Sections

Presentation

History

The skin rash of mycosis fungoides may consist of patches, plaques, or tumors, which may have a long natural history. The median duration from the onset of skin symptoms to diagnosis is 6 years. Early in the course of mycosis fungoides, as well as in erythrodermic cases, the skin lesions may be nonspecific, with a nondiagnostic biopsy result, so that confusion with benign conditions is common (eg, eczema, neurodermatitis, pseudolymphoma syndrome). Obtain repeated biopsies in patients who have progressive chronic dermatosis or whose condition is refractory to topical treatments.

In patients who present with pruritus or erythroderma, the diagnosis of mycosis fungoides is often made possible through the examination of a noncutaneous site (eg, blood, lymph nodes).

Classic mycosis fungoides

Classic mycosis fungoides is divided into 3 stages: patch (atrophic or nonatrophic), plaque, and tumor. 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. Sometimes, these patches have a thin, wrinkled quality, often with reticulated pigmentation. In this stage, pruritus is usually minimal or absent.^[60]

Classic mycosis fungoides is usually preceded by a nonspecific, indolent inflammatory process manifesting as atopic dermatitis, nonspecific chronic dermatitis, or parapsoriasis (most commonly large-plaque parapsoriasis), which may progress over years to decades to early plaque-stage mycosis fungoides. Some regard large-plaque parapsoriasis as patch-stage mycosis fungoides. (See the images below.)

Plaque-stage parapsoriasis.

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Patch-stage mycosis fungoides progressing to plaque stage, with cutaneous cigarette-paper appearance evident.

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In many cases, the disease never progresses beyond this stage, and the diagnosis of mycosis fungoides is never confirmed. In other cases, the disease appears from the beginning as rather well-defined, superficial plaques that range from 2 cm to more than 20 cm in greatest diameter. In children, early stage disease and unusual forms, such as the hypopigmented variant, tend to predominate.^[61]

Stages IA and IB

While well-developed plaques that are clinically diagnostic for mycosis fungoides are usually intensely pruritic, less characteristic ones typically are not, and the development of pruritus in such lesions is a sign of progression towards mycosis fungoides. Depending on whether the lesions involve up to 10% of the body surface or involve 10% or more of the body surface, such cases are classified as stage IA or IB, respectively. Many cases remain at these stages for many years or decades without further progression.

Stages IIA and IIB

Clinical lymphadenopathy may develop (stage IIA), sometimes with progression of the plaques to form tumors (stage IIB), or tumors may form from plaques in the absence of lymphadenopathy (stage IIB). Either process usually takes years, or even decades, to develop. Once tumors form, they are prone to ulceration.

D'embleemycosis fungoides

The sudden multifocal development of tumors of apparent mycosis fungoides may rarely occur without preceding patches or plaques. Most, if not all, of such cases probably represent primary cutaneous CD30⁺ pleomorphic, medium or large cell T-cell lymphomas.

Stage III (erythrodermicmycosis fungoides)

Mycosis fungoides that is evident as an erythroderma but with too few circulating lymphocytes to warrant a diagnosis of Sézary syndrome is designated erythrodermic mycosis fungoides. Dermatopathic lymphadenopathy is present in these cases. Rarely, such patients may present with a nodulotumorous eruption.^[62]

Stages IVA and IVB

Development of lymph nodes that are histologically positive for tumor (stage IVA) and/or visceral lesions (stage IVB) may occur rather rapidly after tumor-stage disease develops and/or clinical lymphadenopathy is detected. Alternatively, either or both may arise from erythrodermic disease (stage III) at a very variable rate. Both are associated with a poor prognosis.

Transformation ofmycosis fungoides

Mycosis fungoides in any stage may suddenly become much more aggressive, progressing rapidly to more advanced stages (see the images below). This is associated with the histologic appearance of large, atypical cells; often, these are CD30⁺, and the process is termed large cell transformation. It may be evident as a new, solitary nodule within a classic mycosis fungoides patch or plaque, as abrupt onset of multiple scattered papules and/or nodules without spontaneous resolution, or within new or enlarging tumors.^[63]

Middle-aged woman with mycosis fungoides showing ulceration and marked depigmentation of advanced disease.

