Type Of Cutaneous T-cell Lymphoma Research Articles

Linfoma primario cutáneo agresivo epidermotrópico de células T citotóxicas CD8 positivo: Caso clínico

Primary cutaneous aggressive epidermotropic cytotoxic CD8 positive T cell lymphoma, is an uncommon disease, with an aggressive clinical behavior. Differentiation with other types of cutaneous T-cell lymphoma (CTCL) that express a CD8+ cells, is based only on clinical grounds and in certain morphological characteristics, such as a marked epidermotropism with squamous cell necrosis. We report a 50-year-old male presenting with painless cutaneous lesions appearing in trunk, limbs, scalp and face, suggestive of cutaneous lymphoma. He was admitted to the hospital in bad conditions, with confluent papules and tumors, some of them ulcerated and with foul smelling honey-colored crusts, involving the complete body surface. Cutaneous biopsy demonstrated a CD8 positive epidermotropic cytotoxic T cell lymphoma. He was treated with chemotherapy with an excellent initial response, but cutaneous lesions reappeared after four cycles. He did not respond to rescue chemotherapy and died seven months after diagnosis.

Expression of Programmed Death-1 in Primary Cutaneous CD4-Positive Small/Medium-Sized Pleomorphic T-Cell Lymphoma, Cutaneous Pseudo-T-Cell Lymphoma, and Other Types of Cutaneous T-Cell Lymphoma

In this study we investigated whether programmed death-1 (PD-1) could serve as a useful diagnostic marker to differentiate between primary cutaneous CD4 small/medium-sized pleomorphic T-cell lymphoma (PCSM-TCL) and cutaneous pseudo-T-cell lymphomas on the one hand and other types of cutaneous T-cell lymphomas (CTCLs) on the other. Formalin-fixed, paraffin-embedded skin biopsies from 26 patients with PCSM-TCL or pseudo-T-cell lymphoma, including 1 patient with a lymphomatoid drug eruption, and 52 skin biopsies from other types of CTCLs were stained for PD-1. In addition, PD-1-positive cases were stained with antibodies against BCL6, CXCL13, and CD10 to determine a possible relationship with follicular helper T (TFH) cells. In all 26 cases of PCSM-TCL or pseudo-T-cell lymphoma, the medium-sized to large-sized atypical T cells consistently expressed PD-1, BCL6, and CXCL13 but not CD10. PD-1 expression was found in only 2 of 21 cases of mycosis fungoides and in only 2 of 16 cases of cutaneous peripheral T-cell lymphoma, unspecified. All 4 patients with an aggressive epidermotropic cytotoxic CD8 CTCL and all 11 cases with a primary cutaneous CD30 lymphoproliferative disorder were negative for PD-1. In conclusion, PD-1 is typically expressed by atypical cells in PCSM-TCL and pseudo-T-cell lymphoma but is not expressed or is rarely expressed in other types of CTCLs. Therefore, it may serve as a suitable adjunct in differential diagnosis. Our results demonstrate that the atypical cells in PCSM-TCL and pseudo-T-cell lymphomas share a common TFH phenotype and support the view that most cases classified nowadays as PCSM-TCL are identical to cutaneous pseudo-T-cell lymphomas described previously.

Photodynamic Therapy with Methyl-aminolaevulinic Acid for Mycosis Fungoides

Mycosis fungoides is the most common type of cutaneous T-cell lymphoma. There are a wide range of treatments for early-stage and advanced-stage mycosis fungoides. Photodynamic therapy (PDT) has emerged as a new treatment modality due to its safety and efficacy. The aim of this study was to investigate the safety and efficacy of PDT with methyl-aminolaevulinic acid (MAL) for the treatment of mycosis fungoides. Ten patients with mycosis fungoides were enrolled in this study. A 16.8% MAL cream was applied under occlusive dressing for 3 h. The lesion was irradiated at 37.5 J/cm2 with red light. The patients underwent two sessions of PDT at one-week intervals. Follow-up biopsy was performed 3 months after the last treatment. In case of partial response, treatment was repeated once a week until complete response. Seven patients had a good therapeutic response. Complete and partial responses were seen in 5 and 2 patients, respectively. During the follow-up period (8-31 months), 6 of the 7 patients remained in stable remission. The treatment was well-tolerated overall, and no patients discontinued the PDT due to pain. In conclusion, PDT with MAL is a fast, effective and well-tolerated treatment for unilesional mycosis fungoides.

