Severe acitretin-induced diffuse idiopathic skeletal hyperostosis: a case report.

Diffuse idiopathic skeletal hyperostosis (DISH), also known as Forestier-Rotes-Querol disease, is a systemic noninflammatory disease characterized by ossification of the entheses. DISH predominantly affects the spine. Although peripheral involvement is also often reported, it rarely affects patients' function. A 77-year-old man presented to our emergency department because of incapacitating pain and stiffness in the spine and hips. The patient had been diagnosed with biopsy-proven mycosis fungoides 3 years earlier and had been treated with oral acitretin at 25 to 50 mg daily since diagnosis. However, the patient gradually developed a severely limited range of motion in his spine and hips (left > right), significantly impairing his mobility and activities of daily living. Cervical and dorsolumbar radiographs showed extensive ossification along the anterior longitudinal ligament; this finding was compatible with DISH and had not been present in radiographs taken 3 years earlier. Pelvic radiographs showed multiple enthesophytes predominantly around the coxofemoral joints. DISH has been reported as a possible long-term adverse effect of acitretin. Despite optimal conservative treatment, the patient remained severely impaired and thus finally underwent extensive osteophyte excision and total hip replacement on the left side. His acitretin therapy was also stopped to prevent further progression of his DISH.

Diffuse idiopathic skeletal hyperostosis in a 33-year-old woman with PCOS and metabolic syndrome: a rare scenario.

Diffuse idiopathic skeletal hyperostosis (DISH) is a common degenerative enthesopathy seen in the elderly with male preponderance. It is uncommon in patients before 50 years of age and is extremely rare in patients younger than 40 years. We report a case of 33-year-old unmarried woman who presented with inflammatory spinal pain and stiffness, limited chest expansion, decreased range of spinal motion and postural abnormalities, all of which suggested the diagnosis of ankylosing spondylitis, considering the patient's age. But, further evaluation led us to the final diagnosis of DISH with associated metabolic syndrome and polycystic ovarian syndrome (PCOS). To the best of our knowledge, our patient is the first reported case of DISH in a woman less than 40 years of age, and also the first case of DISH associated with PCOS and metabolic syndrome.

Presentación simultánea de espondilitis anquilosante e hiperostosis esquelética idiopática difusa (enfermedad de Forestier-Rotès-Quérol) / Simultaneous occurrence of ankylosing spondylitis and diffuse idiopathic skeletal hyperostosis (Forestier-Rotès-Quérol disease)

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Diffuse idiopathic skeletal hyperostosis and isotretinoin in cystic acne.

We present the case of a 35-year-old man with thoracic back pain and stiffness, whose only medical history was cystic acne treated with repeated courses of retinoids. His thoracic spine was severely limited in range of movement and was found, on X-ray, to have unilateral hyperostosis typical of diffuse idiopathic skeletal hyperostosis (DISH)--an often asymptomatic condition rarely found in those under 50. Back stiffness in young patients with prolonged retinoid exposure should be investigated.

A case of psoriasis vulgaris with diffuse idiopathic skeletal hyperostosis involved with ossifications of posterior and anterior longitudinal ligament.

Diffuse idiopathic skeletal hyperostosis (DISH) is difficult to distinguish from various forms of inflammatory arthritis, including psoriatic arthritis (PsA), rheumatoid arthritis, and ankylosing spondylitis. A 67-year-old Japanese male had been treated for psoriasis vulgaris for 13 years. Numbness of his right arm and lower limbs and spinal stiffening had developed 7 years prior to his initial evaluation at our facility. He noticed pain mainly while exercising. There were symmetrical marginal syndesmophytes in the spine, from the thoracic vertebrae to the upper lumbar vertebrae, on radiological examinations. We therefore suspected DISH. Furthermore, ossifications of the posterior and anterior longitudinal ligaments were noted in the cervical spine. Laboratory examinations revealed a normal peripheral white blood cell count, serum C-reactive protein, and erythrocyte sedimentation rate, and he was negative for rheumatoid factor. We detected human leukocyte antigen B39 but not B27. All distal interphalangeal joints were swollen but without pain. X-ray imaging showed narrowing of the joint space, and the consolidation of the joint was recognized, but there was no new juxta-articular bone formation. Based on clinical and radiological findings, we concluded that he had DISH and not PsA. DISH was indicated by marked radiological features of the axial skeleton, particularly the thoracic spine, but may also have involved the peripheral joints. DISH is one of the entheseal disorders, and 10% of Japanese middle-aged and elderly men have DISH. Therefore, the differentiation of DISH from PsA is necessary in psoriasis patients with spinal involvement.

[Cervical osteophytosis as cause of dysphagia. Forestier-Rotes Querol's disease]. / Osteofitosis cervical como causa de disfagia. Enfermedad de Forestier-Rotes Querol.

The so called Forestier-Rotes Querol's disease or diffuse idiopathic skeletal hyperostosis can affect the neck caused by osteophytos and ossification-calcification of the vertebral ligaments. The location in that region can produce dysphagia or hoarseness, among other symptoms, so that the patient can assist or be sent to ENT valoration. We are reporting a case of this pathology which was diagnosed in our hospital. Finally a bibliographic review is performed.

Clinical manifestations of diffuse idiopathic skeletal hyperostosis of the cervical spine.

