Cutaneous T-Cell Lymphoma Clinical Presentation

  • Author: Robert A Schwartz, MD, MPH; Chief Editor: Dirk M Elston, MD   more...
 
Updated: Mar 22, 2012
 

History

Classic MF

Classic cutaneous T-cell lymphoma (CTCL) or mycosis fungoides (MF) 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 usually consisting of patches, often 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.

Classic MF 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 MF. Some authorities regard large-plaque parapsoriasis as patch-stage MF. In many cases, the disease never progresses beyond this stage, and the diagnosis of MF 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.[19] Note the images below.

Early patch-stage cutaneous T-cell lymphoma. Early patch-stage cutaneous T-cell lymphoma. Patch-stage cutaneous T-cell lymphoma. Courtesy ofPatch-stage cutaneous T-cell lymphoma. Courtesy of Jeffrey Meffert, MD. Plaque-stage parapsoriasis. Plaque-stage parapsoriasis. Patch-stage mycosis fungoides progressing to plaquPatch-stage mycosis fungoides progressing to plaque stage with cutaneous cigarette-paper appearance evident.

Stages IA and IB

While well-developed plaques that are clinically diagnostic for MF are usually intensely pruritic, less characteristic ones typically are not, and the development of pruritus in such lesions is a sign of progression towards MF. Depending on whether the lesions involve up to 10% 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'emblee MF

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

Stage IVA and stage IVB

Development of lymph nodes 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 of MF

MF in any stage may suddenly become much more aggressive, progressing rapidly to more advanced stages. 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 MF patch or plaque, as abrupt onset of multiple scattered papules and/or nodules without spontaneous resolution, or within new or enlarging tumors.[20]

Pagetoid reticulosis, localized (Woringer-Kolopp) type

Patients with this condition 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 MF, extracutaneous dissemination or disease-related deaths rarely occur.

Multilesional pagetoid reticulosis (Ketron-Goodman disease) has a clinical course similar to MF and is regarded as a variant of MF. Some cases may actually represent primary cutaneous epidermotropic CD8+ (cytotoxic) T-cell lymphoma.

Erythrodermic (stage III) MF

MF evident as an erythroderma but with too few circulating lymphocytes to warrant a diagnosis of SS is designated erythrodermic MF. Dermatopathic lymphadenopathy is present in these cases. Rarely, such patients may present with a nodulotumorous eruption.[21]

See the images below.

Erythematous partially confluent plaques in advancErythematous partially confluent plaques in advancing mycosis fungoides. Close-up view of advancing plaque-stage mycosis fuClose-up view of advancing plaque-stage mycosis fungoides with partially confluent erythematous plaques.

Variants of MF

Folliculotropic MF (FMF) is commonly first evident clinically with alopecia, follicular cysts, or comedolike lesions and is usually associated with follicular mucinosis and strong epidermotropism.[22] 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.[23] When mucin is present, the disease is also called alopecia mucinosa. However, the benign form of alopecia mucinosa, not associated with MF, must be distinguished from MF 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 MF 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 MF. Note the images below.

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

A granulomatous reaction pattern occasionally is seen in MF and its variants.

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

Pustular MF is often limited to the palms, but the lesions may occur elsewhere.

Hyperpigmented MF is diffuse macular hyperpigmentation accompanied by typical MF, or, rarely, 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 MF manifests as a single area of otherwise typical MF that, by definition, comprises a single lesion. Histological changes are identical to those that occur with multiple disseminated lesions of MF. The prognosis is excellent following treatment, although it may recur after surgical excision. In addition to hair follicles, atypical cells in MF may rarely be tropic to eccrine glands. In the even rarer syringotropic MF (syringolymphoid hyperplasia), these are the principal or only lesions observed. Both 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; only 4 of 14 cases were women in one series.[24]

Pagetoid reticulosis (Woringer-Kolopp disease) arises preferentially on acral skin as a single, slowly growing psoriasiform plaque. Dissemination or extracutaneous manifestation does not occur. The histologic hallmark of the disease is the pagetoid spread of haloed lymphoid cells in the epidermis.

Poikilodermic MF is when poikiloderma (ie, poikiloderma vasculare atrophicans, which is a combination of atrophic, dry, dyspigmented skin and telangiectasia) develops in cases of otherwise typical MF; 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.

Other variants of MF include hyperkeratotic/verrucous and vegetating/papillomatous MF, typically arising in the axillae, perineum, cervical area (neck), and 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 MF.

