Large plaque parapsoriasis, a stage in the evolution of mycosis fungoides. Genotypic pattern observed in small plaque parapsoriasis is similar to that observed in chronic dermatitis, and the pattern of clonality of t cells is consistent with the response of a specific subset of t cells that have been stimulated by an antigen. Background broadband uvb phototherapy has been used for many years in the treatment of small plaque parapsoriasis spp and earlystage mycosis fungoides mf. This area in dermatopathology has been always challenging to me. These authors confirmed that largeplaque parapsoriasis is more closely related to patchstage mycosis fungoides 2, 3, 4. Mycosis fungoides lesions include flat, red, scaly patches, thicker raised lesions plaques, and sometimes larger nodules or tumors. Large plaque parapsoriasis lpp and small plaque parapsoriasis spp are, in general, diseases of middleaged and older people, with a peak incidence in the fifth decade.
They can disappear and reappear or remain stable over time. Erythematous patches with fine scales in a patient with large plaque parapsoriasis. Cutaneous tcell lymphoma presenting as benign dermatoses. Mycosis fungoides mf is the most common primary cutaneous lymphoma. Revisions to the staging and classification of mycosis.
Broadband uvb phototherapy has been used for many years in the treatment of small plaque parapsoriasis spp and earlystage mycosis fungoides mf. Apr 18, 2020 burg g, dummer r, nestle fo, doebbeling u, haeffner a. Patients presenting only with small, sometimes digitated patches, typically located on the trunk and upper extremities, are variously diagnosed as digitate dermatosis, chronic superficial scaly dermatitis, small plaque parapsoriasis, or mycosis fungoides. In some affected individuals, patches progress to plaques, the next stage of mycosis fungoides. Terminological confusion with benign dermatosis, such as parapsoriasis en plaques, makes it difficult to diagnose mycosis fungoides in the early patch stage. Narrowband 311nm uvb therapy for small plaque parapsoriasis and earlystage mycosis fungoides. The conundrum of parapsoriasis versus patch stage of. Small plaque parapsoriasis appears as circular to oval erythematous to hyperpigmented patches or minimally elevated plaques. Parapsoriasis knowledge for medical students and physicians. Patients with fmf might also notice areas of hair loss, especially around the face or scalp, pimples or blackheads, or increased infections within their plaques. Mycosis fungoides, parapsoriasis, small plaque, large plaque.
Patients may go for years or even decades before a definitive diagnosis of mf is established. Patches are most commonly found on the lower abdomen, upper thighs, buttocks, and breasts. Tthe conundrum of parapsoriasis versus patch stage he conundrum of parapsoriasis versus patch stage oof mycosis fungoidesf mycosis fungoides kk. If small plaque digitate parapsoriasis is a cutaneous tcell lymphoma, even an abortive one, it must be mycosis fungoides. Large plaque parapsoriasis an overview sciencedirect topics. Pustular mycosis fungoides small plaque parapsoriasis syringotropic mycosis fungoides unilesional solitary mycosis fungoides. Small plaque parapsoriasis is a benign disorder that rarely if ever progresses. Jan, 2019 a patient with clinicopathologic features of small plaque parapsoriasis presenting later with plaquestage mycosis fungoides. Retrospective study of 24 patients with large or small plaque parapsoriasis. Mycosis fungoides mf is the most common type of primary cutaneous tcell lymphoma. These disease processes are large plaque parapsoriasis and small plaque parapsoriasis. Ulceration, with secondary infection of tumors, is a common cause of morbidity. Vitiligolike lesions of mycosis fungoides coexisting with.
Parapsoriasis, large plaque 5minute clinical consult. Treatment of patchstage mycosis fungoides with topical corticosteroids. Idiopathic follicular mucinosis or mycosis fungoides. The conundrum of parapsoriasis versus patch stage of mycosis fungoides. In mycosis fungoides, the histopathology is characterised by infiltrates of malignant t cells. The term parapsoriasis was coined originally by brocq in 1902.
A patient with clinicopathologic features of small plaque. Our purpose was to investigate the effect on these diseases of narrowband 311nm uvb therapy, which was recently established for the treatment of psoriasis and found to be more. Topical nitrogen mustard therapy in patients with mycosis fungoides or parapsoriasis. It generally affects the skin, but may progress internally over time. Others think its just an early stage of mycosis fungoides.
They are considered to be lymphoproliferative in origin, as a number of patients progress to cutaneous tcell lymphoma or mycosis fungoides. Cutaneous lymphomas consist of a spectrum of nosologically. Association of mycosis fungoides and large plaque parapsoriasis with human herpes virus 8. It is also known as chronic superficial dermatitis, small plaque parapsoriasis and digitate dermatosis. Parapsoriasis and pityriasis lichenoides plastic surgery key. The histopathological picture may resemble mycosis fungoides, in fact, large plaque parapsoriasis does progress to frank mycosis fungoides i. Mycosis fungoides is the most common type of cutaneous lymphoma, representing almost 50% of all lymphomas arising primarily in the skin.
