British Journal of Radiology (2005) 78, 662-671
© 2005 British Institute of Radiology
doi: 10.1259/bjr/66219766
Radiological associations with dermatological disease
J Jones, MBBS, BSc, MRCP1,
C Brenner, FRCR2,
R Chinn, MBBS, MRCP, FRCR2 and
C B Bunker, MA, MD, FRCP1
1 Departments of Dermatology and 2 Radiology, Chelsea and Westminster Hospital, Fulham Road, London, UK
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Abstract
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Skin disease and its treatment may have radiological connotations. Though this article is not a comprehensive account of dermatological and radiological associations, it provides an eclectic selection of some common and some rare clinical entities, which the practicing radiologist may recognize or remember.
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Crohn's disease
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Crohn's disease is a disease primarily of the gastrointestinal (GI) tract, with non-caseating granulomas leading to inflammation, ulceration and fibrosis of bowel. It can cause disease from mouth to anus. There are many extraintestinal manifestations. Pyoderma gangrenosum is illustrated in Figure 1
. Granulomatous cheilitis is illustrated in Figure 2.
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Figure 1. Irregular ulcerations with indurated erythematous margins and necrotic centres; Pyoderma gangrenosum in Crohn's disease.
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Figure 2. Granulomatous cheilitis in Crohn's disease. The differential diagnosis includes sarcoidosis.
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Gorlin's syndrome
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Also known as basal cell naevus syndrome, Gorlin's syndrome is an autosomal dominant inherited condition encompassing a group of cranial, maxillofacial and systemic disorders the radiology of which are well described [1–3] (Figure 3). Basal cell carcinomas begin to appear in late childhood mainly on sun-exposed areas. Palmoplantar lesions occur in 50%. Figure 4 shows a superficial basal cell carcinoma. It illustrates that the presentation may be subtle.
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Kaposi's sarcoma
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Kaposi's sarcoma (KS) (Figure 5) is the most common Acquired Immune Deficiency Syndrome (AIDS) related malignancy in Western countries and Africa. It can affect the skeletal system, the GI tract and the lungs (Figure 6), although rarely without the cutaneous manifestations. Pulmonary KS occurs in 18–47% of patients with known cutaneous KS and can affect the lung parenchyma, pleura or tracheobronchial tree. Bilateral perihilar pulmonary infiltrates are seen in the majority of patients, which extend into the pulmonary parenchyma along the bronchovascular bundles. Thickening of the interlobular septae and nodularity of the fissures may also be seen. Pleural effusions, pericardial effusions and mediastinal lymphadenopathy are recognized findings. Chest wall disease involving the sternum, thoracic spine and subcutaneous tissues may also occur [4].
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Figure 6. Chest radiograph in an HIV patient, showing multiple bilateral soft tissue nodules (pulmonary Kaposi's sarcoma).
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Langerhan's cell histiocytosis
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Langerhan's cell histiocytosis is now the collective term for the diseases previously described separately as Histiocytosis X, eosinophillic granuloma, Hand-Schuller-Christian syndrome and Letterer-Siwe disease. Diagnostic imaging plays a major role in the diagnosis and management of the disease, from plain films and radionuclide scintigraphy to CT and MRI. Isolated bone lesions (Figure 7), typically with bevelled margins and without sclerosis, have the best prognosis, while those with multisystem involvement may have a rapidly potentially fatal course (Figure 8) [5, 6]. Skin lesions include yellowish nodules, ulcers affecting oral and genital mucosa, and papulosquamous vesicular or purpuric eruptions that may become necrotic or crusted. Figure 9 shows a subtle lesion in a child with Langerhan's cells histiocytosis
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Figure 7. Lateral lumbar spine in a child, showing a vertebra plana – complete collapse of a vertebral body with normal disc spaces.
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Figure 8. (a) Chest radiograph and (b) axial high resolution CT of the thorax in a patient with histiocytosis, showing reticulonodular shadowing and honeycombing of the lung with preserved lung volume.
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Neurofibromatosis
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Type 1 neurofibromatosis (peripheral neurofibromatosis or Von Recklinghausen's disease) accounts for 90% of the disease, with Type 2 (central neurofibromatosis) being less common. The former has autosomal recessive inheritance and the latter autosomal dominant inheritance. The bulk of the radiology findings apply to Type 1 disease, while Type 2 disease is confined to the brain and spinal cord [7–9]. Examples of characteristic findings are shown in Figures 10 and 11.
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Figure 10. Axial enhanced CT scan of the abdomen in a patient with neurofibromatosis showing a 3 cm phaeochromocytoma with heterogeneous enhancement and central calcification in the right adrenal gland.
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Figure 11. Coronal T2 weighted MRI of the lumbosacral region showing multiple plexiform neurofibromata.
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Cutaneous lesions include macules which may vary in size, axillary freckling, and café-au-lait spots (which appear during the first 3 years of life). Neurofibromas (papules or nodules) may be skin coloured, pink or brown, may be pedunculated or dome shaped, and may be soft or firm. Neurofibromata appear during late adolescence and may be painful. Figure 12 shows axillary freckling.