Mucosal involvement by MF 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.

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

Sézary syndrome

The combination of erythroderma and leukemia is defined as SS. 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 MF, 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.

Extranodal NK/T-cell lymphoma, nasal type

Extranodal nasal-type NK/T-cell lymphoma is an EBV-positive lymphoma of small, medium, or large cells with an NK-cell or cytotoxic T-cell phenotype.[25]

Next

Physical

Mycosis fungoides (MF) is a commonly epidermotropic cutaneous T-cell lymphoma (CTCL) characterized by small-to-medium T lymphocytes with cerebriform nuclei. The term MF is used only for the classic Alibert-Bazin type characterized by the evolution of patches, plaques, and tumors or for variants showing a similar clinical course. MF is the most common form of CTCL and accounts for almost 50% of all primary cutaneous lymphomas. MF has an indolent clinical course with slow progression over years or decades, from patches to more infiltrated plaques and, eventually, tumors.

Initially, MF has a predilection for the buttocks and other sun-protected areas. In tumor-stage MF, the usual presentation is a combination of patches, plaques, and tumors; the tumors often show ulceration. However, if only tumors are present, without preceding or concurrent patches or plaques, a diagnosis of MF is highly unlikely and another type of CTCL should be considered.[1, 2]

SS occurs almost exclusively in adults. It is characterized by erythroderma, often associated with marked pruritus and exfoliation, edema, and lichenification. Lymphadenopathy, alopecia, onychodystrophy, and palmoplantar hyperkeratosis are commonly associated.

GSS syndrome shows circumscribed areas of pendulous lax skin with a predilection for the axillae and groin. An association with Hodgkin lymphoma or with classic MF may be apparent.[26] Most patients have an indolent clinical course.

Acute ATLL is characterized by the presence of leukemia, lymphadenopathy, organomegaly, hypercalcemia, and, in approximately 50%, skin lesions. Skin lesions most commonly include nodules or tumors (33%), generalized papules (22%), or plaques (19%).[8] Chronic and smoldering variants frequently manifest as skin lesions that closely resemble MF, whereas circulating neoplastic T cells are few or absent.

SPTL is a rare form of CTCL; only alpha/beta-types are included. Patients are usually first seen with solitary or multiple nodules and plaques, mainly involving 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.[27] Dissemination to extracutaneous sites is unusual. SPTL may be preceded for years or decades by a seemingly benign panniculitis suggestive of chronic erythema nodosum. Rarely, it may produce scalp alopecia.[28]

Primary cutaneous PTL, unspecified, is a heterogeneous group of diseases for which the common characteristic is a lack of typical features of MF.

Cutaneous gamma/delta T-cell lymphoma[12] belongs to the PTL group.[2, 3] It usually has an aggressive course and manifests with disseminated plaques and/or ulceronecrotic nodules or tumors, particularly on the extremities.[29]

Primary cutaneous aggressive epidermotropic CD8+ cytotoxic T-cell lymphoma, provisional entity, manifests localized or disseminated eruptive papules, nodules, and tumors that show central ulceration and necrosis or superficial, hyperkeratotic patches and plaques.[5, 30]

Primary cutaneous CD4+ small/medium-sized pleomorphic T-cell lymphoma 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 one or several papules, nodules, or tumors.[17, 31]

Extranodal NK/T-cell lymphoma, nasal type, usually appears as a midfacial destructive tumor or 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. Extranodal NK/T-cell lymphoma, nasal type, 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.[32, 33]

A few patients with MF/SS, have patchy, total-scalp, or universal alopecia. Alopecia may present follicular MF; total-body hair loss may be evident in some with generalized erythroderma and SS.[34] It 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.

Ocular involvement may be evident in advanced cutaneous T-cell lymphoma.[35] Direct tumor infiltration may produce or contribute to corneal ulceration in such patients.

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Causes

Chemical, physical, and microbial irritants have been discussed as causes for cutaneous T-cell lymphoma (CTCL) or mycosis fungoides (MF), but evidence related to an etiology is not convincing.[1] They may play the role of a persistent antigen, which, in a stepwise process, leads to an accumulation of mutations in oncogenes, suppressor genes, and signal-transducing genes.[3]

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Contributor Information and Disclosures
Author

Robert A Schwartz, MD, MPH  Professor and Head, Dermatology, Professor of Pathology, Pediatrics, Medicine, and Preventive Medicine and Community Health, University of Medicine and Dentistry of New Jersey-New Jersey Medical School

Robert A Schwartz, MD, MPH is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American College of Physicians, and Sigma Xi

Disclosure: Nothing to disclose.