These authors confirmed that large plaque parapsoriasis is more closely related to patch stage mycosis fungoides 2, 3, 4. The tumor stage of disease may appear without an antecedent patch or plaque phase the demblee presentation. Folliculotropic mycosis fungoides cutaneous lymphoma. Mf is defined as an epidermotropic, primary cutaneous tcell lymphoma ctcl characterized by infiltrates of small to mediumsized t lymphocytes with cerebriform nuclei. Large plaque parapsoriasis or early patch stage mycosis. Mf is a mature t cell nonhodgkin lymphoma with presentation in the skin but with potential involvement of the nodes, blood, and viscera. Mycosis fungoides mf has an indolent lowgrade clinical course, which means that it may persist in one stage, or may slowly progress to another stage from patches to thicker plaques. Mycosis fungoides genetic and rare diseases information.
Many dermatopathologists consider large plaque parapsoriasis lpp equivalent to early patch stage mycosis fungoides. In 1953, degos divided parapsoriasis en plaques into two forms. Parapsoriasis is a peculiar reaction pattern of the skin that appears to have a spectrum with small plaque parapsoriasis at one end and large plaque at the other. Kim yh, jensen ra, watanabe gl, varghese a, hoppe rt.
Lpp and its variants are closely related to the patch stage of mycosis fungoides. Parapsoriasis is a term encompassing several disorders of the skin that possess a similar clinical presentation to psoriasis. 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. If small plaque parapsoriasis is a cutaneous t cell lymphoma, even an abortive one, it must be mycosis fungoides. Atypical dermal lymphoid infiltrate is seen along with epidermotropism. Small plaque parapsoriasis is characterized by a chronic, waxing and waning course early on, which often progresses slowly to a more persistent form. Large plaque parapsoriasis, a disease of the middleaged, and with no racial and geographical predilection, can be regarded as the clinically benign end of the mycosis fungoides. Parapsoriasis dermatologic disorders merck manuals. 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. Further, the who stated that the term mycosis fungoides should be exclusively reserved for classical cases typified by the evolution of cutaneous patches, plaques, and tumors, or for variants that show a similar clinical course. What is the pathophysiology of small plaque parapsoriasis spp. Epidemiology, etiology, genetics, and cytogenetic abnormalities.
Small plaque parapsoriasis is characterized by a chronic, waxing and waning. Early diagnosis of mycosis fungoides mf is important for deciding on type of therapy, prognosis and for further followup. It may mimic many benign processes, such as eczema, psoriasis and contact dermatitis. Large plaque parapsoriasis is considered a premalignant condition that can progress to mycosis fungoides. It can be hard for your doctor to know for sure that you have mycosis fungoides. Retrospective study of 24 patients with large or small plaque parapsoriasis treated with ultraviolet b therapy. Genotypic pattern observed in small plaque parapsoriasis is similar to that observed in chronic dermatitis, and the pattern of clonality of t cells is consistent with the response of a specific subset of t cells that have been. Large plaque parapsoriasis is considered by some to be a less aggressive variant of mycosis fungoides see later. The clinical stages patch, plaque, tumour correlate with the progressive density of malignant t cells. The symptoms and skin biopsy findings of mf are similar to other benign skin conditions like eczema, psoriasis, parapsoriasis, or. Small plaque parapsoriasis is defined by lesions plaque parapsoriasis has lesions 5 cm in diameter. This is associated with the histologic appearance of large, atypical cells.
These are small, medium in size, and characteristically have irregular cerebriform nuclei. Mycosis fungoides mf is the most common primary cutaneous t cell lymphoma ctcl, which comprise a heterogeneous group of nonhodgkins lymphoma. Its a cancer of the white blood cells that starts in the skin. Clinical stage ia limited patch and plaque mycosis fungoides. In advanced cases, ulcerated tumors and infiltration of lymph nodes by diseased cells. In individuals with mycosis fungoides, the skin becomes infiltrated with plaques and nodules that are composed of lymphocytes. Pdf association of mycosis fungoides and large plaque. Occasionally, lesions arise in childhood and may be associated with pityriasis lichenoides. Mycosis fungoides in any stage may suddenly become much more aggressive, progressing rapidly to more advanced stages see the images below. Learn vocabulary, terms, and more with flashcards, games, and other study tools. Wang, md key facts terminology primary cutaneous tcell lymphoma characterized by epidermotropism clinical course showing stepwise evolution of patches, plaques. Large plaque parapsoriasis can either coexist with mycoses fungoides, or with time, can lead to the condition. Different entities including mycosis fungoides and small plaque parapsoriasis. The disease has a protean clinical and histological presentation in its early patch and plaque stages, when distinction.