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Psoriasis
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Arthropathy affects <5% of patients with psoriasis. The radiology of psoriasis is well recognized, as an asymmetrical erosive arthropathy associated with periosteal new bone and preservation of bone density. Progressive joint deformity and ankylosis may occur. Typically the small joints of the hand and feet are affected (Figure 13).
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Figure 13. Radiograph of the index finger in a psoriatic patient showing an asymmetric erosive arthropathy.
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Vertebral involvement is predominantly with paravertebral syndesmophytes, squaring of the vertebral bodies and paravertebral calcification [10]. Skin lesions include salmon pink papules and plaques, which are sharply marginated with silvery white scale. Scalp, genital and nail involvement is common. Figure 14 shows chronic plaque psoriasis.
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Sarcoidosis
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This is a non-caseating granulomatous disorder with multisystem involvement. The respiratory manifestations are most commonly encountered. The abdominal, neurological and bone manifestations are well described. Radiology has a role in both diagnosis and monitoring of the disease [3, 11–13]. Skin lesions are brownish-purple infiltrated plaques (Figure 15) which may be annular or serpiginous. They appear mainly on the extremities, buttocks and trunk. Lupus pernio, diffuse violaceous soft doughy infiltrations, may occur on the nose, cheeks or earlobes.
Sarcoidosis may infiltrate scars which then exhibit translucent purple-red or yellow papules or nodules. An acute arthralgia may accompany erythema nodosum. A more chronic polyarthritis and dactylitis can occur (Figure 16).
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Tuberous sclerosis
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Previously known as Bourneville Disease, this is an autosomal dominant condition characterized by epilepsy, mental retardation and adenoma sebaceum, but with multisystem involvement. The neuroradiology findings are perhaps best known (Figure 17) [8, 18].
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Figure 17. Axial post contrast CT brain at sequential levels, showing the typical calcified subependymal and parenchymal nodules, and an enhancing intraventricular giant cell astrocytoma at the foramen of Monro.
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Shagreen patches (Figure 18) are skin coloured and occur in 40% of patients, usually affecting the back and buttocks. Periungal papules or nodules (Figure 19) are present in 22% of patients. They arise in late childhood and are similar histologically to angiofibromas.
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Dermatomyositis and systemic sclerosis
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Dermatomyositis affects mainly the subcutaneous soft tissues and skeleton (Figures 20 and 21) although other systems may be involved. Progressive systemic sclerosis may also have multisystem involvement [19]. Figures 22 and 23 show the cutaneous and nailfold changes, respectively. In older patients, dermatomyositis has a significant association with underlying malignancy. Raynaud's phenomenon may result in painful ulceration of the fingertips. Later, sclerodactyly with tapering of the fingers and tight waxy skin occurs. Bony resorption and ulceration leads to loss of the distal phalanges. Raynaud's phenomenon is illustrated in Figure 24, and severe sclerodactyly in Figure 25. Cutaneous calcification may occur on fingertips or over bony prominences. Oedema and fibrosis of the facial skin results in loss of the normal facial lines. Facial telangiectasia may also occur.
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Figure 20. Radiograph of the left hand in a patient with subcutaneous calcification of the digits in a patient with scleroderma.
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Figure 21. Chest and upper abdominal radiograph demonstrating with extensive subcutaneous calcification in a patient with dermatomyositis.
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Figure 22. Gottran's papules affecting the knuckles in a patient with dermatomyositis and breast cancer.
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Figure 23. Nail fold erythema, telangiectasia and ragged cuticles in a patient with dermatomyositis and breast cancer.
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Rosai Dorfman Disease (sinus histiocytosis with massive lymphadenopathy)
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Rosai Dorfman Disease is a rare histiocytic lymphoproliferative disorder. It is thought to be a reactive condition. A number of infectious agents have been implicated, including Epstein-Barr virus and Klebsiella. It usually affects young adults. About 90% of patients present with cervical lymphadenopathy, although adenopathy elsewhere is also common (Figure 26). Extranodal involvement is common, and the skin is the most commonly affected organ. Skin lesions are usually yellow but may be purple or violaceous. Macular erythema, papules, nodules (Figure 27) and infiltrated plaques (Figure 28) have been reported. Scaling is often present and telangiectasia may be seen. Other organs involved include the CNS, bone and salivary glands.
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Figure 26. Axial post contrast CT of the pelvis showing lymphadenopathy in a patient with Rosai Dorfman syndrome.
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DISH and systemic retinoids
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Diffuse idiopathic skeletal hyperostosis (DISH), is characterized by anterolateral flowing osteophytes of at least four contiguous vertebrae, with sparing of the disc spaces (Figure 29). Long term use of systemic retinoids for dermatological disease carries a significant risk of DISH.
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Figure 29. Lateral radiograph of the thoracolumbar spine showing anterior bridging osteophytes over four vertebral bodies, with sparing of the disc spaces. These findings developed while on treatment with retinoids.
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Received for publication October 31, 2003.
Revision received December 20, 2004.
Accepted for publication January 13, 2005.
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References
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Abstract
Crohn's disease