Coauthor(s)

W Clark Lambert, MD, PhD  Professor and Head, Dermatopathology, Departments of Pathology and Dermatology, UMDNJ-New Jersey Medical School

W Clark Lambert, MD, PhD is a member of the following medical societies: American Academy of Dermatology, American College of Physicians, American Dermatological Association, American Society of Dermatopathology, International Academy of Pathology, Medical Society of New Jersey, Sigma Xi, and Society for Investigative Dermatology

Disclosure: Nothing to disclose.

Specialty Editor Board

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

Disclosure: Nothing to disclose.

Jeffrey Meffert, MD  Assistant Clinical Professor of Dermatology, University of Texas School of Medicine at San Antonio

Jeffrey Meffert, MD is a member of the following medical societies: American Academy of Dermatology, American Medical Association, Association of Military Dermatologists, and Texas Dermatological Society

Disclosure: Nothing to disclose.

Catherine M Quirk, MD  Clinical Assistant Professor, Department of Dermatology, University of Pennsylvania

Catherine M Quirk, MD is a member of the following medical societies: Alpha Omega Alpha and American Academy of Dermatology

Disclosure: Nothing to disclose.

Chief Editor

Dirk M Elston, MD  Director, Ackerman Academy of Dermatopathology, New York

Dirk M Elston, MD is a member of the following medical societies: American Academy of Dermatology

Disclosure: Nothing to disclose.

References

  1. Burg G, Kempf W. Etiology and pathogenesis of cutaneous lymphomas. In: Burg G, Kempf W. Cutaneous Lymphomas. London: Taylor & Francis; 2005.

  2. Willemze R, Jaffe ES, Burg G, et al. WHO-EORTC classification for cutaneous lymphomas. Blood. May 15 2005;105(10):3768-85. [Medline].

  3. Burg G, Kempf W, Cozzio A, et al. Cutaneous malignant lymphomas: update 2006. J Dtsch Dermatol Ges. Nov 2006;4(11):914-33. [Medline].

  4. Khamaysi Z, Ben-Arieh Y, Izhak OB, Epelbaum R, Dann EJ, Bergman R. The applicability of the new WHO-EORTC classification of primary cutaneous lymphomas to a single referral center. Am J Dermatopathol. Feb 2008;30(1):37-44. [Medline].

  5. Berti E, Tomasini D, Vermeer MH, Meijer CJ, Alessi E, Willemze R. Primary cutaneous CD8-positive epidermotropic cytotoxic T cell lymphomas. A distinct clinicopathological entity with an aggressive clinical behavior. Am J Pathol. Aug 1999;155(2):483-92. [Medline].

  6. Kempf W, Ostheeren-Michaelis S, Paulli M, et al. Granulomatous mycosis fungoides and granulomatous slack skin: a multicenter study of the Cutaneous Lymphoma Histopathology Task Force Group of the European Organization For Research and Treatment of Cancer (EORTC). Arch Dermatol. Dec 2008;144(12):1609-17. [Medline].

  7. Vonderheid EC, Bernengo MG, Burg G, et al. Update on erythrodermic cutaneous T-cell lymphoma: report of the International Society for Cutaneous Lymphomas. J Am Acad Dermatol. Jan 2002;46(1):95-106. [Medline].

  8. Setoyama M, Katahira Y, Kanzaki T. Clinicopathologic analysis of 124 cases of adult T-cell leukemia/lymphoma with cutaneous manifestations: the smouldering type with skin manifestations has a poorer prognosis than previously thought. J Dermatol. Dec 1999;26(12):785-90. [Medline].

  9. Weenig RH, Ng CS, Perniciaro C. Subcutaneous panniculitis-like T-cell lymphoma: an elusive case presenting as lipomembranous panniculitis and a review of 72 cases in the literature. Am J Dermatopathol. Jun 2001;23(3):206-15. [Medline].

  10. Hoque SR, Child FJ, Whittaker SJ, et al. Subcutaneous panniculitis-like T-cell lymphoma: a clinicopathological, immunophenotypic and molecular analysis of six patients. Br J Dermatol. Mar 2003;148(3):516-25. [Medline].

