Cd30positive large cell transformation of mycosis fungoides. Update on skin directed therapies in mycosis fungoides. Hi mona, a very interesting blog which will be sure to cause controversy. Article in italian pimpinelli n, santucci m, ghersetich i, brogelli l, moretti s.
For unknown reasons, mycosis fungoides affects males nearly twice as often as females. Large plaque parapsoriasis an overview sciencedirect. Bajaj a 2018 patch, plaque, tumour mycosis fungoides. Practical aspects regarding the histopathological diagnosis of. Tests that examine the skin and blood are used to detect find and diagnose mycosis fungoides. The clinical picture of mycosis fungoides is variable and, in the early stages, is reminiscent of psoriasis vulgaris. 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. I have always hated the term parapsoriasis because it has many variants all of which are different conditions.
In patch stage mycosis fungoides, the skin lesions are flat. Update on skin directed therapies in mycosis fungoides mycosis fungoides mf represents the majority of the primary cutaneous tcell lymphomas ctcl. Large plaque parapsoriasis is more ominous in that approximately 10% of patients progress to mycosis fungoides or cutaneous tcell lymphoma 9. Large plaque parapsoriasis lpp is an idiopathic, chronic scaly dermatosis classified within parapsoriasis group of diseases, occurring commonly in middle aged patients of all races and geographic regions. The patch stage of mycosis fungoides is characterized by usually erythematous macules that may have a fine scale, may be single or multiple, and may be pruritic see the image below. Conventional mf begins as eczematoid or psoriasiform patches and. Mycosis fungoides is rarely cured, but some people stay in remission for a long time. Classic mycosis fungoides is divided into 3 stages. As parapsoriasis and specifically lpp is related to mycosis. Smallplaque parapsoriasis digitate dermatosis, chronic superficial dermatitis presents as ovoid or digitate plaques measuring 2 to 6 cm in diameter, with a predilection for the lateral trunk. Mycosis fungoides causes, symptoms, diagnosis, stages. A patient with clinicopathologic features of small plaque parapsoriasis presenting later with plaquestage mycosis fungoides. Terminological confusion with benign dermatosis, such as parapsoriasis en plaques, makes it difficult to diagnose mycosis fungoides in the early patch stage.
Histopathologic features of early patch lesions of mycosis fungoides. Parapsoriasis these skin problems can look similar at the skin surface, but they are different under a microscope. This is associated with the histologic appearance of large, atypical cells. Parapsoriasis en plaques mycosis fungoides psoriasis causes. Lpp and its variants are closely related to the patch stage of mycosis fungoides. The term mycosis fungoides was only adopted by alibert in 1832. Parapsoriasis is an etiologically heterogeneous group of disorders that tend to have a similar appearancethin, scaling, dull, pink patches and plaques with a slightly atrophic or wrinkled appearance. The symptoms and skin biopsy findings of mf are similar to other benign skin conditions like eczema, psoriasis, parapsoriasis, or pityriasis lichenoides. The differences between the patients with each variation of stage i mf are as follows.
Subsequent cancers, mortality, and causes of death in patients with mycosis fungoides and parapsoriasis. The patch stage of mf represents the early stages of ctcl, and the. Parapsoriasis en plaque has been suggested to be an early manifestation of mycosis fungoides cutaneous tcell lymphoma. Such description is classic and has been used to this date. It may be evident as a new, solitary nodule within a classic mycosis fungoides patch or. In one study, large plaque parapsoriasis has expression of tox similar to that of mycosis fungoides. Although large plaque parapsoriasis is considered an early form of mf, the relation of small plaque parapsoriasis spp to mf is still a matter of discussion. Sep 12, 2006 making a differential diagnosis between early mycosis fungoides mf and parapsoriasis is often difficult at the clinical and histological level. Mycosis fungoides mf is a cutaneous tcell lymphoma. Plaques are thicker, raised, usually scaly lesions.
