Adiposis dolorosa of scalp presenting with severe headache: an unusual case
© Springer-Verlag 2010
Received: 29 May 2010
Accepted: 16 August 2010
Published: 2 September 2010
A 46-year-old female, known case of adiposis dolorosa since adolescence, noticed painful thickening of scalp in bilateral parieto-occipital areas and vertex 1 year back. Six weeks prior to the presentation to our service, she developed severe occipital headache refractory to drug treatment. She improved after bilateral greater occipital nerve blocks. She was subjected to bilateral greater occipital chemical neurolysis which has given her complete pain relief.
The clinical picture of adiposis dolorosa makes a lasting impression on the examining clinician. Adiposis dolorosa is a disease characterized by painful subcutaneous fatty tumors. This disorder has been described to occur commonly in obese, post-menopausal women and is associated with weakness and mental disturbances such as depression, confusion, lethargy, and dementia . However, it begins in most cases prior to the post-menopausal period and can also occur in men [2, 3]. The cause is unknown, and there is no specific treatment [1, 2]. This case is reportable because adiposis dolorosa is thought to occur primarily on the body and not the head [4, 5], but our patient had involvement of scalp with severe headache.
Adiposis dolorosa is a disease of unusual distribution of fatty tumors in which the patient is easily dismissed as a malingerer, as pain in these tumors seems out of proportion to the physical findings. Pain may be relieved by steroids, intravenous lidocaine, or analgesics [1, 2]. Surgical treatment consists of excision or liposuction of the painful masses in drug refractory cases .
Diffuse Dercum’s disease of scalp is difficult to treat as other causes of headache and psychological factors have to be ruled out. These lesions cannot be treated by liposuction or excision of mass unlike other body parts . We believe that severe headache in our patient may be caused by pressure over the greater occipital nerves due to abnormally increased fat content in scalp. Ethyl alcohol produced the pain relief by local destruction of axons. The greater occipital nerve (GON) block has been described to be effective in treatment of cluster headache, cervicogenic headache occipital neuralgia and post lumbar puncture headache [7–10]. It is technically easy to perform and has a low incidence of neurological complications. This is a simple, outpatient procedure and can be repeated depending upon the recurrence of pain . We did chemical neurectomy in our patient as intractable pain dramatically responded to bilateral GON blocks and the patient did not give consent for surgical neurectomy. Our patient is under close follow-up for recurrence of symptoms. We did not find similar case report in English literature.
Chemical neurolysis has been used in the treatment of severe or intractable pain associated with cancer, trigeminal or post herpetic neuralgia and Morton’s neuroma [11–14]; however, its use in adiposis dolorosa has not been reported. We believe that it may be considered by a pain clinician when the drug refractory intractable pain is confined along a specific superficial sensory nerve territory. It should be preceded by successful response to injection of local anesthetic agent . Patient should be explained about the potential risk of permanent hypoaesthesia and local tissue necrosis. Larger numbers of such cases are required to establish the efficacy of procedure.
Adiposis dolorosa of scalp may present with drug refractory localized headache. Chemical neurolysis of nerve in the territory of headache may be considered a treatment modality for lasting relief.
The authors acknowledge with gratitude, the encouragement and support of Prof. Atul Goel, Head of the Department of Neurosurgery, KEM Hospital, Mumbai, India.
Conflict of interest
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