OBJECTIVES: To describe the clinical manifestations and the complications of cervical spine (C-spine) involvement in diffuse idiopathic skeletal hyperostosis (DISH). METHODS: Two patients, who presented with dysphagia resulting from large anterior osteophytes of the C-spine, were diagnosed as having DISH. A Medline search from 1964 to present, using the terms "diffuse idiopathic skeletal hyperostosis" and "cervical spine," identified several clinical manifestations associated with DISH. RESULTS: Two groups of conditions associated with DISH were found. 1. Spontaneous complications such as: dysphagia, being the commonest, dyspnea, stridor, myelopathy associated with ossification of the posterior longitudinal ligament (OPLL) or with atlanto-axial pseudoarthrosis or subluxation. Other rare events were aspiration pneumonia, sleep apnea and thoracic outlet syndrome. 2. Provoked complications such as endoscopic and intubation difficulties and fractures of the C-spine with frequent transverse shift of the fractured segment and resultant myelopathy. CONCLUSIONS: C-spine involvement in DISH is a recognized cause of various clinical manifestations involving the pharynx, larynx and the esophagus. Prior knowledge of the existence of cervical DISH should alert the clinicians for possible complications, at times severe, during invasive procedures in the neck region and as a consequence of trauma.

Calcitonin simultaneously regulates both periosteal hyperostosis and trabecular osteopenia in the spinal hyperostotic mouse (twy/twy) in vivo.

The twy (tiptoe-walking-Yoshimura) mouse, established in Japan in 1978 by brother-sister mating of ICR strain mice, is a valuable mutant as a model of ossification of the posterior longitudinal ligament (OPLL). OPLL causes severe myelopathy and has been thought to be very similar to ankylosing spinal hyperostosis (ASH) and diffuse idiopathic skeletal hyperostosis (DISH). In the twy mouse, both an increase in vertebral cortical membranous bone formation and a decrease in trabecular bone mass due to accelerated bone resorption occur simultaneously. This process is attributed to an inherited autosomal recessive single gene (twy). Calcitonin's suppression of bone resorption has been well established in the past, whereas the effects of this hormone on bone formation remain to be defined. Of particular interest is the simultaneous action of calcitonin on the abnormally accelerated bone formation and resorption. Thirty twy mice and 14 ICR mice were divided into seven groups, and changes induced by calcitonin on vertebral cortical appositional rate and on trabecular bone mass were investigated histomorphometrically. Results were (1) osteoclastic activity on trabecular surface was clearly suppressed by chicken calcitonin injected subcutaneously for 4 weeks; (2) no significant difference between the lumbar vertebral periosteal bone formation of calcitonin (CA) and vehicle-administrated twy mice groups. However, on the periosteal surface of the cervical vertebrae of the 6-week-old twy mice, the abnormally accelerated bone formation was suppressed by CA administration. This was also true for the elderly twy mice, although the effect was less pronounced. In conclusion, CA suppressed the abnormally hyperactivated periosteal bone formation. Results also suggested a possible therapeutic value of CA for OPLL.

Hiperostosis cortical idiopática con disproteinemia (síndrome de Goldbloom) / Idiopathic periosteal hypeostosis with dysproteinemia (Goldblooms syndrome)

La hiperostosis cortical idiopática con disproteinemia o síndrome de Goldbloom (SG) es un síndrome de escasa frecuencia y de etiología desconocida. Suele comenzar después de la primera infancia como una virosis inespecífica, seguido luego por fiebre, hiperostosis y trastornos en el proteinograma. Comentamos la situación de un niño de 5 años de edad con SG que se presentó como un síndrome febril prolongado (SFP) de 9 meses de evolución y dolores óseos. Al cabo del 5º mes de evolución aparecieron induraciones calientes y profundas en las extremidades proximales de ambas tibias. Presentó hipergamaglobulinemia. Las radiografías mostraron hiperostosis cortical y la centellografía un aumento de la captación del material r

Hiperostosis cortical idiopática con disproteinemia (síndrome de Goldbloom) / Idiopathic periosteal hypeostosis with dysproteinemia (Goldbloom's syndrome)

La hiperostosis cortical idiopática con disproteinemia o síndrome de Goldbloom (SG) es un síndrome de escasa frecuencia y de etiología desconocida. Suele comenzar después de la primera infancia como una virosis inespecífica, seguido luego por fiebre, hiperostosis y trastornos en el proteinograma. Comentamos la situación de un niño de 5 años de edad con SG que se presentó como un síndrome febril prolongado (SFP) de 9 meses de evolución y dolores óseos. Al cabo del 5º mes de evolución aparecieron induraciones calientes y profundas en las extremidades proximales de ambas tibias. Presentó hipergamaglobulinemia. Las radiografías mostraron hiperostosis cortical y la centellografía un aumento de la captación del material radionucleico en las zonas afectadas. La enfermedad se prolongó por 2 años, con una mejoría progresiva y total. Debe considerarse este diagnóstico ante todo niño con SFP y dolores óseos

Diffuse idiopathic skeletal hyperostosis with dysphagia (a review).

Dysphagia due to cervical osteophytes is not common. However, diffuse idiopathic skeletal hyperostosis (DISH) with cervical involvement which causes dysphagia is even rarer. The otolaryngologist is not generally familiar with this entity. The diagnosis can be made by plain cervical X-ray films, a barium swallowing esophagogram and or a CT scan of the neck. When doubt still exists, further extra-axial X-ray films can be helpful. Although most patients have been treated surgically, there may be a role for conservative therapy initially, as surgery in elderly DISH patients is often morbid and even fatal. A 79-year-old patient with DISH (Forestier's disease) is reported. Non-steroidal anti-inflammatory therapy was successfully implemented. DISH is compared with other disorders of the cervical spine which may cause dysphagia.