  11. Massone C, Chott A, Metze D, et al. Subcutaneous, blastic natural killer (NK), NK/T-cell, and other cytotoxic lymphomas of the skin: a morphologic, immunophenotypic, and molecular study of 50 patients. Am J Surg Pathol. Jun 2004;28(6):719-35. [Medline].

  12. Burg G, Dummer R, Wilhelm M, et al. A subcutaneous delta-positive T-cell lymphoma that produces interferon gamma. N Engl J Med. Oct 10 1991;325(15):1078-81. [Medline].

  13. El Shabrawi-Caelen L, Cerroni L, Kerl H. The clinicopathologic spectrum of cytotoxic lymphomas of the skin. Semin Cutan Med Surg. Jun 2000;19(2):118-23. [Medline].

  14. Miyamoto T, Yoshino T, Takehisa T, Hagari Y, Mihara M. Cutaneous presentation of nasal/nasal type T/NK cell lymphoma: clinicopathological findings of four cases. Br J Dermatol. Sep 1998;139(3):481-7. [Medline].

  15. Arnulf B, Copie-Bergman C, Delfau-Larue MH, et al. Nonhepatosplenic gammadelta T-cell lymphoma: a subset of cytotoxic lymphomas with mucosal or skin localization. Blood. Mar 1 1998;91(5):1723-31. [Medline].

  16. Jaffe ES, Krenacs L, Raffeld M. Classification of cytotoxic T-cell and natural killer cell lymphomas. Semin Hematol. Jul 2003;40(3):175-84. [Medline].

  17. Bekkenk MW, Vermeer MH, Jansen PM, et al. Peripheral T-cell lymphomas unspecified presenting in the skin: analysis of prognostic factors in a group of 82 patients. Blood. Sep 15 2003;102(6):2213-9. [Medline].

  18. Alsaleh QA, Nanda A, Al-Ajmi H, et al. Clinicoepidemiological features of mycosis fungoides in Kuwait, 1991-2006. Int J Dermatol. Dec 2010;49(12):1393-8. [Medline].

  19. Pope E, Weitzman S, Ngan B, et al. Mycosis fungoides in the pediatric population: report from an international childhood registry of cutaneous lymphoma. J Cutan Med Surg. Jan-Feb 2010;14(1):1-6. [Medline].

  20. Herrmann JL, Hughey LC. Recognizing large-cell transformation of mycosis fungoides. J Am Acad Dermatol. Jan 18 2012;[Medline].

  21. Shimauchi T, Sugita K, Nakamura M, Tokura Y. Leukaemic cutaneous T-cell lymphoma-manifesting papuloerythroderma with CD3(-) CD4(+) phenotype. Acta Derm Venereol. 2010;90(1):68-72. [Medline].

  22. Roeschm A, Schleyer V, Landthaler M, Vogt T. Follicular mycosis fungoides: variability of a rare entity. Skinmed. Jan-Feb 2005;4(1):12-7. [Medline].

  23. Muniesa C, Estrach T, Pujol RM, et al. Folliculotropic mycosis fungoides: clinicopathological features and outcome in a series of 20 cases. J Am Acad Dermatol. Mar 2010;62(3):418-26. [Medline].

  24. Pileri A, Facchetti F, Rutten A, et al. Syringotropic mycosis fungoides: a rare variant of the disease with peculiar clinicopathologic features. Am J Surg Pathol. Jan 2011;35(1):100-9. [Medline].

  25. Kohler S. Extranodal NK/T-cell lymphoma, nasal type. In: LeBoit P, Burg G, Weedon D, Lyon SA. WHO Books: Tumors of the Skin. Geneva: World Health Organization IARC. X. 2006:191-2.

  26. Clarijs M, Poot F, Laka A, Pirard C, Bourlond A. Granulomatous slack skin: treatment with extensive surgery and review of the literature. Dermatology. 2003;206(4):393-7. [Medline].

  27. Marzano AV, Berti E, Paulli M, Caputo R. Cytophagic histiocytic panniculitis and subcutaneous panniculitis-like T-cell lymphoma: report of 7 cases. Arch Dermatol. Jul 2000;136(7):889-96. [Medline].

  28. Torok L, Gurbity TP, Kirschner A, Krenacs L. Panniculitis-like T-cell lymphoma clinically manifested as alopecia. Br J Dermatol. Oct 2002;147(4):785-8. [Medline].

