What we do
Cutaneous T-cell lymphoma is an uncommon lymphoproliferative skin disease increasingly diagnosed in the paediatric population. This condition poses several challenges due to the variable clinical spectrum and difficulties of making an early diagnosis as well as the lack of consensus regarding the ideal treatment. The CTCL clinic focuses on controlling the signs and symptoms and slowing the spread of the disease in children and adolescents.
Cutaneous lymphoma comprises a wide group of skin neoplasms derived from cells of the lymphoid lineage. They are broadly divided into B-cell lymphomas and T-cell lymphomas. Cutaneous T-cell lymphoma (CTCL) is the most common of the primary skin lymphomas. Mycosis fungoides (MF) is the most common form of CTCL. CTCL is a chronic lymphoproliferative disease, often with an insidious onset. It is usually a neoplasm of the T-helper subset of T cells.
SickKids started an interdisciplinary EB clinic in 2004. The clinic has positively impacted on the care provided to these patients by appointment co-ordination, increased local expertise and improved wound care.
EB is a series of genetically inherited disorders where the skin is prone to blistering. There are three major types of EB; each type is very different in the way that it affects the skin:
- Epidermolysis Bullosa Simplex
- Dystrophic Epidermolysis Bullosa
- Junctional Epidermolysis Bullosa
There is no specific treatment for EB and most therapeutic guidelines target prevention of trauma as the desired approach. In everyday life, complete avoidance of friction or trauma is impossible, so most patients experience different degrees of blistering and development of chronic wounds. As in other skin diseases, this condition can have a profound impact on the daily activities of affected patients. The limitations patients may have due to pain, itch, odour and time-consuming dressing changes are problems that affect their quality of life. The EB clinic at SickKids offers an interdisciplinary approach to management of this condition.
Tips for Managing a Patient with EB:
- Find a supportive medical team that both your child and your family can use as a support system.
- Seek counseling for effective footwear (to prevent blistering of the feet as much as possible)
- Buy soft and non-irritating clothing. Avoid clothing seams having contact with the skin whenever possible.
- Avoid warm/hot temperatures, they tend to induce blistering.
- Talk to your child’s physician about a plan for bandaging the blister sites, many bandages can cause more damage to the skin, it is very important to devise a plan that works with your child’s condition.
- Receive genetic counseling. Because EB is a genetically inherited disease, it is very important that both you and your child get advice on the genetics of this condition and what to expect in the future.
- Treat your child as normally as possible, allow them to become comfortable with setting their own boundaries. Encourage independence and growth.
Performing General Nursing Care for the infant with EB:
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Morphea or localized scleroderma refers to a group of skin conditions consisting of an excessive accumulation of collagen in the skin and subdermal tissues. Several classifications have been proposed for this condition. Although there is no uniformity of agreement about its classification, clinicians agree that most patients can be grouped into plaque morphea, linear morphea or generalized morphea.
Morphea is a rare condition with an estimated incidence of 2.7 cases per 100,000.
Plaque morphea is most common presentation, accounting for 56% of all cases. The etiology and pathogenesis of this condition are still unclear. It is known that morphea patients have abnormal collagen fibers that become thickened and hyalinized. It has been postulated that autoimmunity, environmental factors, infection and trauma may trigger cytokine production and release that, in turn, are responsible for increased fibroblast and collagen synthesis.
A new clinical service was initiated at SickKids in January 2006 based on the need for focused, expert, inter-disciplinary care for children with vascular tumours with systemic complications. The service involves a inter-disciplinary outpatient clinic and an inpatient consultation service. The leading services are Dermatology, Paediatric Medicine, and Hematology/Oncology with ‘consultant’ input from Interventional Radiology, General Surgery, Thrombosis, Neurology, and Cardiology. The goals of this service are to provide best care for children with Vascular Tumors by:
- Developing a clinical expertise amongst a consistent group of multi-disciplinary specialists
- Developing management guidelines based on consensus and the available literature
- Conducting scholarly work in the area of VTs
Vascular tumors (VTs) are benign endothelial cell proliferations that include infantile hemangioma (IH), congenital hemangioma, kaposiform hemangioendothelioma (KHE), tufted angioma (TA) and lymphangioendotheliomatosis.
Infantile hemangioma (IH)
Infantile hemangioma is the most common benign vascular tumour of infancy(1). Although most regress spontaneously, 10% of hemangiomas require treatment because of serious medical complications. These include life threatening mass effect (e.g. airway compromise)(2), high-flow shunts resulting in heart failure (e.g. multifocal hepatic hemangiomas)(3), abdominal compartment syndrome (diffuse hepatic hemangioma)(3) and severe hypothyroidism(4). They may also be disfiguring, and cause problems due to ulceration.
Kaposiform hemangioendothelioma (KHE) and Tufted Angioma (TA)
KHE and TA are rare and distinct clinical, morphologic and pathologic entities from IH(5;6). Unlike IH, KHE and TA are associated with Kasabach-Merritt Phenomenom (KMP)(7-9) which results in severe thrombocytopenia, hypofibrinogenemia, coagulopathy, and anemia. These tumours are not responsive to corticosteroids and require multi-modal therapy(7).
Multifocal lymphangioendotheliomatosis with thrombocytopenia is a recently described entity(10). Clinically, affected patients present as numerous congenital cutaneous and gastrointestinal (GI) vascular papules and plaques leading to severe GI bleeding commencing in early infancy. Thrombocytopenia, most likely due to platelet trapping in the vascular lesions is an additional essential finding in all reported patients.
Referrals to Dermatology clinic are accepted from paediatricians, dermatologists and family practitioners. Please visit Ambulatory Clinics for more information.