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Middle-aged woman with mycosis fungoides showing ulceration and marked depigmentation of advanced disease.

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Variants of mycosis fungoides

Pagetoid reticulosis

Pagetoid reticulosis arises preferentially on acral skin. The histologic hallmark of the disease is the pagetoid spread of haloed lymphoid cells in the epidermis.

Patients with localized pagetoid reticulosis are usually first seen with a solitary psoriasiform or hyperkeratotic patch or plaque, which is usually localized on the extremities; it is slowly progressive. In contrast to classic mycosis fungoides, extracutaneous dissemination and disease-related deaths rarely occur. (Ketron-Goodman disease, which is multilesional, has a clinical course similar to that of mycosis fungoides. Some cases may actually represent primary cutaneous epidermotropic CD8⁺ (cytotoxic) T-cell lymphoma.)

Folliculotropic mycosis fungoides

Folliculotropic mycosis fungoides is commonly first evident clinically with alopecia, follicular cysts, or comedolike lesions and is usually associated with follicular mucinosis and strong epidermotropism.^[64]It is most commonly seen on the head and neck, often showing infiltrated plaques together with acneiform comedolike papules, epidermal cysts, and keratosis pilaris–like papules.^[65]When mucin is present, the disease is also called alopecia mucinosa.

However, the benign form of alopecia mucinosa, which is not associated with mycosis fungoides, must be distinguished from mycosis fungoides associated with mucinosis. The most relevant feature, with and without associated follicular mucinosis, is the deep follicular and perifollicular localization of the neoplastic infiltrates, which makes them less accessible to skin-targeted therapies.

Hypopigmented mycosis fungoides

Hypopigmented mycosis fungoides tends to occur in young, slightly to moderately dark-skinned people of Indian, Latin American, or sub-Saharan African American heritage. It manifests as irregular, but fairly well-demarcated, hypopigmented or white patches. They are asymptomatic or are slightly pruritic and may appear with or without other lesions typical of mycosis fungoides. (See the images below.)

Hypopigmented cutaneous T-cell lymphoma. Courtesy of Jeffrey Meffert, MD.

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Hypopigmented cutaneous T-cell lymphoma. Courtesy of Jeffrey Meffert, MD.

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Pustular mycosis fungoides

A granulomatous reaction pattern occasionally is seen in mycosis fungoides and its variants. Pustular mycosis fungoides also can occur; it is often limited to the palms, but the lesions may occur elsewhere.

Bullous mycosis fungoides

Bullous mycosis fungoides manifests with flaccid, tense, or ruptured bullae arising on normal skin or an erythematous base or within typical patch- or plaque-stage lesions of mycosis fungoides. It tends to arise on the trunk and extremities. Rarely, it may clinically resemble pemphigus vulgaris or even erythema multiforme.

Hyperpigmented mycosis fungoides

Hyperpigmented mycosis fungoides is diffuse macular hyperpigmentation accompanied by typical mycosis fungoides, although in rare cases the hyperpigmentation may be the only manifestation. These lesions may resemble ashy dermatosis or may appear as more or less well-defined macules. Ultrastructural studies have revealed atypical lymphocytes and keratinocytes, macrophages, and Langerhans cells that contain giant melanosomes within lysosomes.

Unilesional mycosis fungoides

Unilesional mycosis fungoides manifests as a single area of otherwise typical mycosis fungoides that, by definition, manifests as a single lesion. Histologic changes are identical to those that occur with multiple disseminated lesions of mycosis fungoides. The prognosis is excellent following treatment, although it may recur after surgical excision.