  29. Toro JR, Liewehr DJ, Pabby N, et al. Gamma-delta T-cell phenotype is associated with significantly decreased survival in cutaneous T-cell lymphoma. Blood. May 1 2003;101(9):3407-12. [Medline].

  30. Santucci M, Pimpinelli N, Massi D, et al. Cytotoxic/natural killer cell cutaneous lymphomas. Report of EORTC Cutaneous Lymphoma Task Force Workshop. Cancer. Feb 1 2003;97(3):610-27. [Medline].

  31. von den Driesch P, Coors EA. Localized cutaneous small to medium-sized pleomorphic T-cell lymphoma: a report of 3 cases stable for years. J Am Acad Dermatol. Apr 2002;46(4):531-5. [Medline].

  32. Barrionuevo C, Anderson VM, Zevallos-Giampietri E, et al. Hydroa-like cutaneous T-cell lymphoma: a clinicopathologic and molecular genetic study of 16 pediatric cases from Peru. Appl Immunohistochem Mol Morphol. Mar 2002;10(1):7-14. [Medline].

  33. Chen HH, Hsiao CH, Chiu HC. Hydroa vacciniforme-like primary cutaneous CD8-positive T-cell lymphoma. Br J Dermatol. Sep 2002;147(3):587-91. [Medline].

  34. Bi MY, Curry JL, Christiano AM, Hordinsky MK, et al. The spectrum of hair loss in patients with mycosis fungoides and Sezary syndrome. J Am Acad Dermatol. Jan 2011;64(1):53-63. [Medline].

  35. Livingstone I, Ramamurthi S, Drummond S, Kemp E, Roberts F. Corneal perforation due to limbal involvement in Sézary syndrome. Graefes Arch Clin Exp Ophthalmol. Jan 21 2011;[Medline].

  36. Gutte R, Kharkar V, Mahajan S, Chikhalkar S, Khopkar U. Granulomatous mycosis fungoides with hypohidrosis mimicking lepromatous leprosy. Indian J Dermatol Venereol Leprol. Nov-Dec 2010;76(6):686-90. [Medline].

  37. Karube K, Aoki R, Nomura Y, et al. Usefulness of flow cytometry for differential diagnosis of precursor and peripheral T-cell and NK-cell lymphomas: analysis of 490 cases. Pathol Int. Feb 2008;58(2):89-97. [Medline].

  38. Russell-Jones R. Diagnosing erythrodermic cutaneous T-cell lymphoma. Br J Dermatol. Jul 2005;153(1):1-5. [Medline].

  39. Lange-Asschenfeldt S, Babilli J, Beyer M, Ri´us-Diaz F, Gonza´lez S, Stockfleth E, et al. Consistency and distribution of reflectance confocal microscopy features for diagnosis of cutaneous T cell lymphoma. J Biomed Opt. Jan 2012;17(1):016001. [Medline].

  40. Petrella T, Comeau MR, Maynadie M, et al. 'Agranular CD4+ CD56+ hematodermic neoplasm' (blastic NK-cell lymphoma) originates from a population of CD56+ precursor cells related to plasmacytoid monocytes. Am J Surg Pathol. Jul 2002;26(7):852-62. [Medline].

  41. Smoller BR, Santucci M, Wood GS, Whittaker SJ. Histopathology and genetics of cutaneous T-cell lymphoma. Hematol Oncol Clin North Am. Dec 2003;17(6):1277-311. [Medline].

  42. Haghighi B, Smoller BR, LeBoit PE, Warnke RA, Sander CA, Kohler S. Pagetoid reticulosis (Woringer-Kolopp disease): an immunophenotypic, molecular, and clinicopathologic study. Mod Pathol. May 2000;13(5):502-10. [Medline].

  43. Mao X, Lillington D, Scarisbrick JJ, et al. Molecular cytogenetic analysis of cutaneous T-cell lymphomas: identification of common genetic alterations in Sézary syndrome and mycosis fungoides. Br J Dermatol. Sep 2002;147(3):464-75. [Medline].

  44. Burg G, Zwingers T, Staegemeir E, Santucci M. Interrater and intrarater variabilities in the evaluation of cutaneous lymphoproliferative T-cell infiltrates. EORTC-Cutaneous Lymphoma Project Group. Dermatol Clin. Apr 1994;12(2):311-4. [Medline].