Mycosis Fungoides: Tumour d′emblee

Mycosis Fungoides is a Cutaneous T-cell lymphoma characterized by infiltration of skin with patches, plaques, and nodules composed of T-lymphocytes. It is the most common type of Cutaneous T-Cell Lymphoma and accounts for almost 50% of all primary cutaneous lymphoma. Tumour d’ emblee is the term used for the patient presenting with skin tumors not preceded by patches or plaques. We report a rare case of mycosis fungoides d’ emblee variant with tumors of only 3 months duration without any preceding skin lesions.

Primary cutaneous anaplastic large cell lymphoma with subsequent leg involvement

Primary cutaneous anaplastic large cell lymphoma (CALCL) is regarded as an indolent type of cutaneous T-cell lymphoma. However, a few recent publications revealed that C-ALCL patients with initial leg involvement had significantly worse survival than those without initial leg involvement. Herein, we report a case of C-ALCL with subsequent leg involvement, which led to death after chemoradiation therapy. A 75 years old Japanese man presented with multiple erythematous nodules in his left arm and the side of his left chest. Histopathological and immunohistochemical studies led to the diagnosis of primary C-ALCL. At the initial diagnosis, no leg lesion was found. One year after the initial diagnosis, C-ALCL appeared in his right lower thigh and left hip. Radiation therapy, low-dose etoposide and CHOP therapy were performed; however, the patient died of malignant lymphoma 4 years after the initial diagnosis. We speculated that the occurrence of subsequent leg involvement may also be indicative of a worse prognosis, as in the case with initial leg involvement in C-ALCL. Therefore, we propose that C-ALCL patients with initial or subsequent leg involvement should be classified as a distinct clini copathological variant of C-ALCL (“leg -type ” involvement) and that they may require intense therapy.

Childhood mycosis fungoides: a report of 20 cases from Turkey

Mycosis fungoides (MF) is the most common type of cutaneous T-cell lymphomas in adults. In the recent past, several reports have focused on an increased prevalence of MF in children. The present study was aimed to evaluate the clinical characteristics, treatment modalities and disease progression in childhood MF patients from Turkey. In a retrospective analysis of 368 MF patients in a referral center at Istanbul, Turkey, 20 patients were diagnosed before the age of 18 years and were included in the study. Childhood cases constituted 5.4% of all MF patients. The age at the time of diagnosis ranged between 2-18 years with a mean of 9.20 ± 4.52 and a median value of 9.50 years. The clinical presentation of MF consisted of solely patches in 60%, followed by plaques or plaques and patches in 40% of the patients. Hypopigmented lesions were seen in 45% and purpuric lesions in 30% of the patients. Four patients (20%) were diagnosed to have unilesional MF. The large majority of the patients (95%) had T1 N0 M0 or T2 N0 M0 disease. Sixteen patients were followed between 1-13 years (median: 3.75 years). All the patients were treated with skin directed treatments including topical corticosteroids, topical bexarotene, topical carmustine, narrow band UVB and PUVA. None of the patients progressed to an advanced stage. After an initial clearance, 69.2% of the patients were observed to have recurrences. In our experience, patches were more prevalent among childhood MF cases. Overrepresentation of hypopigmented and purpuric lesions was remarkable. Progression to an advanced stage was not seen. However, recurrences after discontinuation of therapy were common.