Syringotropic mycosis fungoides

In addition to hair follicles, atypical cells in mycosis fungoides may rarely be tropic to eccrine glands. In the even rarer syringotropic mycosis fungoides (syringolymphoid hyperplasia), these are the principal or only lesions observed. The eccrine duct and the eccrine gland are typically involved and eccrine epithelium may appear hyperchromatic and atypical, mimicking eccrine carcinoma. Lesions manifest as red to skin-colored papules, red to brown patches, or red scaly plaques. Hair loss without mucinous degeneration in the affected areas is common. Most reported cases have been in men; in one series, only 4 of 14 patients were female.^[66]

Poikilodermic mycosis fungoides

Poikilodermic mycosis fungoides occurs when poikiloderma (ie, poikiloderma vasculare atrophicans, which is a combination of atrophic, dry, dyspigmented skin and telangiectasia) develops in cases of otherwise typical mycosis fungoides; this is not an infrequent occurrence. Occasionally, it may predominate or even be the only presenting manifestation of the disease. It may rarely involve skin over the entire body.

Granulomatous slack skin syndrome

Granulomatous slack skin syndrome is a distinct subtype of mycosis fungoides that is characterized by ponderous, more-or-less infiltrated folds of skin that arise slowly in intertriginous areas, especially the axillae and groin. Granulomatous slack skin syndrome may give rise to Hodgkin disease.

Additional variants and characteristics

Other variants of mycosis fungoides include hyperkeratotic/verrucous and vegetating/papillomatous mycosis fungoides, typically arising in the axillae, perineum, and cervical area (neck), as well as, sometimes, on the breasts near the areolae, resembling acanthosis nigricans or multiple seborrheic keratosis.

Persistent, pigmented, purpuralike to lichenoid processes also may be a manifestation of mycosis fungoides.

Mucosal involvement by mycosis fungoides is rare and may occur as part of generalized involvement in advanced cases, particularly those that have undergone large cell transformation; it is a poor prognostic sign.

Sézary syndrome

The combination of erythroderma and leukemia is defined as Sézary syndrome. However, different clinicians use different criteria regarding the number of circulating atypical lymphocytes sufficient to warrant this diagnosis. According to some, the diagnosis is established in an erythrodermic patient if more than 5% of peripheral lymphocytes are atypical. Others use the absolute number of atypical lymphocytes in the peripheral blood (>1000/µL). In obvious cases, some use a quick and easy criterion of greater than 10 CD4⁺ T cells for every CD8⁺ T cell.

Lymphadenopathy is usually present, and the skin itself is usually edematous. Other frequently observed changes include palmar and/or plantar hyperkeratosis, alopecia, nail dystrophy, and ectropion. Hepatosplenomegaly may be present. As in other forms of mycosis fungoides, a nonspecific dermatitis and/or pruritus may precede the disease. Transformation of the disease to a more aggressive form is common. It may occur in lymph nodes even as skin lesions are showing improvement or a response to treatment.

Physical Examination

The following signs of cutaneous T-cell lymphoma are discussed in this section:

Patches and plaques
Skin tumors
Erythroderma (exfolliative dermatitis)
Lymphadenopathy

Ocular involvement may be evident in advanced cutaneous T-cell lymphoma.^[4]Direct tumor infiltration may produce or contribute to corneal ulceration in such patients.

Cutaneous patches and plaques

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). In dark-skinned individuals, the patches may appear as hypopigmented or hyperpigmented areas. As the patches become infiltrative, they evolve into palpable plaques.

Patch-stage mycosis fungoides.

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The plaques tend to be raised, demonstrating fine-scale, well-demarcated, erythematous shapes with irregular borders. Annular or serpiginous patterns with central clearing and pruritus are common. (See the images below.)

Plaque-stage mycosis fungoides.

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Partially confluent, erythematous plaques in advancing mycosis fungoides.

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Close-up view of advancing plaque-stage mycosis fungoides with partially confluent, erythematous plaques.