  45. Santucci M, Biggeri A, Feller AC, Massi D, Burg G. Efficacy of histologic criteria for diagnosing early mycosis fungoides: an EORTC cutaneous lymphoma study group investigation. European Organization for Research and Treatment of Cancer. Am J Surg Pathol. Jan 2000;24(1):40-50. [Medline].

  46. Santucci M, Smoller B. ISCL study on early diagnosis of mycosis fungoides. (in preparation).

  47. Pimpinelli N, Olsen EA, Santucci M, et al. Defining early mycosis fungoides. J Am Acad Dermatol. Dec 2005;53(6):1053-63. [Medline].

  48. Zelger B, Sepp N, Weyrer K, Grünewald K, Zelger B. Syringotropic cutaneous T-cell lymphoma: a variant of mycosis fungoides?. Br J Dermatol. Jun 1994;130(6):765-9. [Medline].

  49. Gerami P, Guitart J. Basaloid folliculolymphoid hyperplasia: a distinctive finding in follicular mycosis fungoides. J Cutan Pathol. Dec 2007;34 Suppl 1:29-32. [Medline].

  50. Imai S, Burg G, Braun-Falco O. Mycosis fungoides and Sezary's syndrome show distinct histomorphological features. Dermatologica. 1986;173(3):131-5. [Medline].

  51. Kamarashev J, Burg G, Kempf W, Hess Schmid M, Dummer R. Comparative analysis of histological and immunohistological features in mycosis fungoides and Sézary syndrome. J Cutan Pathol. Sep 1998;25(8):407-12. [Medline].

  52. Sibaud V, Beylot-Barry M, Thiebaut R, et al. Bone marrow histopathologic and molecular staging in epidermotropic T-cell lymphomas. Am J Clin Pathol. Mar 2003;119(3):414-23. [Medline].

  53. Takeshita M, Okamura S, Oshiro Y, et al. Clinicopathologic differences between 22 cases of CD56-negative and CD56-positive subcutaneous panniculitis-like lymphoma in Japan. Hum Pathol. Feb 2004;35(2):231-9. [Medline].

  54. Beljaards RC, Meijer CJ, Van der Putte SC, et al. Primary cutaneous T-cell lymphoma: clinicopathological features and prognostic parameters of 35 cases other than mycosis fungoides and CD30-positive large cell lymphoma. J Pathol. Jan 1994;172(1):53-60. [Medline].

  55. Chan JK, Sin VC, Wong KF, et al. Nonnasal lymphoma expressing the natural killer cell marker CD56: a clinicopathologic study of 49 cases of an uncommon aggressive neoplasm. Blood. Jun 15 1997;89(12):4501-13. [Medline].

  56. Natkunam Y, Smoller BR, Zehnder JL, Dorfman RF, Warnke RA. Aggressive cutaneous NK and NK-like T-cell lymphomas: clinicopathologic, immunohistochemical, and molecular analyses of 12 cases. Am J Surg Pathol. May 1999;23(5):571-81. [Medline].

  57. de Wolf-Peeters C, Achten R. gammadelta T-cell lymphomas: a homogeneous entity?. Histopathology. Apr 2000;36(4):294-305. [Medline].

  58. Jones D, Vega F, Sarris AH, Medeiros LJ. CD4-CD8-"Double-negative" cutaneous T-cell lymphomas share common histologic features and an aggressive clinical course. Am J Surg Pathol. Feb 2002;26(2):225-31. [Medline].

  59. Olsen EA, Rook AH, Zic J, et al. Sezary syndrome: Immunopathogenesis, literature review of therapeutic options, and recommendations for therapy by the United States Cutaneous Lymphoma Consortium (USCLC). J Am Acad Dermatol. Feb 2011;64(2):352-404. [Medline].

  60. Ahern K, Gilmore ES, Poligone B. Pruritus in cutaneous T-cell lymphoma: A review. J Am Acad Dermatol. Jan 26 2012;[Medline].

  61. Knobler E. Current management strategies for cutaneous T-cell lymphoma. Clin Dermatol. May-Jun 2004;22(3):197-208. [Medline].

  62. Kanokrungsee S, Rajatanavin N, Rutnin S, Vachiramon V. Efficacy of narrowband ultraviolet B twice weekly for hypopigmented mycosis fungoides in Asians. Clin Exp Dermatol. Mar 2012;37(2):149-52. [Medline].

