site in a young woman diagnosed incidentally and discuss its implications with the relevant literature review.
This condition is usually diagnosed incidentally and involves the bones of the vertebrae, trunk, and upper and lower extremities. It is sometimes associated with soft-tissue abnormalities such as focal and subcutaneous fibrosis, vascular malformation, neurofibromatosis, arterial aneurysms, linear scleroderma, and hemangiomas. These conditions may be the initial presenting feature.[2],[3],[4]
| Case Report | | |
A 36-year-old woman, right-hand dominant, presented with complaints of swelling of the left hand, severe pain, associated pulsatility, and a feeling of hotness/warmth in the palm. She reported no history of previous trauma or similar symptoms of the hand in the past.
On examination, there was indistinct swelling in the thenar area of size 3 cm × 2 cm, slightly warm, nontender with mild pulsatility. On thenar muscle contraction, the swelling becomes more prominent. Sensation over the thumb, index, and middle fingers was reduced compared to the right side. There was no restriction in the range of motion of fingers, and motor testing of hand muscles was grade 5.
The hand X-ray revealed [Figure 1] a melorheostosis of the distal phalanx of the right middle finger on investigation [Figure 2]. Ultrasound with a Doppler study showed a probable vascular lesion (vascular malformation) from the common digital vessels supplying the thenar area. | Figure 1: Bilateral hand X-ray showing hyperostotic sclerotic bone lesion in the right hand
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 | Figure 2: Close-up X-ray showing melorheostosis of the distal phalanx of the right middle finger
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The patient was managed conservatively with analgesics as, according to her, the lesion in the left hand was decreasing in size and was not disabling. She was counseled about the incidental finding of melorheostosis in the contralateral hand and the possible morbidity associated with it. She was advised to come for regular follow-up and report back immediately if she developed symptoms of neurovascular compromise or contractures in the melorheostotic hand.
| Discussion | | |
Melorheostosis, a rare, benign sclerotic lesion of bones, was first described by Leri and Joanny in 1922.[2],[5] It is most commonly seen as an incidental finding on plain radiographs but can present with swelling, pain, joint stiffness or contractures, muscle atrophy, and limb-length discrepancy.[2],[3],[4],[5]
The investigation of choice in the diagnosis is plain radiography which shows the “classic dripping candle wax” appearance. Bony sclerosis appears to flow along the length of the bone, similar to candle wax dripping down along the side of the candle. The other patterns described in the literature include osteoma-like patterns, myositis ossificans-like patterns, osteopathia striata like, and mixed patterns. Magnetic resonance imaging and bone scintigraphy may be needed in selected cases to delineate the extent of soft-tissue involvement and multiple foci of lesions.[3],[6]
Pruitt and Manske reported two cases of patients with melorheostosis presenting with contractures of the thumb and index finger and symptoms of median nerve compression.[7] Complications such as trigger finger or carpal tunnel syndrome, joint stiffness, and contractures could develop due to soft-tissue swelling and fibrosis. These complications, when they occur, would invariably require surgical intervention.[7],[8]
In the case presented here, the patient had a history of pain and swelling in the contralateral left hand with an incidental finding of melorheostotic lesion of the right hand. Nevertheless, it is essential to consider the differential diagnosis in such scenarios, including myositis ossificans, periosteal osteosarcoma, osteomyelitis, osteopoikilosis, osteopathia striata, osteopetrosis, and pycnodysostosis.[2],[8],[9]
The treatment of melorheostosis is watchful expectancy depending on the severity of symptoms and signs of neurovascular compression. Medical therapy is helpful for patients with pain as the predominant symptom and without evidence of nerve compression. The medications commonly used include COX-2 inhibitor (celecoxib), bisphosphonates (for concomitant osteopenia), and rheologic drugs. Physiotherapy may also be beneficial for these patients.[2],[8],[10]
In cases where there is pain and paresthesia of the hand due to median nerve compression, carpal tunnel decompression must be performed as early as possible to alleviate these symptoms. Other surgical options described in the literature include debulking the sclerotic cortex, cortical fenestration, nerve decompression, neurolysis with/without nerve grafting, and trigger finger release.[1],[2],[7],[10]
We present a rare and incidental finding of melorheostosis in an unusual site (finger) of a young woman who presented with a vascular malformation of the opposite hand. Nevertheless, the patient was asked to follow-up regularly and report back if she developed symptoms of neurovascular compression. The case presented here needed no active intervention, but melorheostosis is a benign dysplasia with hyperostosis that can cause severe morbidity. The symptoms to watch out for include pain, swelling, paresthesia, restriction in joint motion, contractures, limb-length discrepancy, and bone deformities. The author recommends that the diagnosis of melorheostosis must be contemplated in cases of hyperostotic sclerotic lesion of bones with the potential for severe morbidity. Early surgical nerve decompression (carpal tunnel release), trigger finger release, and contracture release must be performed when these symptoms arise.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient has given her consent for her images and other clinical information to be reported in the journal. The patient understands that their name and initials will not be published, and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.
Financial support and sponsorship
Nil.
Conflicts of interest
There are no conflicts of interest.
| References | | |
| 1. | Farr S. Melorheostosis in the pediatric hand. Clin Case Rep 2019;7:1110-1.  |
| 2. | Fernandes CH, Nakachima LR, Santos JB, Fernandes AR, Jannini MG, Faloppa F. Melorheostosis of the thumb and trapezium bone. Hand (N Y) 2011;6:80-4. |
| 3. | Waiter JM, King JC, Louis DS, Smith J. Melorheostosis of the hand: A comprehensive review. Hand Surg 1998;3:123-3. |
| 4. | Birtane M, Eryavuz M, Unalan H, Tüzün F. Melorheostosis: Report of a new case with linear scleroderma. Clin Rheumatol 1998;17:543-5. |
| 5. | Tekin L, Akarsu S, Durmuş O, Kiralp MZ. Melorheostosis in the hand and forearm. Am J Phys Med Rehabil 2012;91:96. |
| 6. | Freyschmidt J. Melorheostosis: A review of 23 cases. Eur Radiol 2001;11:474-9. |
| 7. | Pruitt DL, Manske PR. Soft tissue contractures from melorheostosis involving the upper extremity. J Hand Surg Am 1992;17:90-3. |
| 8. | Abed AH, Mashrah HT, Almahdaly AM, Shaheen M. A rare case of melorheostosis in the hand of a Saudi woman. Cureus 2020;12:e8877. |
| 9. | Azuma H, Sakada T, Tanabe H, Handa M. Melorheostosis of the hand: A report of two cases. J Hand Surg Am 1992;17:1076-8. |
| 10. | Vyskocil V, Koudela K Jr., Pavelka T, Stajdlova K, Suchy D. Incidentally diagnosed melorheostosis of upper limb: Case report. BMC Musculoskelet Disord 2015;16:2. |
[Figure 1], [Figure 2]
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