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Patches and plaques may affect any area of the skin, but they are often distributed asymmetrically in the sun-protected areas that a bathing suit would cover (ie, hips, buttocks, groin, lower trunk, axillae, breasts). When mycosis fungoides affects the scalp, it is often accompanied by alopecia.

Skin tumors

Patients with evidence or a history of patchy or plaquelike skin lesions can also have tumors, which are red-violet nodules that may be dome-shaped, exophytic, or ulcerated. However, if only tumors are present, without preceding or concurrent patches or plaques, a diagnosis of mycosis fungoides is highly unlikely and another type of cutaneous T-cell lymphoma should be considered.^{[29, 2]}(See the images below.)

Tumor-stage cutaneous T-cell lymphoma.

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Tumor-stage mycosis fungoides.

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Skin erythroderma

Sézary syndrome is characterized by erythroderma, which is often associated with marked pruritus and exfoliation, edema, and lichenification. Lymphadenopathy, onychodystrophy, and palmoplantar hyperkeratosis are also commonly associated. In addition, patients experience thickening of the facial skin folds (leonine facies) and ectropion of the eyelids. Sun exposure may be painful, as well as pruritic. (See the image below.)

Erythroderma of Sézary syndrome.

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A few patients with Sézary syndrome have patchy, total-scalp, or universal alopecia. Follicular mycosis fungoides may present with alopecia; total-body hair loss may be evident in some patients with generalized erythroderma and Sézary syndrome.^[67]The alopecia may also appear clinically identical to alopecia areata. However, skin biopsy specimens may reveal atypical T lymphocytes within the follicular epithelium or epidermis, sometimes with follicular mucinosis.

Lymphadenopathy

Extracutaneous involvement in mycosis fungoides becomes more clinically evident as the stages and extent of the disease progress. Peripheral lymphadenopathy is the most frequent site of extracutaneous involvement in mycosis fungoides.

Evaluate palpable lymphadenopathy by obtaining a biopsy, because the result influences the patient's stage, prognosis, and treatment.

Other signs of cutaneous T-cell lymphoma

Granulomatous slack skin syndrome shows circumscribed areas of pendulous, lax skin with a predilection for the axillae and groin. An association with Hodgkin lymphoma or with classic mycosis fungoides may be apparent.^[68]Most patients have an indolent clinical course.

Acute adult T-cell leukemia/lymphoma is characterized by the presence of leukemia, lymphadenopathy, organomegaly, hypercalcemia, and, in approximately 50% of cases, skin lesions. Skin lesions most commonly include nodules or tumors (33%), generalized papules (22%), or plaques (19%).^[44]Chronic and smoldering variants frequently manifest as skin lesions that closely resemble mycosis fungoides, whereas circulating neoplastic T cells are few or absent.

Subcutaneous panniculitis-like T-cell lymphoma is a rare form of cutaneous T-cell lymphoma. Patients are usually first seen with solitary or multiple nodules and plaques; these mainly involve the legs, although they may be more generalized. Ulceration is uncommon, but systemic symptoms such as fever, fatigue, and weight loss may be present. A hemophagocytic syndrome may be present and is generally associated with a rapidly progressive course.^[69]Dissemination to extracutaneous sites is unusual. Subcutaneous panniculitis-like T-cell lymphoma may be preceded for years or decades by a seemingly benign panniculitis suggestive of chronic erythema nodosum. Rarely, it may produce scalp alopecia.^[70]

Primary cutaneous peripheral T-cell lymphomas, unspecified, are a heterogeneous group of diseases for which the common characteristic is a lack of typical features of mycosis fungoides. Cutaneous gamma/delta-positive T-cell lymphoma,^[30]which belongs to this group,^{[2, 31]}usually has an aggressive course and manifests with disseminated plaques and/or ulceronecrotic nodules or tumors, particularly on the extremities.^[59]