Small plaque parapsoriasis differential diagnosis psoriasis. Our purpose was to investigate the effect on these diseases of narrowband 311nm uvb therapy, which was recently established for. Mar 15, 20 mycosis fungoides mf is well known but relatively rare tcell lymphoma of the skin. If small plaque digitate parapsoriasis is a cutaneous tcell. The condition is roughly classified into two types. Parapsoriasis diffuse, slightly scaling, mildly erythematous, small patches and. The differential diagnosis between early stages of mycosis fungoides and large plaque parapsoriasis both considered a form of ctcl can be often difficult.
Spp shows a definite male predominance of approximately 3. A patient with clinicopathologic features of small plaque parapsoriasis presenting later with plaque stage mycosis fungoides. The estimated annual incidence rate in the united states is only approximately 0. Sometimes digitate plaques develop along the dermatomes, especially on the flanks and abdomen, in small plaque parapsoriasis.
The natural history of parapsoriasis en plaques and prereticulotic poikiloderma. Pityriasis lichenoides, large plaque parapsoriasis than 5 cm in diameter and small plaque parapsoriasis plaque parapsoriasis and small plaque parapsoriasis. Some people with this form develop a kind of lymphoma called mycosis fungoides. The term mf should be used only for the classical cases characterized by the evolution of patches, plaques. It affects usually the covered areas of the body in elderly males in 6th and 7th decades of life. Parapsoriasis small and large plaqueparapsoriasis en. The patches or plaques can look like eczema, psoriasis, or another common skin problem. Poorly defined entity with overlap with mycosis fungoides. Conventional mf begins as eczematoid or psoriasiform patches and.
It is defined as a tumor composed of small mediumsized, epidermotropic thelper lymphocytes but tcytotoxic variants are not uncommon and tumor cells may be mediumlarge in advanced stages. Parapsoriasis these skin problems can look similar at the skin surface, but they are different under a microscope. Large plaque parapsoriasis psoriasis en grandes plaques, see separate textbook entry presents as a small number of welldefined, reddish, scaly plaques typically measuring over 10 cm in diameter on the trunk or extremities. Large plaque parapsoriasis an overview sciencedirect. Diagnosis is based on clinical examination and histopathological findings on biopsy. Conventional mf begins as eczematoid or psoriasiform patches. Tthe conundrum of parapsoriasis versus patch stage he. The symptoms and skin biopsy findings of mf are similar to other benign skin conditions like eczema, psoriasis, parapsoriasis, or pityriasis lichenoides. Plaques of mycosis fungoides are characterized by a dense, bandlike infiltrate within the upper. Expression of cancertestis antigens in cutaneous t cell. Digitate dermatosis smallplaque parapsoriasis escholarship. Mycosis fungoides is very difficult to diagnose, especially in early stages.
Large plaque and small plaque parapsoriasis blogger. Cutaneous lymphomas consist of a spectrum of nosologically different entities including mycosis fungoides and small plaque parapsoriasis. The nonconfirmatory microscopic morphology of the premycotic stage may depict a picture akin to chronic nonspecific dermatitis accompanied by psoriasiform alteration of the epidermis. 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. 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. These disease processes are large plaque parapsoriasis and small. It is unsettled whether small plaque parapsoriasis spp represents an. It may be evident as a new, solitary nodule within a classic mycosis fungoides patch or plaque. 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 nord national organization for rare. Parapsoriasis definition, causes, signs, symptoms, diagnosis. The conundrum of parapsoriasis versus patch stage of mycosis. He felt that transformation to mycosis fungoides occurred only in large plaque parapsoriasis, with small plaque digitate parapsoriasis.
Large plaque parapsoriasis is marked by rashes that are bigger and sometimes irregularly shaped. Small plaque 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, also known as alibertbazin syndrome or granuloma fungoides, is the most common form of cutaneous tcell lymphoma. The features are very nonspecific, and distinction from early mycosis fungoides and eczema may be. 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 history of small or large plaque parapsoriasis spp or lpp is remarkable for its chronic indolent course sometimes for decades. In individuals with mycosis fungoides, the skin becomes infiltrated with plaques. Mycosis fungoides is a rare form of tcell lymphoma of the skin cutaneous. Chronic superficial scaly dermatitis cssd is an uncommon chronic skin condition characterised by round or oval red, slightly scaly patches on limbs and trunk. Patch stage of mycosis fungoides laxman mavarkar indian. Sarveswari, patrick yesudian view point how to cite this article. Hypopigmented mycosis fungoides is an uncommon variant usually observed in darkskinned individuals, especially children. 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. The full text of this article hosted at is unavailable due to technical difficulties.
Regarded by some as a precursor lesion, this probably represents an early latent stage of mycosis fungoides. Mycosis fungoides in plaque stage with pronounced eosinophilic infiltration, folliculotropism, and concomitant invasive squamous cell carcinoma. Large plaque parapsoriasis and mycosis fungoides jones robert e. Because of this ongoing controversy and the inconsistent use of the terms large plaque parapsoriasis and small plaque.