I am afriad that i am one of those dermatopathologists who regard large plaque parapsoriasis as synonymous with mycosis fungoides. Parapsoriasis with plaques parapsoriasis with larger plaques transforms into cutaneous tcell lymphoma in about 10% of patients per decade. Parapsoriasis is a term encompassing several disorders of the skin that possess a similar clinical presentation to psoriasis. Mycosis fungoides mf is the most common type of cutaneous tcell lymphoma ctcl, accounting for 50% of them. For example, it can develop into cutaneous tcell lymphoma. Controversy exists currently in the classification of large plaque parapsoriasis because some believe it is equivalent to the earliest stage of cutaneous tcell lymphoma, the patch stage 10. In plaque stage mycosis fungoides, the patches become thickened and may resemble psoriasis. The pathology of cutaneous tcell lymphoma cancer network. Treatment of patchstage mycosis fungoides with topical corticosteroids.
Mycosis fungoides mycosis fungoides mf is the most common type of ctcl, accounting for 50% of all primary ctcl cases. They may spontaneously disappear, remain the same size, or. Cutaneous tcell lymphoma presenting as benign dermatoses. A total of 88 patients were included in 2 categories. Treatment of small and large plaque parapsoriasis and mycosis fungoides mf for many years has consisted of uvb 1 as well as psoralenuva puva 26 therapy.
Parapsoriasis knowledge for medical students and physicians. Parapsoriasis dermatologic disorders merck manuals. The term parapsoriasis was coined originally by brocq in 1902. Pityriasis lichenoides, large plaque parapsoriasis than 5 cm in diameter and small plaque parapsoriasis parapsoriasis presenting later with plaquestage mycosis fungoides. Request pdf the conundrum of parapsoriasis versus patch stage of mycosis fungoides terminological confusion with benign dermatosis, such as parapsoriasis en plaques, makes it. Actuarial diseasespecific survival of 270 patients with stage i mycosis fungoides mf. The definitive diagnosis of mf, particularly patchplaque stage disease, is challenging, as many of its clinical and pathologic features are non. The patch stage of mycosis fungoides can be difficult to distinguish from psoriasis, discoid eczema or parapsoriasis.
The earliest lesion seen in mycosis fungoides is an. 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. The name mycosis fungoides is very misleadingit loosely means mushroomlike fungal disease. Learn vocabulary, terms, and more with flashcards, games, and other study tools. In dermatology, the early stage of this disease is also referred to as parapsoriasis en plaques. Parapsoriasis refers to one of a group of skin disorders that are characterized primarily by their resemblance to psoriasis red, scaly lesions, rather than by their underlying cause. Mycosis fungoides is very difficult to diagnose, especially in early stages.
Three distinct entities are now recognized as having been included in the original concept of parapsoriasis pityriasis lichenoides, chronic superficial dermatitis small plaque parapsoriasis, digitate dermatosis, and large plaque parapsoriasis atrophic parapsoriasis, retiform parapsoriasis, patchstage mycosis fungoides. Others think its just an early stage of mycosis fungoides. What is the clinical history of classic mycosis fungoides. Skin patches were limited to sunlightcovered body areas. Large plaque parapsoriasis, a stage in the evolution of mycosis fungoides, is considered with other cutaneous. The t cells that mediate most inflammatory skin diseases belong to the skinassociated lymphoid tissue salt. The history of small or large plaque parapsoriasis spp or lpp is remarkable for its chronic indolent course sometimes for decades. Mycosis fungoides mf is the most common primary cutaneous t cell lymphoma, which is characterised in its early stages by epidermotropism of small to mediumsized t lymphocytes with cerebriform nuclei. Mycosis fungoides variantsclinicopathologic features. In 1806, mf was first described in france by alibert, who named it in 1814 pian fungoides.
Tthe conundrum of parapsoriasis versus patch stage he. The patch and plaque stages of mycosis fungoides are relatively indolent and. Mycosis fungoides nord national organization for rare. Hi, i have this spot on the back of my neck and some parts on scalp since last summer. It accounts for approximately 70 percent of cutaneous tcell lymphomas. Small plaque parapsoriasis appears as circular to oval erythematous to hyperpigmented patches or minimally elevated plaques, with lesions smaller than 5 cm in diameter and usually covered with fine scales. Jul 08, 2014 parapsoriasis describes a group of skin diseases that can be characterized by scaly patches or slightly elevated papules andor plaques red, scaly patches that have a resemblance to psoriasis. How are patches and plaques characterized in cutaneous tcell.