  63. Ysebaert L, Truc G, Dalac S, et al. Ultimate results of radiation therapy for T1-T2 mycosis fungoides (including reirradiation). Int J Radiat Oncol Biol Phys. Mar 15 2004;58(4):1128-34. [Medline].

  64. Onozuka T, Yokota K, Kawashima T, et al. An elderly patient with mycosis fungoides successfully treated with chronic low-dose oral etoposide therapy. Clin Exp Dermatol. Jan 2004;29(1):91-2. [Medline].

  65. Dummer R, Urosevic M, Kempf W, Kazakov D, Burg G. Imiquimod induces complete clearance of a PUVA-resistant plaque in mycosis fungoides. Dermatology. 2003;207(1):116-8. [Medline].

  66. Apisarnthanarax N, Talpur R, Ward S, Ni X, Kim HW, Duvic M. Tazarotene 0.1% gel for refractory mycosis fungoides lesions: an open-label pilot study. J Am Acad Dermatol. Apr 2004;50(4):600-7. [Medline].

  67. Singh F, Lebwohl MG. Cutaneous T-cell lymphoma treatment using bexarotene and PUVA: a case series. J Am Acad Dermatol. Oct 2004;51(4):570-3. [Medline].

  68. Jones GW, Hoppe RT, Glatstein E. Electron beam treatment for cutaneous T-cell lymphoma. Hematol Oncol Clin North Am. Oct 1995;9(5):1057-76. [Medline].

  69. Leverkus M, Rose C, Bröcker EB, Goebeler M. Follicular cutaneous T-cell lymphoma: beneficial effect of isotretinoin for persisting cysts and comedones. Br J Dermatol. Jan 2005;152(1):193-4. [Medline].

  70. Jacob R, Scala M, Fung MA. A case of syringotropic cutaneous T-cell lymphoma treated with local radiotherapy. J Am Acad Dermatol. Jan 2009;60(1):152-4. [Medline].

  71. Russell-Jones R. Extracorporeal photopheresis in cutaneous T-cell lymphoma. Inconsistent data underline the need for randomized studies. Br J Dermatol. Jan 2000;142(1):16-21. [Medline].

  72. Lundin J, Hagberg H, Repp R, et al. Phase 2 study of alemtuzumab (anti-CD52 monoclonal antibody) in patients with advanced mycosis fungoides/Sezary syndrome. Blood. Jun 1 2003;101(11):4267-72. [Medline].

  73. Fukushima T, Horio K, Matsuo E, et al. Successful cord blood transplantation for mycosis fungoides. Int J Hematol. Dec 2008;88(5):596-8. [Medline].

  74. Tsuji H, Wada T, Murakami M, et al. Two cases of mycosis fungoides treated by reduced-intensity cord blood transplantation. J Dermatol. Dec 2010;37(12):1040-5. [Medline].

  75. Horwitz SM, Kim YH, Foss F, Zain JM, Myskowski PL, Lechowicz MJ, et al. Identification of an active, well-tolerated dose of pralatrexate in patients with relapsed or refractory cutaneous T-cell lymphoma (CTCL). Blood. Mar 6 2012;[Medline].

  76. Lambert WC, Cohen PJ, Schwartz RA. Surgical management of mycosis fungoides. J Med. 1997;28(3-4):211-22. [Medline].

  77. Foss F. Overview of cutaneous T-cell lymphoma: prognostic factors and novel therapeutic approaches. Leuk Lymphoma. 2003;44 Suppl 3:S55-61. [Medline].

  78. Kaye FJ, Bunn PA Jr, Steinberg SM, et al. A randomized trial comparing combination electron-beam radiation and chemotherapy with topical therapy in the initial treatment of mycosis fungoides. N Engl J Med. Dec 28 1989;321(26):1784-90. [Medline].

  79. Meyer N, Paul C, Misery L. Pruritus in cutaneous T-cell lymphomas: frequent, often severe and difficult to treat. Acta Derm Venereol. 2010;90(1):12-7. [Medline].

  80. Agar NS, Wedgeworth E, Crichton S, et al. Survival outcomes and prognostic factors in mycosis fungoides/Sézary syndrome: validation of the revised International Society for Cutaneous Lymphomas/European Organisation for Research and Treatment of Cancer staging proposal. J Clin Oncol. Nov 1 2010;28(31):4730-9. [Medline].

