Especially in early lesions of mycosis fungoides, i. Mycosis fungiodes is a misnomer, that name was given by the french physicians who first wrote about the disease a 100 years ago. Mycosis fungoides and cutaneous tcell lymphomas patient. Conventional mf begins as eczematoid or psoriasiform patches and. Nonlymphoid intraepidermal mononuclear cell collections pseudopautrier. Mycosis fungoides mf is the most common cutaneous t cell lymphoma ctcl. Atypical mononuclear cells are not necessary for the diagnosis of early patch lesions of mycosis fungoides, but they are found commonly in late patch lesions. Mycosis fungoides and sezary syndrome are neoplasias of malignant t lymphocytes that affect the skin. They may spontaneously disappear, remain the same size, or.
Cutaneous tcell lymphoma ctcl was a topic of discussion during several lectures presented at the 64th annual meeting of the american academy of dermatology aad. This type of lymphoma can sometimes look like mycosis fungoides, but a biopsy can tell them apart. While the cause remains unclear, most cases are not hereditary. Mycosis fungoides is a rare form of tcell lymphoma of the skin cutaneous. It generally affects the skin, but may progress internally over time.
Mycosis fungoides pictures, staging, symptoms, treatment. The conundrum of parapsoriasis versus patch stage of. Mycosis fungoides including sezary syndrome treatment pdq. Classic mycosis fungoides is divided into 3 stages. Ctcl is a type of malignancy wherein the special kind of white blood cells called tlymphocytes become infectious and drawn or deposited to the skin. In most cases, it is a chronic, slowly progressive disease that usually begins as erythematous scaly patches that over time may evolve into more infiltrated plaques or even tumors. The tumors in mycosis fungoides, which are composed of cancerous t cells, are raised nodules that are thicker and deeper than plaques. It happens when white blood cells called tcells grow out of control and move from the blood into the skin. I have a really rare nh lymphoma called mycosis fungoides, that typically manifests itself as itchy patches on the skin in the early stages.
Parapsoriasis is the term for a group of rare skin problems that look like psoriasis but behave differently. Mycosis fungoides is the most common primary cutaneous lymphoma. Mycosis fungoides is rare and for many affected people does not affect the quality of life or life expectancy. Jan 26, 2011 mycosis fungoides is a disease in which tcell lymphocytes a type of white blood cell become malignant cancerous and affect the skin. It has long been claimed that a specific histologic diagnosis of mycosis fungoides cannot be made in the premycotic or eczematous patch stage of the disease. Mycosis fungoides presenting with papules as the only clinical manifestation is a rare variant of the disease. Mycosis fungoides is a peripheral nonhodgkins tcell neoplastic process, representing the most common type of primary cutaneous malignant. Excimer laser in the treatment of mycosis fungoides. Most often there are oval or ringshaped annular pink dry patches on covered skin. Types of lymphoma of the skin american cancer society. Mycosis fungoides mf is the most common type of cutaneous tcell lymphoma ctcl and represents nearly 50% of all primary cutaneous lymphomas. In practice, however, the diagnosis of patch and early plaque stage mf can be very. Mycosis fungoides can appear anywhere on the body, but tends to affect areas of the skin protected from sun by clothing. Often, the first stage goes on for many years and is characterized by a nonspecific dermatitis, which usually consists of patches and is often found on the lower trunk and buttocks.
The first is characterized by patches and plagues covering less than 10 percent of the skin. The conundrum of parapsoriasis versus patch stage of mycosis. To enhance learning, the clues are followed by answers, explanations, and differential diagnoses. Mycosis fungoides is generally presented with plaques or patches covering the skin surface. They can arise from patches or plaques or occur on their own. This condition is the most common variety of cutaneous t cell lymphoma ctcl. Sep 04, 2019 as the nomenclature and description of the disease spectrum under the descriptive term parapsoriasis evolved, the primary focus has been on the distinction of whether the disorder progresses to mycosis fungoides mf or cutaneous tcell lymphoma ctcl.
