Parapsoriasis
PUVA Therapy
Mycosis Fungoides
Ultraviolet Therapy
Lymphoma, T-Cell, Cutaneous
Demonstration of frequent occurrence of clonal T cells in the peripheral blood but not in the skin of patients with small plaque parapsoriasis. (1/13)
Clinical, immunohistological, and molecular biological data suggest the chronic dermatosis small plaque parapsoriasis (SPP) to be a precursor of mycosis fungoides (MF). However, most data are contradictory and confusing due to inexact definition of SPP. Recently, clonal T cells were detected in skin and blood samples of early MF. Because demonstration of identical T-cell clones in skin and blood of SPP patients would indicate a close relationship of SPP to MF, we investigated the clonality of skin and blood specimens from 14 well-defined SPP patients. By a polymerase chain reaction (PCR) amplifying T-cell receptor gamma rearrangements and subsequent high-resolution electrophoresis, clonal T cells were detected in 9 of 14 initial and 32 of 49 follow-up blood samples, but in 0 of 14 initial skin specimens. Even a clone-specific PCR showing the persistence of the initial blood T-cell clone in 20 of 20 follow-up samples, failed to detect the T-cell clone in the skin. In 2 patients, the clonal T cells were shown to be CD4(+). For the first time, the majority of SPP patients was shown to carry a T-cell clone in the peripheral blood. Although a relation between circulating clonal T cells and SPP cannot directly be proven by the applied techniques, our results indicate blood T-cell clonality to be a characteristic feature of SPP and CTCL because analysis of multiple controls and clinical workup of our SPP patients excluded other factors simulating or causing a clonal T-cell proliferation. A sufficient cutaneous antitumor response but also an extracutaneous origin of the T-cell clones might explain the failure to detect skin infiltrating clonal T cells. (+info)The aetiology of maculopapular rash diseases in Niteroi, State of Rio de Janeiro, Brazil: implications for measles surveillance. (2/13)
A study investigating the causes of rash diseases using systematic laboratory testing was conducted in Niteroi, Rio de Janeiro, between January 1994 to April 1998. Sera from 327 patients were tested for evidence of anti-rubella virus, measles virus, human parvovirus B19 and dengue fever virus specific immunoglobulin IgM and anti-human herpes virus type 6 (HHV-6) IgG antibodies. A laboratory confirmed diagnosis was achieved in 71.3% of the cases investigated: dengue fever (33.0%), rubella (20.2%), parvovirus B19 (9.2%), measles (6.7%) and HHV-6 (2.1%). No diagnosis was established for 94 cases (28.7%). An outbreak of measles was detected during 1997, with a peak in September and October. All of the diseases studied here presented with clinical features similar to measles and classical symptoms were found in all measles confirmed cases. The large overlap of combinations of signs and symptoms seen in this study highlights the difficulties of diagnosing a rash illness on clinical grounds alone. (+info)Mycosis fungoides. (3/13)
The case of a 46-year-old woman with poikiloderma vasculare atrophicans is discussed. It is a rare clinical form of patch-stage mycosis fungoides characterized by generalized poikiloderma, atrophy, mottled dyspigmentation, and telangiectases. (+info)CD13 and TCR clone: markers of early mycosis fungoides. (4/13)
Making a differential diagnosis between early mycosis fungoides and parapsoriasis is often difficult at the clinical and histological level. The aim of this study was to explore markers that could help in this process. A total of 88 patients were included in 2 categories: large plaque parapsoriasis and digitiform parapsoriasis. A histological examination was performed for each patient, and expression of the antigen My7 (CD13), which is lacking in cutaneous T-lymphomas (but not in inflammatory lesions) and rearrangement of the T-cell receptor gene were analysed. A histological aspect of epidermotropic cutaneous T-cell lymphoma was observed in 23.5% of cases of large plaque parapsoriasis and 15% of cases of digitiform parapsoriasis. A disappearance of My7 antigen was noted in the 2 forms of parapsoriasis, more frequently when there was cutaneous T-cell lymphoma histology. A cutaneous clone was observed in 10.3% of cases of large plaque parapsoriasis, but not of digitiform parapsoriasis. For 3 patients, a cutaneous clone and a disappearance of My7 were associated with a non-specific histology. Considering these histological, immunological and molecular biological data, it appears that My7 antigen combined with T-cell clone may help the dermatologist to confirm the diagnosis of early mycosis fungoides. Moreover, further studies will determine whether CD13 is an early prognostic marker of evolution of a parapsoriasis to mycosis fungoides. Finally, these results demonstrate that digitiform parapsoriasis can be an early stage of MF. (+info)Cutaneous manifestations in renal failure patients: a case series. (5/13)
