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Panniculitis is a group of diseases whose hallmark is inflammation of subcutaneous adipose tissue. Symptoms include tender skin nodules, and systemic signs such as weight loss and fatigue.
Mycosis fungoides, also known as Alibert-Bazin syndrome or granuloma fungoides, is the most common form of cutaneous T-cell lymphoma. It generally affects the skin, but may progress internally over time. Symptoms include rash, tumors, skin lesions, and itchy skin.
Cutaneous T-cell lymphoma (CTCL) is a class of non-Hodgkin lymphoma, which is a type of cancer of the immune system. Unlike most non-Hodgkin lymphomas, CTCL is caused by a mutation of T cells. The cancerous T cells in the body initially migrate to the skin, causing various lesions to appear. These lesions change shape as the disease progresses, typically beginning as what appears to be a rash which can be very itchy and eventually forming plaques and tumors before spreading to other parts of the body.
Sézary disease, or Sézary syndrome, is a type of cutaneous T-cell lymphoma that was first described by Albert Sézary. The affected T cells, known as Sézary's cells or Lutzner cells, have pathological quantities of mucopolysaccharides. Sézary disease is sometimes considered a late stage of mycosis fungoides with lymphadenopathy.
Florid cutaneous papillomatosis (FCP), is an obligate paraneoplastic syndrome.
Proliferating Angioendotheliomatosis has historically been divided into two groups, (1) a reactive, involuting type and (2) a malignant, rapidly fatal type.
Papuloerythroderma of Ofuji is a rare disorder most commonly found in Japan, characterized by pruritic papules that spare the skinfolds, producing bands of uninvolved cutis, creating the so-called deck-chair sign. Frequently there is associated blood eosinophilia. Skin biopsies reveal a dense lymphohistiocytic infiltrate, eosinophils in the papillary dermis, and increased Langerhans cells. Systemic steroids are the treatment of choice and may result in long-term remissions.
Cutaneous lymphoid hyperplasia refers to a groups of benign cutaneous disorders characterized by collections of lymphocytes, macrophages, and dendritic cells in the skin. Conditions included in this groups are:
Pagetoid reticulosis is a cutaneous condition, an uncommon lymphoproliferative disorder, sometimes considered a form of mycosis fungoides.
Granulomatous slack skin (GSS) is a rare cutaneous condition, a variant of lymphoma that typically presents in middle-aged adults.
CD30+ cutaneous T-cell lymphoma, also known as primary cutaneous anaplastic large cell lymphoma, is a cutaneous (skin) condition characterized by solitary or localized skin lesions that have a tendency to ulcerate.
Secondary cutaneous CD30+ large-cell lymphoma is a cutaneous condition that may arise in cases of mycosis fungoides, and in patients with lymphomatoid papulosis.
Non-mycosis fungoides CD30− cutaneous large T-cell lymphoma is a cutaneous condition that usually presents as solitary or generalized plaques, nodules, or tumors of short duration.
Pleomorphic T-cell lymphoma is a cutaneous condition characterized by a 5-year survival rate of 62%.
Lennert lymphoma is a systemic T-cell lymphoma that presents with cutaneous skin lesions roughly 10% of the time.
Cutaneous B-cell lymphomas constitute a group of diseases that occur less commonly than cutaneous T-cell lymphoma, and are characterized histologically by B-cells that appear similar to those normally found in germinal centers of lymph nodes. Conditions included in this group are:
Primary cutaneous immunocytoma is a skin condition that represents a low grade B-cell lymphoma related to marginal zone B-cell lymphoma but with a predominance of cells having plasmacytic features.
Primary cutaneous marginal zone lymphomas represent a heterogeneous group of diseases characterized by solitary or multiple dermal or subcutaneous nodules. Lymphomas included in this group are:
Poikiloderma vasculare atrophicans (PVA), is a cutaneous condition characterized by hypo- or hyperpigmentation, telangiectasia and skin atrophy. Other names for the condition include prereticulotic poikiloderma and atrophic parapsoriasis. The condition was first described by pioneer American pediatrician Abraham Jacobi in 1906. PVA causes areas of affected skin to appear speckled red and inflamed, yellowish and/or brown, gray or grayish-black, with scaling and a thinness that may be described as "cigarette paper". On the surface of the skin, these areas may range in size from small patches, to plaques, to neoplasms.
Günter Burg is a German dermatologist. Born in Mayen, Germany, he holds German and Swiss citizenship. He has been married to Dr. Doris Burg-Nicklas, a neurologist, since 1968. They have two sons: Andreas and Thomas.
References
- ↑ Paller, Amy S.; Mancini, Anthony J. (2015). Hurwitz Clinical Pediatric Dermatology: A Textbook of Skin Disorders of Childhood and Adolescence (5 ed.). Elsevier Health Sciences. p. 240. ISBN 978-0-323-24476-3.
- ↑ Rapini, Ronald P.; Bolognia, Jean L.; Jorizzo, Joseph L. (2007). Dermatology: 2-Volume Set. St. Louis: Mosby. ISBN 978-1-4160-2999-1.
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