Patches, plaques and tumors are the clinical names for different skin manifestations and are generally defined as lesions. Mycosis fungoideslike lesions associated with phenytoin and. At an early stage, patches of dry, discoloured usually red skin often appear. The patches or skin lesions are asymmetrically distributed in the areas of the skin such as in the buttocks, groin, breasts, hips and lower trunks. A sign of mycosis fungoides is a red rash on the skin. Late atrophic patches show a thinned epidermis, loss of the usual configuration between rete ridges and dermal papillae, and coarse collagen throughout a thickened papillary dermis. It is really a form of cutaneous tcell lymphoma ctcl. Mycosis fungoides can occur at any age but it is most common in the 40 to 60 yearold age group. Bernard ackerman, md histopathologic clues help the user arrive at a diagnosis. Mycosis fungoides is the most common form of cutaneous tcell lymphoma ctcl. Dec 01, 2018 mycosis fungoides mf is a cutaneous tcell nonhodgkins lymphoma.
Apr 19, 2019 mycosis fungoides is a type of lymphoma or the lymphoid system cancer. Symptoms include rash, tumors, skin lesions, and itchy skin. Mycosis fungoides is a rare form of tcell lymphoma of the skin. Mycosis fungoides pictures, staging, symptoms, causes. Know if mycosis fungoides is contagious or a skin cancer, its causes, signs, symptoms, treatment, prognosis and complications. Learn more about how it is diagnosed, treated and its prognosis. Such idiopathic cutaneous pseudot cell lymphomas may have a. Mycosis fungoides mf is a clinical diagnosis that requires strong correlation with histopathologic and sometimes molecular findings to exclude benign inflammatory diseases, more aggressive primary cutaneous lymphomas, and extracutaneous lymphomas that can involve the skin. Mycosis fungoides which is also referred to as alibertbazin syndrome is a rare skin cancer. Mycosis fungoides symptoms, diagnosis, treatments and causes. This condition is one of the most common types of tcell lymphoma.
Small plaque parapsoriasis is a benign disorder that rarely if ever progresses. Cutaneous tcell lymphomas occur when certain white blood cells, called t cells, become cancerous. Mycosis fungoides genetic and rare diseases information. Mycosis fungoides mf is the most common form of primary cutaneous tcell lymphoma ctcl characterized by epidermotropic smalltomediumsized t lymphocytes with cerebriform nuclei. A case of mycosis fungoideslike lesions developing after. Mycosis fungoides is the most common form of a type of blood cancer called cutaneous tcell lymphoma. Mycosis fungoides nord national organization for rare. The most common type of lesion of ctcl is the patchplaque mf initially described. The histological spectrum of early mycosis fungoides.
Sometimes a red rash is more than an annoying skin problem. Mycosis fungoides was so named because the tumors can resemble mushrooms, a type of fungus. Common locations for tumor development include the upper thighs and groin, breasts, armpits, and the crook of the elbow. Mycosis fungoides mf is the most common type of cutaneous tcell lymphoma, which typically presents as a patch or plaque in earlystage disease. The naming of mycosis fungoides is quite misleading and it has nothing to do with fungal infection. Mycosis fungoides is characterized by a scaly, red rash that develops on the skin, particularly on areas that are not usually exposed to the sun. Mycosis fungoides mf is the most common type of cutaneous tcell. Mycosis fungoides american journal of clinical pathology. Mycosis fungoides is rarely cured, but some people stay in remission for a long time.
In patch stage mycosis fungoides, the skin lesions are flat. Mycosis fungoides and ctcl are often used interchangeably, which is imprecise, as mycosis fungoides is just one type of ctcl. In the beginning stage, it is a lowgrade malignancy and as the tumor progresses, it can become highlyaggressive. Mycosis fungoideslike lesions associated with phenytoin and carbamazepine therapy. Revisions to the staging and classification of mycosis fungoides and sezary syndrome. Request pdf the conundrum of parapsoriasis versus patch stage of mycosis fungoides terminological confusion with benign dermatosis, such as parapsoriasis en plaques, makes it difficult to. Mycosis fungoides and sezary syndrome are diseases in which lymphocytes a type of white blood cell become malignant cancerous and affect the skin. Selected inflammatory imitators of mycosis fungoides. Usually, druginduced cutaneous pseudolymphoma is a tcell proliferation, but occasional bcellpredominant forms have been reported. In individuals with mycosis fungoides, the skin becomes infiltrated with plaques and nodules that are composed of lymphocytes.