Primary cutaneous aggressive epidermotropic CD8⁺ cytotoxic T-cell lymphoma, a provisional entity, is characterized by localized or disseminated eruptive papules, nodules, and tumors that show central ulceration and necrosis or superficial, hyperkeratotic patches and plaques.^{[38, 71]}

Primary cutaneous CD4⁺ small/medium T-cell lymphoproliferative disorder tends to be first apparent as a solitary plaque or tumor, generally on the face, neck, or upper trunk, although it may less commonly appear as 1 or several papules, nodules, or tumors.^{[72, 55]}

Nasal-type extranodal NK/T-cell lymphoma usually appears as a destructive midfacial tumor or as multiple plaques or tumors, often with ulceration, preferentially on the trunk and extremities. Systemic symptoms such as fever, malaise, and weight loss may be present, and some cases are accompanied by a hemophagocytic syndrome.

Nasal-type extranodal NK/T-cell lymphoma has a variant that clinically resembles hydroa vacciniforme in which children, mainly in Latin America and Asia, have a papulovesicular eruption that typically occurs on sun-exposed areas, such as the face and upper extremities.^{[73, 74]}

Differential Diagnoses

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Media Gallery

Patch-stage mycosis fungoides.
Plaque-stage mycosis fungoides.
A 40-year-old woman complained of a recurrent skin rash, which she described as "bug bites." Skin biopsy results demonstrated an atypical lymphoid infiltrate that was CD30 positive. The clinical picture and pathologic findings were consistent with lymphomatoid papulosis. This condition has a benign natural history, despite clonal gene rearrangement in some cases. Skin eruptions occur in self-limited crops, which do not require treatment.
Erythroderma of Sézary syndrome.
Nail changes of Sézary syndrome.
Electron micrograph of a Sézary cell.
Tumor-stage mycosis fungoides.
Tumor-stage cutaneous T-cell lymphoma.
Patch-stage cutaneous T-cell lymphoma. Courtesy of Jeffrey Meffert, MD.
Plaque-stage parapsoriasis.
Patch-stage mycosis fungoides progressing to plaque stage, with cutaneous cigarette-paper appearance evident.
Partially confluent, erythematous plaques in advancing mycosis fungoides.
Close-up view of advancing plaque-stage mycosis fungoides with partially confluent, erythematous plaques.
Early patch-stage cutaneous T-cell lymphoma.
Hypopigmented cutaneous T-cell lymphoma. Courtesy of Jeffrey Meffert, MD.
Hypopigmented cutaneous T-cell lymphoma. Courtesy of Jeffrey Meffert, MD.
Nodular mycosis fungoides.
Middle-aged woman with mycosis fungoides showing ulceration and marked depigmentation of advanced disease.
Middle-aged woman with mycosis fungoides showing ulceration and marked depigmentation of advanced disease.
Middle-aged woman with mycosis fungoides showing ulceration and marked depigmentation of advanced disease.
Middle-aged woman with mycosis fungoides showing ulceration and marked depigmentation of advanced disease.
Mycosis fungoides with large, atypical T lymphocytes in the epidermis and dermis.
Mycosis fungoides with an epidermal nest of large, atypical T cells displaying bizarre, convoluted nuclei surrounded by a clear space (Pautrier microabscess).
A 42-year-old woman who had moved to the United States from Peru several year ago presented with an ulcerative lesion on her face near her nose, and destruction of her hard palate. Tissue biopsy revealed NK/T-cell lymphoma. Image courtesy of Jason Kolfenbach, MD, and Kevin Deane, MD, Division of Rheumatology, University of Colorado Denver School of Medicine.