Large plaque parapsoriasis is considered a premalignant condition that can progress to mycosis fungoides. However, until recently, there has been no consensus on criteria that would help in diagnosing the disease early. The skin biopsies of ten patients with mycosis fungoides and nine patients with parapsoriasis were analyzed using immunohistochemistry with a panel of different antibodies for t cellassociated. They are considered to be lymphoproliferative in origin, as a number of patients progress to cutaneous tcell lymphoma or mycosis fungoides. It may mimic many benign processes, such as eczema, psoriasis and contact dermatitis. You probably dont have that, but theres no way youre going to figure that out yourself with the help of the internet. While the plaque and tumor stages are diagnosed with relative ease, the diagnosis of mf in the patch stage continues to be a vexing issue to both clinicians and pathologists alike.
If small plaque digitate parapsoriasis is a cutaneous tcell lymphoma, even an. Small plaque parapsoriasis appears as circular to oval erythematous to hyperpigmented patches or minimally elevated plaques, with lesions smaller than 5. Skin lesions include patches or plaques that may be localized or widespread, tumors, and erythroderma. The condition is roughly classified into two types.
Large plaque parapsoriasis an overview sciencedirect topics. Dermatology parapsoriasis small and large plaqueparapsoriasis en plaques small and large plaque parapsoriais, digitate dermatosis small plaque parapsoriasis, chronic superficial dermatitis small plaque parapsoriasis, retiform parapsoriasis parakeratosis variegata a subtype of large plaque parapsoriasis, xanthoerythrodermia perstans digitate type of small plaque parapsoriasis. Three distinct entities are now recognized as having been included in the original concept of parapsoriasis pityriasis lichenoides, chronic superficial dermatitis small plaque parapsoriasis, digitate dermatosis, and large plaque parapsoriasis atrophic parapsoriasis, retiform parapsoriasis, patch stage mycosis fungoides. Parapsoriasis small plaque and large plaque parapsoriasis. It describes the great variability of the clinical expression of mycosis fungoides in its early stages as well as the histopathological and immunohistochemical aspects that help with diagnosis. Patients may go for years or even decades before a definitive diagnosis of mf is established. Parapsoriasis describes a group of cutaneous diseases that can be. Large plaque parapsoriasis has an irregularly shaped, welldemarcated, and. Primary cutaneous tcell lymphoma is a heterogenous group of nonhogkins lymphomas, including mycosis fungoides mf, anaplastic large cell lymphoma, adult tcell lymphomaleukemia, subcutaneous panniculitislike tcell lymphoma, and extranodal natural killer nktcell lymphoma, nasal type, each uniquely distinguishable based on clinical presentation, immunohistochemistry, prognosis, and. Often, the first stage goes on for many years and is characterized by a nonspecific.
In early stages, its often treated with medicines or therapies that target just your skin. Mycosis fungoides rarely occurs before age 40 years. 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. A diagnosis of mycosis fungoides may be made by a thorough clinical evaluation and a variety of specialized techniques and tests including dna cytophotometry, nuclear contour analysis, and analysis of tcell receptor gene rearrangement.
Mycosis fungoides ubersicht altmeyers enzyklopadie. Mycosis fungoides and sezary syndrome are types of cutaneous tcell lymphoma. Traditionally, three stages have been described the patch, plaque, and tumor. Mf is a mature t cell nonhodgkin lymphoma with presentation in the skin but with potential involvement of the nodes, blood, and viscera. Symptoms include rash, tumors, skin lesions, and itchy skin. It is likely that a complete understanding of the pathogenesis of parapsoriasis will develop with our understanding of the pathogenesis of both chronic dermatitis and mycosis fungoides mf, because parapsoriasis appears to bridge these disorders. The disease, however, is not a fungal infection but rather a type of nonhodgkins lymphoma. The name mycosis fungoides is historical and confusing. Mycosis fungoides is a blood cancer that may need to be treated with chemotherapy or radiation.