What is the pathophysiology of cutaneous pseudolymphoma. Mycosis fungoides is a condition that may progress slowly, quickly, or not at all. More detailed information about the symptoms, causes, and treatments of mycosis fungoides is available below. The early diagnosis of mycosis fungoides mf, the major subtype of cutaneous tcell lymphoma ctcl, has important ramifications for therapeutic options, determination of prognosis, and outcomes in clinical trials. Mf encompasses a collection of different disorders that mimic mycosis fungoides primarily histologically. In general, mycosis fungoides is described with patches or plaques coating the surface of the skin. Mycosis fungoides american journal of clinical pathology oxford. This condition, also known as alibertbazin syndrome, is rare. Mycosis fungoides mf is the most common type of cutaneous tcell lymphoma ctcl, accounting for 50% of them.
The clinical as well as histopathological diagnosis of mf, especially in the early stage, can be challenging in the face of overlapping features with inflammatory dermatoses plus the fact that patients usually have received multiple treatments. The skin lesions or patches are unevenly scattered in certain skin areas like in the breasts, lower trunks, groin, hips, and buttocks. Your doctor may need to take a sample of your skin biopsy to be sure of what you. Mycosis fungoides is the most common type of ctcl, representing 4462% of cases. The name mycosis fungoides is historical and confusing. Patch, plaques, and tumors, sometimes erythroderma depending on. Mycosis fungoides and sezary syndrome are types of cutaneous tcell lymphoma. Early patch phase mycosis fungoides in a 51yearold woman. Light microscopic assessment of 100 patients with patchplaquestage mycosis fungoides.
Aug 26, 2016 mycosis fungoides, sezarys syndrome and primary cutaneous peripheral tcell lymphomas not otherwise specified are among the most important subtypes of the ctcls. Mycosis fungoides, also known as alibertbazin syndrome or granuloma fungoides, is the most common form of cutaneous tcell lymphoma. Patches of mycosis fungoides showing areas of dry, red skin. Parapsoriasis describes a group of cutaneous diseases that can be characterized by scaly patches or slightly elevated papules andor plaques dispersed on the trunk or proximal extremities that have a resemblance to psoriasishence the nomenclature. The most common skin lymphoma is a tcell skin lymphoma called mycosis fungoides. In advanced cases, ulcerated tumors and infiltration of lymph nodes by diseased cells. Patients with classic mf, as originally described by alibert and bazin two centuries ago, initially present with erythematous patches andor plaques picture 1ab, which represent the early stage of mf. Mycosis fungoides, dermatoses, patch stage of mycosis fungoides, histopathology introduction cutaneous mycosis fungoides mf, the subtype of cutaneous tcell lymphoma is a clinically and pathologically distinct form of peripheral extra nodal nonhodgkins tcell lymphoma. Patches are usually flat, can be smooth or scaly, and look like a rash. They can look like more common skin conditions such as dermatitis, eczema or psoriasis. The histopathology of patch stage mycosis fungoides mf is characterized by patchy to welldeveloped bandlike infiltrates of small to mediumsized lymphocytes within the papillary dermis combined with epidermotropism of lymphocytes and fibrosis of the papillary dermis. The disease is typically slowly progressive and chronic. Arch pathol lab medvol 8, october 2014 selected inflammatory imitators of mycosis fungoidesarps et al 21 the epidermis, a feature that is sometimes called dispro portionate epidermotropism. It exhibits a protracted clinical course with slow progression from slightly scaly skin lesions patches to infiltrated plaques and tumors.
626 925 266 993 688 750 1346 758 399 596 778 1355 1438 1 782 1560 359 580 177 830 324 146 564 1008 1660 1 639 1000 1376 884 867 263 306 879 646 590 1138