  81. Tancrede-Bohin E, Ionescu MA, de La Salmoniere P, et al. Prognostic value of blood eosinophilia in primary cutaneous T-cell lymphomas. Arch Dermatol. Sep 2004;140(9):1057-61. [Medline].

  82. van Doorn R, Scheffer E, Willemze R. Follicular mycosis fungoides, a distinct disease entity with or without associated follicular mucinosis: a clinicopathologic and follow-up study of 51 patients. Arch Dermatol. Feb 2002;138(2):191-8. [Medline].

  83. Scarisbrick JJ, Whittaker S, Evans AV, et al. Prognostic significance of tumor burden in the blood of patients with erythrodermic primary cutaneous T-cell lymphoma. Blood. Feb 1 2001;97(3):624-30. [Medline].

  84. Shimoyama M. Diagnostic criteria and classification of clinical subtypes of adult T-cell leukaemia-lymphoma. A report from the Lymphoma Study Group (1984-87). Br J Haematol. Nov 1991;79(3):428-37. [Medline].

  85. Fink-Puches R, Zenahlik P, Back B, Smolle J, Kerl H, Cerroni L. Primary cutaneous lymphomas: applicability of current classification schemes (European Organization for Research and Treatment of Cancer, World Health Organization) based on clinicopathologic features observed in a large group of patients. Blood. Feb 1 2002;99(3):800-5. [Medline].

  86. Bekkenk MW, Jansen PM, Meijer CJ, Willemze R. CD56+ hematological neoplasms presenting in the skin: a retrospective analysis of 23 new cases and 130 cases from the literature. Ann Oncol. Jul 2004;15(7):1097-108. [Medline].

  87. Mraz-Gernhard S, Natkunam Y, Hoppe RT, LeBoit P, Kohler S, Kim YH. Natural killer/natural killer-like T-cell lymphoma, CD56+, presenting in the skin: an increasingly recognized entity with an aggressive course. J Clin Oncol. Apr 15 2001;19(8):2179-88. [Medline].

  88. Burg G, Kempf W. Therapy of cutaneous lymphomas. In: Burg G, Kempf W. Cutaneous Lymphomas. London: Taylor & Francis; 2005:475-528.

  89. Burg G, Kempf W, Cozzio A, et al. WHO/EORTC classification of cutaneous lymphomas 2005: histological and molecular aspects. J Cutan Pathol. Nov 2005;32(10):647-74. [Medline].

 
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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.
Erythematous partially confluent 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 both epidermis and dermis.
Mycosis fungoides with epidermal nest of large atypical T cells displaying bizarre, convoluted nuclei surrounded by a clear space (Pautrier microabscess).
Table 1. Staging of CTCL
StageSkin LesionsLymphadenopathyErythrodermaHistological Lymphoma
Patches



< 10%



Plaques



³10%



TumorsLymph NodesViscera
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 2. TNMB Staging of CTCL
Stage ClassStageDefinition
T



(Tumor)



T1Patches/plaques involving < 10% of body surface
T2Patches/plaques involving ³10% of body surface
T3Tumor(s) present on skin
T4Erythroderma
N



(Nodes)



N0No enlarged lymph node present
N1Enlarged lymph nodes, histologically uninvolved
N2No enlarged lymph node; one or more nodes histologically involved*
N3Enlarged lymph nodes, histologically involved
M



(Metastasis to viscera)



M0No visceral lesion present
M1Visceral involvement
B



(Blood involvement)



B0Circulating atypical lymphocytes (Sézary cells) £5% of lymphocytes
B1Circulating atypical lymphocytes ³5% of lymphocytes (Sézary syndrome)
*Uncommon finding, usually not considered/investigated.
Table 3. Comparison of Staging Systems for CTCL
Clinical StageTNM (B) Stage
IAT1 N0 M0
IIBT2 N0 M0
IIAT1 N1 M0T2 N1 M0
IIBT3 N0 M0T3 N1 M0
IIIT4 N0 M0T4 N1 M0
IVAT1 N2 M0T2 N2 M0T3 N2 M0T4 N2 M0
T1 N3 M0T2 N3 M0T3 N3 M0T4 N3 M0
IVBT1 N0 M1T2 N0 M1T3 N0 M1T4 N0 M1
T1 N1 M1T2 N1 M1T3 N1 M1T4 N1 M1
T1 N2 M1T2 N2 M1T3 N2 M1T4 N2 M1
T1 N3 M1T2 N3 M1T3 N3 M1T4 N3 M1
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