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Table 1. WHO-EORTC Classification of Cutaneous T-Cell Lymphoma

WHO-EORTC Classification	Frequency (%)	5-Year Survival Rate (%)
Indolent Clinical Behavior
Mycosis fungoides	39	88
Mycosis fungoides variants and subtypes
Folliculotropic mycosis fungoides	5	75
Pagetoid reticulosis	< 1	100
Granulomatous slack skin	< 1	100
Primary cutaneous CD30⁺ lymphoproliferative disorder
Primary cutaneous anaplastic large cell lymphoma	8	95
Lymphomatoid papulosis	12	100
Subcutaneous panniculitis-like T-cell lymphoma (provisional)	1	82
Primary cutaneous CD4⁺ small/medium-sized pleomorphic T-cell lymphoma (provisional)	2	75
Aggressive Clinical Behavior
Sézary syndrome	2%	36%
Adult T-cell leukemia/lymphoma	< 1	NDA
Extranodal NK/T-cell lymphoma, nasal type	< 1	16
Primary cutaneous peripheral T-cell lymphoma, unspecified	2	15
Primary cutaneous peripheral T-cell lymphoma, rare subtypes
Primary cutaneous acral CD8⁺ T-cell lymphoma (provisional)	< 1	100
Primary cutaneous CD8+ aggressive epidermotropic cytotoxic T-cell lymphoma (provisional)	< 1	31
Primary cutaneous gamma/delta-positive T-cell lymphoma	< 1	11
Source: Adapted from Willemze et al. Blood. 2019;133(16):1703-14.^[3] EORTC = European Organization of Research and Treatment of Cancer; NDA = no data available; NK = natural killer; WHO = World Health Organization.

Table 2. Cluster Designations

CD Type	Representative Cells	Also Known As
CD2	T, NK	Sheep RBC
CD3	T
CD4	T subset	Helper
CD5	T
CD7	T, NK	Prothymocyte
CD8	T subset, NK	Suppressor
CD25	Active T, B, M	IL-2R (Tac)
CD30	Active T, B	Ki-1
CD45	T subset	CLA
CD56	NK	NCAM
B = B cell; CD = cluster designation; CLA = common leukocyte antigen; IL-2R = interleukin-2 receptor; M = M cell; NCAM = neural cell adhesion molecule; NK = natural killer; RBC = red blood cell; T = T cell; Tac = Tac antigen.

Table 3. Staging of Cutaneous T-Cell Lymphoma

Stage	Skin Lesions			Lymphadenopathy	Erythroderma	Histology
Stage	Patches/ plaques (less than10%)	Plaques/ plaques (10% or more)	Tumors	Lymphadenopathy	Erythroderma	Lymph Nodes	Viscera
IA	+	-	-	-	-	-	-
IB	+ or -	+	-	-	-	-	-
IIA	+ or -	+ or -	+ or -	+	-	-	-
IIB	+ or -	+ or -	+	+ or -	-	-	-
III	+ or -	+ or -	+ or -	+ or -	+	-	-
IVA	+ or -	+ or -	+ or -	+ or -	+ or -	+	-
IVB	+ or -	+ or -	+ or -	+ or -	+ or -	+ or -	+

Table 4. TNMB Staging of Cutaneous T-Cell Lymphoma

Stage Class	Stage	Definition
T (tumor)	T₁	Patches/plaques involving less than 10% of body surface
	T₂	Patches/plaques involving 10% or more of body surface
	T₃	Tumor(s) present on skin
	T₄	Erythroderma
N (nodes)	N₀	No enlarged lymph node present
	N₁	Enlarged lymph nodes, histologically uninvolved
	N₂	No enlarged lymph node; 1 or more nodes histologically involved*
	N₃	Enlarged lymph nodes, histologically involved
M (metastasis to viscera)	M₀	No visceral lesion present
M (metastasis to viscera)	M₁	Visceral involvement
B (blood involvement)	B₀	Circulating atypical lymphocytes (Sézary cells) less than 5% of lymphocytes
B (blood involvement)	B₁	Circulating atypical lymphocytes 5% or more of lymphocytes (Sézary syndrome)
*Uncommon finding, usually not considered/investigated.

