Large plaque parapsoriasis is considered by some to be a less aggressive variant of mycosis fungoides see later. Parapsoriasis with plaques parapsoriasis with larger plaques transforms into cutaneous tcell lymphoma in about 10% of patients per decade. Symptoms include rash, tumors, skin lesions, and itchy skin. The definitive diagnosis of mf, particularly patchplaque stage disease, is challenging, as many of its clinical and pathologic features are non. Large plaque parapsoriasis has an irregularly shaped, welldemarcated, and. If small plaque digitate parapsoriasis is a cutaneous tcell lymphoma, even an. 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. Cd30positive large cell transformation of mycosis fungoides. The history of small or large plaque parapsoriasis spp or lpp is remarkable for its chronic indolent course sometimes for decades. The name mycosis fungoides is very misleadingit loosely means mushroomlike fungal disease.
However, this description includes several inflammatory cutaneous diseases that are unrelated with respect to pathogenesis, histopathology, and. They may spontaneously disappear, remain the same size, or. Lpp and its variants are closely related to the patch stage of mycosis fungoides. Mycosis fungoides is very difficult to diagnose, especially in early stages. The differences between the patients with each variation of stage i mf are as follows. Large plaque parapsoriasis an overview sciencedirect topics. Parapsoriasis describes a group of cutaneous diseases that can be. The earliest lesion seen in mycosis fungoides is an. The disease, however, is not a fungal infection but rather a type of nonhodgkins lymphoma. Mf is a mature t cell nonhodgkin lymphoma with presentation in the skin but with potential involvement of the nodes, blood, and viscera. Learn vocabulary, terms, and more with flashcards, games, and other study tools.
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. 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. It may be evident as a new, solitary nodule within a classic mycosis fungoides patch or. 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.
Terminological confusion with benign dermatosis, such as parapsoriasis en plaques, makes it dif. The patch stage of mf represents the early stages of ctcl, and the. 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. In plaque stage mycosis fungoides, the patches become thickened and may resemble psoriasis. Mycosis fungoides mf is a cutaneous tcell lymphoma. Topical nitrogen mustard therapy in patients with mycosis fungoides or parapsoriasis. Most often there are oval or ringshaped annular pink dry patches on covered skin. For unknown reasons, mycosis fungoides affects males nearly twice as often as females. 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. What is the clinical history of classic mycosis fungoides.
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. Parapsoriasis these skin problems can look similar at the skin surface, but they are different under a microscope. It may mimic many benign processes, such as eczema, psoriasis and contact dermatitis. Mycosis fungoides in any stage may suddenly become much more aggressive, progressing rapidly to more advanced stages see the images below. Treatment of patchstage mycosis fungoides with topical corticosteroids. 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. 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. In patch stage mycosis fungoides, the skin lesions are flat. The conundrum of parapsoriasis versus patch stage of. The t cells that mediate most inflammatory skin diseases belong to the skinassociated lymphoid tissue salt. Terminological confusion with benign dermatosis, such as parapsoriasis en plaques, makes it difficult to diagnose mycosis fungoides in the early patch stage. 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 and cutaneous tcell lymphomas patient. Parapsoriasis dermatologic disorders merck manuals. Article in italian pimpinelli n, santucci m, ghersetich i, brogelli l, moretti s. Parapsoriasis small and large plaqueparapsoriasis en. The name mycosis fungoides is historical and confusing. How are patches and plaques characterized in cutaneous tcell. Request pdf the conundrum of parapsoriasis versus patch stage of mycosis fungoides terminological confusion with benign dermatosis, such as parapsoriasis en plaques, makes it.
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. Others think its just an early stage of mycosis fungoides. Parapsoriasis en plaques mycosis fungoides psoriasis causes. 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. Mycosis fungoides is the most common type of cutaneous tcell lymphoma, a type of lymphoma that initially occurs on the skin. Mycosis fungoides ubersicht altmeyers enzyklopadie. Tests that examine the skin and blood are used to detect find and diagnose mycosis fungoides. Subsequent cancers, mortality, and causes of death in patients with mycosis fungoides and parapsoriasis. 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. Parapsoriasis small plaque and large plaque parapsoriasis.
It generally affects the skin, but may progress internally over time. 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. Mycosis fungoides mf is the most common type of cutaneous tcell lymphoma ctcl, accounting for 50% of them. It accounts for approximately 70 percent of cutaneous tcell lymphomas. The aim of this study was to explore markers that could help in this process. As parapsoriasis and specifically lpp is related to mycosis. In dermatology, the early stage of this disease is also referred to as parapsoriasis en plaques.
Large plaque parapsoriasis or early patch stage mycosis. Update on skin directed therapies in mycosis fungoides mycosis fungoides mf represents the majority of the primary cutaneous tcell lymphomas ctcl. Bajaj a 2018 patch, plaque, tumour mycosis fungoides. For example, it can develop into cutaneous tcell lymphoma. Mycosis fungoides variantsclinicopathologic features. The patch stage of mycosis fungoides can be difficult to distinguish from psoriasis, discoid eczema or parapsoriasis. Practical aspects regarding the histopathological diagnosis of. Parapsoriasis knowledge for medical students and physicians. Large plaque parapsoriasis, a stage in the evolution of mycosis fungoides, is considered with other cutaneous. 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. Hi, i have this spot on the back of my neck and some parts on scalp since last summer. In 1806, mf was first described in france by alibert, who named it in 1814 pian fungoides. 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. A total of 88 patients were included in 2 categories.