Cutaneous involvement in renal disease is due to a host of factors ranging from metabolic disturbances to immunosuppressive drugs. Herein we report a series of six cases of renal failure with varied cutaneous manifestations ranging from infections to neoplasms due to prolonged immunosuppression. Our first case had cutaneous cryptococcosis where skin lesions gave a clue to the diagnosis of altered sensorium and underlying meningitis. The second case initially presented with florid warts and was treated successfully but later presented with an explosive recurrence of skin lesions due to malignant transformation. Our third case had basal cell carcinoma over the presternal region that was successfully treated with liquid nitrogen cryotherapy. Our fourth case had diabetic nephropathy that presented with septicemia and purpura fulminans. The last case had cutaneous manifestations of drug therapy because of heparin infusion. To conclude, cutaneous manifestations in patients with renal failure are varied and a high degree of suspicion is needed for early diagnosis and aggressive treatment to effectively combat mortality and morbidity. (+info)Retroperitoneal liposarcoma associated with small plaque parapsoriasis. (6/13)
BACKGROUND: Extremely rare cases of paraneoplastic syndromes or ectopic production of proteins associated with liposarcoma are reported in literature. Production of Granulocyte-Colony Stimulating Factor, alpha-fetoprotein, paraneoplastic pemphigus and leucocytosis, Acrokeratosis paraneoplastica (Bazex's syndrome) are reported. The present report describes a case of retroperitoneal liposarcoma associated with small plaque parapsoriasis. Our search in the English literature of such a kind of association did not reveal any case reported. CASE PRESENTATION: A 74 year male patient was admitted to our hospital because of the presence of an abdominal mass in right iliac fossa. He also complained of a two-year history of psoriasiform eruptions. The CT scan showed a retroperitoneal pelvic mass. Therefore surgical resection of the tumor was performed. After surgery, the skin eruptions disappeared completely in seven days and so a diagnosis of parapsoriasis syndrome was done. CONCLUSION: Parallel disappearing of skin eruptions after surgery, typical clinical picture and not specific histology of the cutaneous lesions suggest the diagnosis of small plaque parapsoriasis. Therefore we propose to add Small Plaque Parapsoriasis to the list of paraneoplastic syndromes associated to liposarcoma. (+info)TCRgamma gene rearrangement analysis in skin samples and peripheral blood of mycosis fungoides patients. (7/13)
BACKGROUND: Diagnosing mycosis fungoides (MF) can be challenging in the early stage of the disease because histopathological features may simulate a variety of benign inflammatory skin diseases. Assessment of T-cell clonality was found to be useful in diagnosis and follow-up of patients. OBJECTIVE: In this study, PCR-based TCRgamma gene rearrangement analysis was performed in skin and peripheral blood samples of patients with MF treated at the two largest referral centers in Serbia, and the results obtained were correlated with clinical and follow-up data. METHODS: Skin and peripheral blood samples were obtained with informed consent from 37 patients treated at the Department of Dermatology of the Military Medical Academy and the Medical Center of Serbia from 2001 to 2006. The median time of follow-up was 4 years. Multiplex PCR was used for TCRgamma gene rearrangement analysis in skin and peripheral blood samples. Clonality results were correlated with the clinical data and disease course data. RESULTS: Monoclonality was detected in skin samples of 30/37 patients (81%), in 2/5 patients with large-plaque parapsoriasis (LPP), in 28/32 (88%) patients with histologically proven MF, and in 1/16 (6%) patients with benign inflammatory dermatoses. A monoclonal pattern in both skin and peripheral blood was detected in 7/16 (44%) patients in the late stage of the disease, and in 1/7 (14%) patients in the early stage of the disease. A dominant clone was found in both skin and peripheral blood in 1/4 patients in remission, 2/5 with a stable disease, and 4/9 (44%) with disease progression. CONCLUSION: TCR-gamma gene rearrangement analysis can be regarded as a useful adjunct to diagnosis of epidermotropic lymphoproliferative disorders. The presence of a dominant clone in both the skin and peripheral blood was more frequently detected in late stages and in patients with disease progression, confirming the usefulness of clonality detection by TCR-gamma gene rearrangement analysis in follow-up of patients with primary cutaneous T-cell lymphomas. (+info)The conundrum of parapsoriasis versus patch stage of mycosis fungoides. (8/13)
(+info)Parapsoriasis is a term used to describe two uncommon, chronic, and relatively benign inflammatory skin conditions. These are small plaque parapsoriasis (SPP) and large plaque parapsoriasis (LPP), also known as retiform or digitate dermatosis of Köbner.
Small plaque parapsoriasis is characterized by scaly, thin, pink to red patches or plaques, usually less than 3-5 cm in diameter. The lesions are often asymptomatic or mildly pruritic and can be found on the trunk and proximal extremities.