Table 5. Comparison of Staging Systems for CTCL

Clinical Stage	TNM (B) Stage
IA	T₁ N₀ M₀
IIB	T₂ N₀ M₀
IIA	T₁ N₁ M₀	T₂ N₁ M₀
IIB	T₃ N₀ M₀	T₃ N₁ M₀
III	T₄ N₀ M₀	T₄ N₁ M₀
IVA	T₁ N₂ M₀	T₂ N₂ M₀	T₃ N₂ M₀	T₄ N₂ M₀
IVA	T₁ N₃ M₀	T₂ N₃ M₀	T₃ N₃ M₀	T₄ N₃ M₀
IVB	T₁ N₀ M₁	T₂ N₀ M₁	T₃ N₀ M₁	T₄ N₀ M₁
	T₁ N₁ M₁	T₂ N₁ M₁	T₃ N₁ M₁	T₄ N₁ M₁
	T₁ N₂ M₁	T₂ N₂ M₁	T₃ N₂ M₁	T₄ N₂ M₁
	T₁ N₃ M₁	T₂ N₃ M₁	T₃ N₃ M₁	T₄ N₃ M₁

Table 4. International Society for Cutaneous Lymphoma/European Organization for Research and Treatment of Cancer tumor-node-metastasis-blood revised classification for mycosis fungoides and Sezary syndrome

Skin	Involvement	Node	Involvement	Viscera	Involvement
T1	Patchy or plaquelike skin disease involving ≤10% of the skin surface area	N0	No abnormal lymph nodes	M0	No visceral organ involvement
T2	Patchy or plaquelike skin disease involving ≥10% of the skin surface area	N1	Histopathology Dutch Gr 1 or NCI LN 0-2	M1	Visceral organ involvement
T3	Tumors are present ≥1 cm in diameter	N2	Histopathology Dutch Gr 2 or NCI LN 3	MX	Abnormal visceral site; no histologic confirmation
T4	Erythroderma ≥80% of body area	N3	Histopathology Dutch Gr 3-4 or NCI LN 4	Blood	Involvement
		Nx	Abnormal lymph nodes; no histologic confirmation	B0	≤5% of peripheral blood lymphocytes are Sezary cells
				B1	>5% of peripheral blood lymphocytes are Sezary cells but do met B2 criteria
				B2	≥1000/mcL Sezary cells or CD4/CD8 ≥10 or ≥40% CD4+/CD7- or ≥30% CD4+/CD26- cells

Table 5. Staging classifications for mycosis fungoides and Sezary syndrome

Clinical Stage	5-Year Survival ^[131]	TNM (B) Stage
IA	98%	T₁N₀M₀B₀	T₁N₀M₀B₁
IB	89%	T₂N₀M₀B₀	T₂N₀M₀B₁
IIA	89%	T₁N₁M₀B₀	T₁N₁M₀B₁	T₁N₂M₀B₀	T₁N₂M₀B₁
IIA		T₂N₁M₀B₀	T₂N₁M₀B₁	T₂N₂M₀B₀	T₂N₂M₀B₁
IIB	56%	T₃N₀M₀B₀	T₃N₀M₀B₁	T₃N₁M₀B₀	T₃N₁M₀B₁
IIB		T₃N₂M₀B₀	T₃N₂M₀B₁
IIIA	54%	T₄N₀M₀B₀	T₄N₁M₀B₀	T₄N₂M₀B₀
IIIB	48%	T₄N₀M₀B₁	T₄N₁M₀B₁	T₄N₂M₀B₁
IVA1	41%	T₁N₀M₀B₂	T₂N₀M₀B₂	T₃N₀M₀B₂	T₄N₀M₀B₂
		T₁N₁M₀B₂	T₂N₁M₀B₂	T₃N₁M₀B₂	T₄N₁M₀B₂
		T₁N₂M₀B₂	T₂N₂M₀B₂	T₃N₂M₀B₂	T₄N₂M₀B₂
IVA2	23%	T₁N₃M₀B₀	T₂N₃M₀B₀	T₃N₃M₀B₀	T₄N₃M₀B₀
		T₁N₃M₀B₁	T₂N₃M₀B₁	T₃N₃M₀B₁	T₄N₃M₀B₁
		T₁N₃M₀B₂	T₂N₃M₀B₂	T₃N₃M₀B₂	T₄N₃M₀B₂
IVB	18%	T₁N₀M₁B₀	T₂N₀M₁B₀	T₃N₀M₁B₀	T₄N₀M₁B₀
		T₁N₁M₁B₁	T₂N₁M₁B₁	T₃N₁M₁B₁	T₄N₁M₁B₁
		T₁N₂M₁B₂	T₂N₂M₁B₂	T₃N₂M₁B₂	T₄N₂M₁B₂
		T₁N₃M₁B₃	T₂N₃M₁B₃	T₃N₃M₁B₃	T₄N₃M₁B₃