Mycosis fungoides may exhibit plaques, patches or tumours appearing singularly or as commingled lesions 1,2. Large plaque parapsoriasis an overview sciencedirect. Pityriasis lichenoides, large plaque parapsoriasis than 5 cm in diameter and small plaque parapsoriasis parapsoriasis presenting later with plaquestage mycosis fungoides. 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. It was so named because alibert described the skin tumors of a severe case as having a mushroomlike appearance. 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.
I first thought it was psoriasis but it now i think it could be mycosis fungoides. The patch and plaque stages of mycosis fungoides are relatively indolent and. Conventional mf begins as eczematoid or psoriasiform patches and. 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. 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. Sep 12, 2006 making a differential diagnosis between early mycosis fungoides mf and parapsoriasis is often difficult at the clinical and histological level. 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. Mycosis fungoides mycosis fungoides mf is the most common type of ctcl, accounting for 50% of all primary ctcl cases. Such description is classic and has been used to this date. Skin lesions include patches or plaques that may be localized or widespread, tumors, and erythroderma. They are considered to be lymphoproliferative in origin, as a number of patients progress to cutaneous tcell lymphoma or mycosis fungoides. Plaques are thicker, raised, usually scaly lesions.
In one study, large plaque parapsoriasis has expression of tox similar to that of mycosis fungoides. Tests that examine the skin and blood are used to detect find and diagnose mycosis fungoides and sezary syndrome. A patient with clinicopathologic features of small plaque parapsoriasis presenting later with plaquestage mycosis fungoides. Mycosis fungoides and sezary syndrome are types of cutaneous tcell lymphoma. Mycosis fungoides rarely occurs before age 40 years. The symptoms and skin biopsy findings of mf are similar to other benign skin conditions like eczema, psoriasis, parapsoriasis, or pityriasis lichenoides. Classic mycosis fungoides is divided into 3 stages. Parapsoriasis and pityriasis lichenoides plastic surgery key.
Parapsoriasis en plaque has been suggested to be an early manifestation of mycosis fungoides cutaneous tcell lymphoma. 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. Disease staging is made clinically, and diagnosis may be difficult during the early stages because several common dermatologic conditions share features with mf. 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. Histopathologic features of early patch lesions of mycosis fungoides. The pathology of cutaneous tcell lymphoma cancer network. Update on skin directed therapies in mycosis fungoides.
The term mycosis fungoides was only adopted by alibert in 1832. Mycosis fungoides mf is the most common primary cutaneous t cell. Mycosis fungoides is a blood cancer that may need to be treated with chemotherapy or radiation. Mycosis fungoides is the most common type of ctcl, representing 4462% of cases. However, the skin patches that develop are very often painful, dry, scaling and red. Tthe conundrum of parapsoriasis versus patch stage he. Large plaque parapsoriasis is considered a premalignant condition that can progress to mycosis fungoides. The conundrum of parapsoriasis versus patch stage of mycosis. Hi mona, a very interesting blog which will be sure to cause controversy.
Parapsoriasis is a term encompassing several disorders of the skin that possess a similar clinical presentation to psoriasis. Often, the first stage goes on for many years and is characterized by a nonspecific. I have always hated the term parapsoriasis because it has many variants all of which are different conditions. 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. Poorly defined entity with overlap with mycosis fungoides. Smallplaque parapsoriasis and largeplaque parapsoriasis are set apart. A sign of mycosis fungoides is a red rash on the skin. Early diagnosis of mycosis fungoides mf is important for deciding on type of therapy, prognosis and for further followup. The clinical picture of mycosis fungoides is variable and, in the early stages, is reminiscent of psoriasis vulgaris. 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.
This is associated with the histologic appearance of large, atypical cells. 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. In early stages, its often treated with medicines or therapies that target just your skin. 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. Traditionally, three stages have been described the patch, plaque, and tumor. However, until recently, there has been no consensus on criteria that would help in diagnosing the disease early. The term parapsoriasis covers a large group of idiopathic cutaneous diseases characterized by asymptomatic or mildly pruritic, erythematous, scaly patches, and a chronic course. 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 rarely cured, but some people stay in remission for a long time. Mycosis fungoides nord national organization for rare. 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. You probably dont have that, but theres no way youre going to figure that out yourself with the help of the internet. Mycosis fungoides occurs in about 1 in 100,000 to 350,000 individuals. In sezary syndrome, cancerous tcells are found in the blood.
Mycosis fungoides, also known as alibertbazin syndrome or granuloma fungoides, is the most common form of cutaneous tcell lymphoma. Parapsoriasis genetic and rare diseases information center. Small plaque parapsoriasis appears as circular to oval erythematous to hyperpigmented patches or minimally elevated plaques, with lesions smaller than 5. 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. Mycosis fungoides causes, symptoms, diagnosis, stages. The conundrum of parapsoriasis versus patch stage of mycosis fungoides. 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. The condition is roughly classified into two types. 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. The term parapsoriasis was coined originally by brocq in 1902. Actuarial diseasespecific survival of 270 patients with stage i mycosis fungoides mf.
1407 142 399 4 1188 1455 1115 467 701 92 1471 1613 911 161 1626 800 1555 528 30 237 1218 1428 113 600 693 795 1131 361 1149 495 1474 941 1011 699 807 1191 746