Smallplaque parapsoriasis and largeplaque parapsoriasis are set apart. Mycosis fungoides mf is the most common form of cutaneous tcell lymphoma, with an estimated 1500 annual cases in the united states as of 2014. Poorly defined entity with overlap with mycosis fungoides. Mycosis fungoides occurs in about 1 in 100,000 to 350,000 individuals. The conundrum of parapsoriasis versus patch stage of. However, this description includes several inflammatory cutaneous diseases that are unrelated with respect to pathogenesis, histopathology, and. Mycosis fungoides in any stage may suddenly become much more aggressive, progressing rapidly to more advanced stages see the images below. Mycosis fungoides is the most common type of cutaneous tcell lymphoma, a type of lymphoma that initially occurs on the skin. Disease staging is made clinically, and diagnosis may be difficult during the early stages because several common dermatologic conditions share features with mf. Parapsoriasis genetic and rare diseases information center. It generally affects the skin, but may progress internally over time.
Parapsoriasis and pityriasis lichenoides plastic surgery key. The conundrum of parapsoriasis versus patch stage of mycosis fungoides. Five patients with type v skin were studied to describe the clinical manifestations, pathological features, and treatment response in hypopigmented mycosis fungoides hmf. Mycosis fungoides is considered to be a rare type of cancer, with only about new cases occuring in the us each year. Mycosis fungoides, also known as alibertbazin syndrome or granuloma fungoides, is the most common form of cutaneous tcell lymphoma. Terminological confusion with benign dermatosis, such as parapsoriasis en plaques, makes it dif. Mycosis fungoides is an epidermotropic ctcl that evolves through distinct disease stages of patch, plaque, and tumor, often leading to transformation in the final stages. The aim of this study was to explore markers that could help in this process.
A sign of mycosis fungoides is a red rash on the skin. The conundrum of parapsoriasis versus patch stage of mycosis. Sep 04, 2019 the close relationship between large plaque parapsoriasis and mycosis fungoides is highlighted by the detection of tox expression, a new marker that has been described to be frequently detected in the abnormal t cells in mycosis fungoides. Because mf develops slowly over several years and may have a variety of clinical presentations, including itchy patches, plaques or tumors that may be confused with common benign conditions such as eczema and psoriasis, the disease presents a. Large plaque parapsoriasis is considered by some to be a less aggressive variant of mycosis fungoides see later. Mycosis fungoides and cutaneous tcell lymphomas patient. Most often there are oval or ringshaped annular pink dry patches on covered skin. It was so named because alibert described the skin tumors of a severe case as having a mushroomlike appearance. Mycosis fungoides mf is the most common primary cutaneous t cell. Mycosis fungoides variantsclinicopathologic features, differential diagnosis, and treatment rein willemze, md, phd m ycosis fungoides mf is the most common type of cutaneous tcell lymphoma ctcl and accounts for approximately 50% of all primary cutaneous lymphomas. Most have early stage mf with localised patches and plaques, which has a favourable survival outcome, but nearly a quarter progress to late stage with tumours, erythroderma, and systemic.
Mycosis fungoides is the most common type of ctcl, representing 4462% of cases. Parapsoriasis small and large plaqueparapsoriasis en. The close relationship between large plaque parapsoriasis and mycosis fungoides is highlighted by the detection of tox expression, a new marker that has been described to be frequently detected in the abnormal t cells in mycosis fungoides. Topical nitrogen mustard therapy in patients with mycosis fungoides or parapsoriasis.
However, the skin patches that develop are very often painful, dry, scaling and red. 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. Large plaque parapsoriasis or early patch stage mycosis. The term parapsoriasis covers a large group of idiopathic cutaneous diseases characterized by asymptomatic or mildly pruritic, erythematous, scaly patches, and a chronic course. Tests that examine the skin and blood are used to detect find and diagnose mycosis fungoides and sezary syndrome. Mycosis fungoides may exhibit plaques, patches or tumours appearing singularly or as commingled lesions 1,2. Patients three hundred nine patients with mycosis fungoides registered between october 1985 and may 1997, including 89 patients with limited patches or plaques stage ia, 5 with generalized patches or plaques stage ib, 46 with skin tumors stage ic, 18 with enlarged but uninvolved lymph nodes stage ii, 18 with lymph node involvement. Erythematous patches and plaques with fine scale and tumors that anatomically favor the buttocks and sunprotected areas of the trunk. Out of the two types, lpp is considered a premalignant dermatosis with a risk of progressing to mycosis fungoides or cutaneous tcell lymphoma, while spp is a chronic benign condition.