Promising Efficacy with the 308 Nm Excimer Laser Phototherapy in Early Stage Mycosis Fungoides

Abstract 4990 Background:Mycosis fungoides is the most common type of cutaneous T-cell lymphoma. Early stage disease is effectively managed with skin directed therapies such as UV light and topical steroids. Excimer laser (EL) delivers monochromatic, UVB light at a wavelength of 308 nm via hand held device that covers an area of 2 × 2 cm. It generates a short-pulse radiation that is concentrated on an affected area of skin, thereby allowing a delivery of higher dose on precisely targeted tissue. In contrast to other phototherapy techniques such as narrow band UV light, the EL is ideally suited for patients with small number of patch lesions. Although EL was successfully utilized in the management of psoriasis and vitiligo, there is not extensive experience with this modality in patients with CTCL. Objectives:To assess safety and efficacy of EL therapy in patients with early stage MF who failed ≥1 skin directed therapy. Design and Methods:This is a retrospective analysis of seven consecutive patients with stage 1 MF treated with EL phototherapy between January 2011 and August 2011 in a single institution. Seven patients with histologically confirmed common type MF, and one patient with folliculotropic MF received 308 nm EL therapy after failure of at least one prior skin directed therapy. The median age was 48 years (range 24–77 yrs). Four (57%) were male, 3 (43%) were female, six (86%) were Caucasian, and 1(14%) were African American. Biopsies and photos were obtained at diagnosis and after the completion of 24 treatments. Results:Treatment was initiated at a dose of 200 millijoules (mJ) and was increased by 10–15% each subsequent treatment. The max dose of treatment ranged from 240 mJ to 850 mJ. The total number of administered treatments was 24 delivered over period of 3 months. The median number of treated lesions was 2 (range 1–5). The surface area of treated lesions was <10%. Six patients achieved clinical improvement in appearance of lesions and intensity of pruritis; 2 (29%) achieved clinical remission as confirmed with photos and post-treatment biopsies. One (14%) patient developed first degree burn and 1 (14%) patient developed pruritis. These resolved by the second treatment with EL. There were no treatment-related serious adverse events observed. Conclusion:Our results suggest that EL is effective and well tolerated skin-directed treatment modality for selected patients with early stage MF. However, longer follow-up will be required to assess durability of responses. Advantages of this approach include shorter treatment duration and lower risk of carcinogenesis in the non-affected areas of skin. Prospective study with a larger cohort of patients is necessary to further assess efficacy and safety of this approach. Disclosures:No relevant conflicts of interest to declare.

Childhood mycosis fungoides: New observations from the Middle East

Mycosis fungoides is the most common type of Cutaneous T-cell Lymphoma. Data on clinical characteristics and disease progression in children with mycosis fungoides are limited. Five patients from the Middle East with childhood mycosis fungoides are reported here. Four of those patients presented with hypopigmented patches as the sole manifestation of the disease. Histopathologic features were consistent with mycosis fungoides in all patients and in four of five patients CD4+ phenotype was over-represented immunohistochemically. All patients had excellent prognosis. Only one patient with the classic erythematous patch/plaque type progressed to stage IIA. It seems the phenotype expression with either CD4+ or CD8+ has no implication on disease progression. However, the clinical morphologic type might impact disease progression with the hypopigmented type being the variant with the best prognosis. Further studies with large number of patients are required for better characterization of Middle Eastern patients with childhood MF.