Large plaque parapsoriasis presents as larger, irregularly shaped, scaly patches or thin plaques, typically greater than 5 cm in diameter. The lesions are often asymptomatic but may occasionally be pruritic. LPP is considered a precursor to a rare cutaneous T-cell lymphoma called mycosis fungoides, especially when the lesions become thicker or more numerous over time.
It's important to note that these conditions can sometimes be challenging to diagnose and may require a skin biopsy for accurate diagnosis. Dermatologists and pathologists should carefully evaluate the clinical presentation, histopathological features, and any potential progression to ensure appropriate management.
PUVA therapy is a type of treatment that uses both medication and light to treat certain skin conditions, such as psoriasis, eczema, and cutaneous T-cell lymphoma. The name "PUVA" stands for Psoralen + UVA, which refers to the two main components of the therapy:
1. Psoralen: This is a medication that makes the skin more sensitive to light. It can be taken orally or applied directly to the skin in the form of a cream or bath.
2. UVA: This stands for Ultraviolet A, which is a type of light that is part of the natural sunlight spectrum. In PUVA therapy, the skin is exposed to a controlled dose of UVA light in a special booth or room.
When psoralen is introduced into the body, it absorbs into the skin and makes it more sensitive to UVA light. When the skin is then exposed to UVA light, it triggers a chemical reaction that slows down the growth of affected skin cells. This helps to reduce inflammation, scaling, and other symptoms associated with the skin condition being treated.
It's important to note that PUVA therapy can have side effects, including sunburn, itching, redness, and an increased risk of skin cancer over time. As such, it is typically used as a second-line treatment when other therapies have not been effective, and it is closely monitored by a healthcare professional to ensure its safe and effective use.
Mycosis fungoides is the most common type of cutaneous T-cell lymphoma (CTCL), a rare cancer that affects the skin's immune system. It is characterized by the infiltration of malignant CD4+ T-lymphocytes into the skin, leading to the formation of patches, plaques, and tumors. The disease typically progresses slowly over many years, often starting with scaly, itchy rashes that can be mistaken for eczema or psoriasis. As the disease advances, tumors may form, and the lymphoma may spread to other organs, such as the lymph nodes, lungs, or spleen. Mycosis fungoides is not contagious and cannot be spread from person to person. The exact cause of mycosis fungoides is unknown, but it is thought to result from a combination of genetic, environmental, and immune system factors.
Ultraviolet (UV) therapy, also known as phototherapy, is a medical treatment that uses ultraviolet light to treat various skin conditions. The UV light can be delivered through natural sunlight or artificial sources, such as specialized lamps or lasers.
In medical settings, controlled doses of UV light are used to target specific areas of the skin. The most common type of UV therapy is narrowband UVB (NB-UVB) phototherapy, which uses a specific wavelength of UVB light to treat conditions such as psoriasis, eczema, vitiligo, and dermatitis.
The goal of UV therapy is to reduce inflammation, slow skin cell growth, and improve the overall appearance of the skin. It is important to note that while UV therapy can be effective in treating certain skin conditions, it also carries risks such as skin aging and an increased risk of skin cancer. Therefore, it should only be administered under the supervision of a qualified healthcare professional.
Cutaneous T-cell lymphoma (CTCL) is a type of cancer that affects T-cells, a specific group of white blood cells called lymphocytes. These cells play a crucial role in the body's immune system and help protect against infection and disease. In CTCL, the T-cells become malignant and accumulate in the skin, leading to various skin symptoms and lesions.
CTCL is a subtype of non-Hodgkin lymphoma (NHL), which refers to a group of cancers that originate from lymphocytes. Within NHL, CTCL is categorized as a type of extranodal lymphoma since it primarily involves organs or tissues outside the lymphatic system, in this case, the skin.
The two most common subtypes of CTCL are mycosis fungoides and Sézary syndrome:
1. Mycosis fungoides (MF): This is the more prevalent form of CTCL, characterized by patches, plaques, or tumors on the skin. The lesions may be scaly, itchy, or change in size, shape, and color over time. MF usually progresses slowly, with early-stage disease often confined to the skin for several years before spreading to lymph nodes or other organs.
2. Sézary syndrome (SS): This is a more aggressive form of CTCL that involves not only the skin but also the blood and lymph nodes. SS is characterized by the presence of malignant T-cells, known as Sézary cells, in the peripheral blood. Patients with SS typically have generalized erythroderma (reddening and scaling of the entire body), pruritus (severe itching), lymphadenopathy (swollen lymph nodes), and alopecia (hair loss).
The diagnosis of CTCL usually involves a combination of clinical examination, skin biopsy, and immunophenotyping to identify the malignant T-cells. Treatment options depend on the stage and subtype of the disease and may include topical therapies, phototherapy, systemic medications, or targeted therapies.