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Author

Lauren C Pinter-Brown, MD Director of Lymphoma Program, Clinical Professor of Medicine, Department of Internal Medicine, Division of Hematology and Oncology, University of California, Los Angeles, Medical Center

Lauren C Pinter-Brown, MD is a member of the following medical societies: Alpha Omega Alpha, American College of Physicians, American Society of Hematology, SWOG

Disclosure: Nothing to disclose.

Coauthor(s)

Robert A Schwartz, MD, MPH Professor and Head of Dermatology, Professor of Pathology, Professor of Pediatrics, Professor of Medicine, Rutgers New Jersey Medical School

Robert A Schwartz, MD, MPH is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, New York Academy of Medicine, Royal College of Physicians of Edinburgh, Sigma Xi, The Scientific Research Honor Society

Disclosure: Nothing to disclose.

Chief Editor

Emmanuel C Besa, MD Professor Emeritus, Department of Medicine, Division of Hematologic Malignancies and Hematopoietic Stem Cell Transplantation, Kimmel Cancer Center, Jefferson Medical College of Thomas Jefferson University

Emmanuel C Besa, MD is a member of the following medical societies: American Association for Cancer Education, American Society of Clinical Oncology, American College of Clinical Pharmacology, American Federation for Medical Research, American Society of Hematology, New York Academy of Sciences

Disclosure: Nothing to disclose.

Additional Contributors

Francisco J Hernandez-Ilizaliturri, MD Professor of Medicine, Department of Medical Oncology, Associate Professor of Immunology, Department of Immunology, Chief, Lymphoma and Myeloma Section, Director, The Lymphoma Translational Research Program, Roswell Park Cancer Institute, University of Buffalo State University of New York School of Medicine and Biomedical Sciences

Francisco J Hernandez-Ilizaliturri, MD is a member of the following medical societies: American Association for Cancer Research, American Society of Hematology

Disclosure: Nothing to disclose.

Priyank P Patel, MD Hematology/Oncology Fellow, Roswell Park Cancer Institute, University

Priyank P Patel, MD is a member of the following medical societies: American Society of Hematology, American Society of Clinical Oncology, European Society for Medical Oncology, American College of Physicians

Disclosure: Nothing to disclose.

Acknowledgements

Günter Burg, MD Professor and Chairman Emeritus, Department of Dermatology, University of Zürich School of Medicine; Delegate of The Foundation for Modern Teaching and Learning in Medicine Faculty of Medicine, University of Zürich, Switzerland

Günter Burg, MD is a member of the following medical societies: American Academy of Dermatology, American Dermatological Association, International Society for Dermatologic Surgery, North American Clinical Dermatologic Society, and Pacific Dermatologic Association

Disclosure: Nothing to disclose

David F Butler, MD Professor of Dermatology, Texas A&M University College of Medicine; Chair, Department of Dermatology, Director, Dermatology Residency Training Program, Scott and White Clinic, Northside Clinic

David F Butler, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American Medical Association, American Society for Dermatologic Surgery, American Society for MOHS Surgery, Association of Military Dermatologists, and Phi Beta Kappa