XV. Primary cutaneous lymphomas

Primary cutaneous lymphomas (PCLs) are defined as non-Hodgkin lymphomas that present in the skin with no evidence of extracutaneous disease at the time of diagnosis. After the gastrointestinal lymphomas, PCL are the second most common group of extranodal non-Hodgkin lymphomas with an estimated annual incidence of 1/100 000. These PCLs must be distinguished from nodal or systemic malignant lymphomas involving the skin secondarily, which often have another clinical behavior, have a different prognosis and require a different therapeutic approach. In recent lymphoma classifications (WHO-EORTC; WHO 2008) the different types of primary cutaneous T-cell lymphoma (CTCL) and primary cutaneous B-cell lymphoma (CBCL) are therefore included as separate entities (see Table 1) [1, 2]. In the western world, CTCL constitutes 75%–80% of all PCLs and CBCL 20%–25%, but different distributions have been observed in other parts of the world. Within the group of CTCLs roughly three categories can be distinguished: (i) the group of classical CTCLs, including mycosis fungoides (MF), variants or subtypes of MF and Sezary syndrome (SS); (ii) the group of primary cutaneous CD30-positive lymphoproliferative disorders (CD30+ LPD); and (iii) a group of rare often aggressive cutaneous T/NK-cell lymphomas, including subcutaneous panniculitis-like T-cell lymphoma (SPTCL), extranodal NK/T-cell lymphoma, nasal type, and primary cutaneous peripheral T-cell lymphoma, not otherwise specified (PTCLNOS). Well-defined types of CBCL include primary cutaneous marginal zone lymphoma (PCMZL), primary cutaneous follicle center lymphoma (PCFCL) and primary cutaneous diffuse large B-cell lymphoma, leg type (PCLBCL, LT). Herein, characteristic features of the main types of CTCL and CBCL are summarized and recent observations regarding diagnosis, prognostic factors and treatment are described.

Diagnostic significance of TCR gene clonal rearrangement analysis in early mycosis fungoides

Mycosis fungoides (MF), the most common type of cutaneous T-cell lymphoma, has various unspecific clinical and histological characteristics. Its early diagnosis is challenging. The application of T-cell receptor (TCR) gene clonal rearrangement to the diagnosis of MF has been widely studied. In this study, we used polymerase chain reaction (PCR) to investigate the diagnostic significance of detecting TCR-γ and -β gene clonal rearrangement in the early diagnosis of mycosis fungoides. PCR for TCR-γ and TCR-β gene rearrangement was performed on 19 patients with suspected early MF, 6 with typical MF, and 6 with chronic dermatitis. Of the 19 patients with suspected early MF, 13 had TCR-γ gene clonal rearrangement, whereas none had TCR-β gene clonal rearrangement. All patients with typical MF had TCR gene clonal rearrangement, in which 4 showed TCR-γ clonal rearrangement, 1 showed TCR-β gene clonal rearrangements, and 1 showed both. No patients with chronic dermatitis had TCR gene clonal rearrangement. These results indicate that TCR gene clonal rearrangement analysis is a useful tool in diagnosing early MF. TCR-γ gene is recommended to the routine analysis, whereas TCR-β gene has potential in combination toward intractable cases.

Dyshidrotic mycosis fungoides

Mycosis fungoides (MF) represents the most common type of cutaneous T-cell lymphoma (CTCL). CTCL often progresses through patch, plaque and tumor stages but can also manifest with varied clinical presentations. MF rarely presents in vesiculobullous fashion, in which vesicles or bullae develop in pre-existing plaques or on the trunk or proximal extremities. We report a patient who presented with a vesiculobullous eruption on the palms and soles, resembling dyshidrotic dermatitis, which we believe represents dyshidrotic MF.

Clinicoepidemiological features of mycosis fungoides in Kuwait, 1991–2006

Mycosis fungoides (MF) is an indolent, most common type of cutaneous T-cell lymphoma (CTCL) with an average estimated incidence of 0.5 cases per 100,000 persons per year in the western world. Although various clinical and epidemiological features are well delineated in the western population, the data is scarce from our region. To study the clinicoepidemiological features of MF from Kuwait. A referral photobiology unit for cutaneous lymphomas in a national dermatology department in collaboration with three other dermatology departments in Kuwait and Kuwait cancer center. One hundred and ninety-three cases of MF registered between July 1991 and June 2006 were included for this study. Eighty-six percent of our MF cases were of Arab ethnicity. Males outnumbered the females by 2:1 ratio. Mean age at diagnosis was 35.20 ± 14.37 years, and 16% of the patients were diagnosed by the age 20 years. The annual incidence rate (IR) of MF in Kuwait was observed to be 0.43 cases per 100,000 persons with a significantly higher IR among Arabs as compared to non-Arab Asians (RR = 4.4; 95% CI = 2.9-6.6). A successive rise in the IR of MF was noticed with the advancing age. The annual IR among males was more or less comparable to that of females. Skin patches were the most prevalent skin lesions (67%) at diagnosis, and 22% of the patients had a pure hypopigmented variant. Patients with hypopigmented MF were observed to have younger mean age at diagnosis (27.60 ± 12.42 years) as compared to other MF cases (38.14 ± 14.37 years) (P = 0.000). Ninety-two percent of the patients had the early stage (IA, IB, and IIA) of disease. Our patients with MF were observed to have a relatively younger age at diagnosis, with a high proportion of patients diagnosed by the age 20 years. Arabs were observed to have a higher annual IR of MF as compared to non-Arab Asians. Hypopigmented MF is prevalent in our population. The study highlights the ethnic and/or regional variations in the clinicoepidemiological characteristics of MF.

Plasma TM2-PK levels in mycosis fungoides patients

Aerobic glycolysis increases in tumor cells and pyruvate kinase (PK) is one of the key enzymes involved; PK exists in different isoforms in various tissues. Tumor M2-PK (TM2-PK) is one of these isoforms and its expression has been observed in various tumor cells, including lymphocytes, and in lymphoproliferative disorders. The present study aimed to compare plasma levels of TM2-PK and serum levels of two established markers of various lymphoproliferative disorders-lactate dehydrogenase (LDH) and β-2 microglobulin, and to evaluate the role of TM2-PK in drug monitorization and disease activity in mycosis fungoides (MF) patients. The study included 27 MF patients and 46 healthy controls. Among the MF patients, 18 were stage IA, 6 were stage IB, 1 was stage IIA, and 2 were stage III. Plasma TM2-PK, and serum LDH and β-2 microglobulin levels in the patients and controls were measured using the ELISA technique, a kinetic method, and a chemiluminescent assay, respectively. Measurements were repeated in the patient group posttreatment. Median levels of TM2-PK, LDH, and β-2 microglobulin level in the MF patients were 22 U mL⁻¹, 375 U L⁻¹, and 1,831 ng mL⁻¹, respectively. TM2-PK and β-2 microglobulin levels did not significantly differ between the MF patients and controls; however, LDH levels were significantly higher in the MF patients. TM2-PK levels in 17 of the MF patients that were in remission did not significantly differ from their pre-therapy levels. Based on a cut-off point of 17.5 U mL⁻¹, the sensitivity and specificity of TM2-PK for diagnosing MF were 55.6 and 60.9%, respectively. β-2 microglobulin was the most sensitive marker for diagnosing MF (63%), while LDH was the most specific marker. Furthermore, the sensitivity of TM2-PK increased when it was analyzed in combination as parallel tests with LDH and β-2 microglobulin (86%), while the specificity was measured as 32%. In serial analysis, the specificity was increased to 98%, while the sensitivity was 5%. Statistically significant agreement in diagnosing MF was also noted between TM2-PK and LDH levels. TM2-PK may not be a useful marker for MF, especially in early-stage patients, because it proliferates slowly. We think that TM2-PK levels should be investigated in advanced-stage MF or in other types of cutaneous T-cell lymphomas; in particular, in combination with other established markers.

Early-stage mycosis fungoides variants: case-based review

Mycosis fungoides (MF) is the most common type of cutaneous T-cell lymphoma. The diagnosis of classic MF is based on a combination of clinical presentation, histopathology, and T-cell monoclonality detected by molecular studies. However, the diagnosis can be difficult in cases of early MF because of the subtle nature of histologic findings and, in cases of variants of MF, because of the unusual clinical and/or pathologic features. In this review, we presented the most frequent variants of MF at early stage including hypopigmented, folliculotropic, pagetoid reticulosis, unilesional, granulomatous, and ichthyosis forms. This case-based clinicopathologic review provides the notion that a comprehensive clinicopathologic correlation is of substantial importance to render the diagnosis of MF. In addition, we discuss the role of molecular studies, which are highly sensitive and recently more applicable to routinely processed skin biopsy specimens in the diagnosis of MF.

P105 Mycosis fungoides or coexistence pemphigus and mycosis fungoides

Introduction Mycosis fungoides is the most common type of cutaneous T-cell lymphoma (CTCL). The disease usually develops and progresses slowly and chroniclly. It generally affects the skin, but may progress internally over time, when the neoplastic process involves the entire lymphoreticular system, the lymphonodes and internal organ. Mycosis fungoides is usually diagnosed after the age of 50, but has been seen as early as childhood. Mycosis fungoides develops twice as often in men as in women and is more common in people of African than of European origin. Virus infections, allergies, environmental chemicals and genes have all been suggested as possible causes of this cancer. Mycosis fungoides is a skin cancer characterized by patches, plaques, nodules and tumors with or without ulcers, poikiloderma, erythrodermic form and other more rare symptoms are also seen, including itching alone. Clinical course, prognosis, and management are different from one patient to another. Case report Clinical featuresWe describe an 81-year-old man,admitted to the Clinic of Dermatovenerology in Banjaluka with a several month history of numerous pustules and green-yellow colored crusting on normal and erythematous skin of face, hull and the upper extremities. The patient had a lot of documentaion about his previous disease. The first skin changes appeared in 1999 as erythematous plaques on the whole body, when the patient was treated, in his city, with local corticostreoid therapy. Skin changes have completely disappeared and patient had no skin changes over 8 years. During the year of 2007, new skin changes appeared in the form of many erythematousqamosus plaques. Then the biopsy was made and diadgnosed as psoriasis vulgaris. He was treated with Azathioprine 150 mg daily and local corticostreoid therapy. After several months there was a worsening of the disease and the patient was hospitalized at our Clinic with many pustulas and green-yellow crusts on normal and erythematous skin of face, hull and the upper extremities. On the scalp was erythema and silvery-white scale. Nikolsky's sign was positive. Laboratory findings SE 17/38,WBC 12,22; other biochemical examinations were in normal values, markers of hepatitis were negative; ANA: was negative; Peripheral blood smear was normal ; PAT (after regression of the disease, because patient was unable to do it previously): was negative Histopathology Intraepidermal bulla filled with many granulocytesites, fibrin and necrotic cells, and it looked more like pustula. Inflamatory cells forming bands and penetrate in dermis, deep between dermal papillae. There was a dense perivascular infiltrate composed of neutofils, eosinophils, lymfocits, nuclear powder and blob of hemosiderin pigment. Pathohistological image corresponds to pustulosis form of pemphigus vulgaris. Treatment Nirypan (methylprednisolone) 60 mg daily during the 21 days, after that we gradually reduced the dose to 4 mg per week, Alfa D3, H-2 blockers, oral antibiotics and local anthiseptic therapy. After the therapy, all of skin changes disappeared, and patient was treated with methylprednisolone 8 mg during the next two years. In June of 2009, two livid plaques came to the appearance on the scalp and back, that were resistant to therapy. Then we made skin biopsy from livid plaq on the back. Histopathology Epidermis was atrophic with overlying hyper and parakeratosis, under epidermis lichenoid lymphonocytic inflammation. There were many areas of epidermotrophism with collections of atypical lymphocytes. The pigmentory incontinence in the dermis was also marked. Imunohistochemical analysis shows: CD3+; CD5+,CD4+; CD8+ T cells in upper dermi. Conclusion Mycosis fungoides is a rare form of T-cell lymphoma of the skin and has variable phenotypic and histopathologic presentations. The diagnosis is made through a combination of the clinical picture and examination, and is confirmed by biopsy and imunohistochemical analysis We present an uncommon and rare case of mycosis fungoides in pustular form, and we are still in dilemma whether it was mycosis fungoides or coexistence pemphigus and mycosis fungoides.

Genome-Wide Analysis of Cutaneous T-Cell Lymphomas Identifies Three Clinically Relevant Classes

This study was undertaken to identify recurrent genetic alterations of the three main types of cutaneous T-cell lymphomas (CTCLs): mycosis fungoides (MF), Sézary syndrome (SS), and cutaneous anaplastic large-cell lymphoma (CALCL). Using array-based comparative genomic hybridization, the molecular cytogenetic profiles of 72 samples obtained from 58 patients with CTCL corresponding to 24 transformed MF (T-MF), 16 SS, and 18 CALCLs were determined. T-MF was characterized by gains of 1q25-31, 7p22-11.2, 7q21, 7q31, and 17q12, and losses of 9p21, 10p11.2, and 10q26. SS exhibited gains of 8q23-24.3 and 17q23-24, as well as losses of 9p21, 10p12-11.2, 10q22-24, 10q25-26, and 17p13-q11.1. Finally, CALCL exhibited 6q27 and 13q34 losses. Such imbalances were statistically associated with one CTCL subtype. Unsupervised hierarchical clustering defined three categories of clinical relevance: (1) CALCL apart from epidermotropic-CTCL, (2) an SS-only category, and (3) a mixed category with T-MF and SS cases, with both primary and secondary SS cases. In rare cases, the genetic classification did not correspond to the inclusion diagnosis, possibly reflecting the association of two diseases in the same patient or initial misdiagnosis according to follow-up. Finally, different samples in the same patient clustered together, showing reproducibility of such a classifier.

Primary cutaneous lymphomas: ESMO Clinical Practice Guidelines for diagnosis, treatment and follow-up

Primary cutaneous lymphomas: ESMO Clinical Practice Guidelines for diagnosis, treatment and follow-up

Widespread comedones as the sole clinical manifestation of follicular mycosis fungoides

Auteur(s) : Tiago Torres, Gloria Velho, Rosario Alves, Manuela Selores Department of Dermatology, Centro Hospitalar do Porto – Hospital de Santo Antonio, Porto, Portugal. Edificio das Consultas Externas do Hospital Geral Santo Antonio, Ex. CICAP, Rua D. Manuel II, s/n, 4100 Porto, Portugal Mycosis fungoides (MF) is the commonest type of cutaneous T-cell lymphoma and can manifest with a variety of clinical presentations. Histologically, it is characterized by infiltration of the epidermis [...]

Mycosis fungoides bullosa: a case report and review of the literature

IntroductionMycosis fungoides, the most common type of cutaneous T-cell lymphoma, can manifest in a variety of clinical and histological forms. Bulla formation is an uncommon finding in mycosis fungoides and only approximately 20 cases have been reported in the literature.Case presentationWe present a case of rapidly progressive mycosis fungoides in a 68-year-old Caucasian man who initially presented with erythematous plaques characterised by blister formation.ConclusionAlthough mycosis fungoides bullosa is extremely rare, it has to be regarded as an important clinical subtype of cutaneous T-cell lymphoma. Mycosis fungoides bullosa represents a particularly aggressive form of mycosis fungoides and is associated with a poor prognosis. The rapid disease progression in our patient confirms bulla formation as an adverse prognostic sign in cutaneous T-cell lymphoma.

Papular mycosis fungoides: two new cases of a recently described clinicopathological variant of early mycosis fungoides

Mycosis fungoides (MF) is the most common type of cutaneous T-cell lymphoma. In early stages of the disease many different clinicopathologic variants have been observed. Currently, a papular variant of MF which is characterized by a good prognosis has been described. Because only seven cases have been reported in the literature, clinical and morphological data of this variant are not well established. We report the clinical and histopathological characteristics of two new patients with papular mycosis fungoides and review the previous cases reported in the literature. The two cases of this early variant of MF were characterized by the presence of papules which, unlike the papules of lymphomatoid papulosis, did not show a tendency for spontaneous resolution. Histologic examination confirmed the diagnosis of MF in all cases. Immunohistochemical staining for CD30 was negative in all two cases. Follow-up data of our two patients confirmed the non-aggressive behavior of the disease, confirming that the lesions were not manifestations of advanced MF. Papular MF is a new variant of early MF characterized by a good prognosis in the long term follow- up. Thus, it should be added to the long list of clinicopathologic subtypes of MF.