1/19. Granulomatous mycosis fungoides: report of a case with some histopathologic features of granulomatous slack skin.We describe a case of granulomatous mycosis fungoides, tumor stage, mimicking sarcoidosis in an 82-year-old man with a 2-year history of skin disease. The final diagnosis was established after one of seven biopsy specimens showed a nongranulomatous histologic picture of patch-stage mycosis fungoides. Monoclonality was proven for the lymphocytic population by T-cell-receptor rearrangement studies. The unusually extensive granulomatous inflammation with huge giant cells surrounded by CD1a-positive cells in the other six biopsy specimens was suggestive of the histopathology of granulomatous slack skin, another rare granulomatous cutaneous T-cell lymphoma. Because both a clinical and histologic overlap between granulomatous mycosis fungoides and granulomatous slack skin have been reported in the literature, we conclude that they may belong to the spectrum of a single disease.- - - - - - - - - - ranking = 1keywords = skin disease (Clic here for more details about this article) |
2/19. mycosis fungoides with mucinosis follicularis in childhood.mycosis fungoides is a cutaneous T-cell lymphoma (CTCL) usually observed in mid to late adulthood. It occurs only rarely during childhood. Follicular mucinosis is a chronic dermatosis involving the sebaceous glands and outer root sheaths. It is normally differentiated into a juvenile benign form and an adult form possibly associated with mycosis fungoides. We report a 12-year-old boy who presented with an 8-month history of erythematous mucinous plaques on the scalp. Three months later, he developed erythematous patches and plaques on his whole body, accompanied by cervical lymphadenopathy. A biopsy showed follicular mucinosis and epidermotropism of the lymphocytic infiltrate. immunophenotyping and a PCR clonality test were consistent with CTCL. The patient received PUVA treatment and local steroids, resulting in partial remission. mycosis fungoides should be considered in the differential diagnosis of chronic, scaling dermatoses in childhood. Moreover, follicular mucinosis in childhood can be associated with mycosis fungoides.- - - - - - - - - - ranking = 0.87144526940589keywords = dermatosis (Clic here for more details about this article) |
3/19. Nevoid hyperkeratosis of the areola with histopathological features mimicking mycosis fungoides.Hyperkeratosis of the areola is a rare benign condition of unknown etiology characterized by slowly growing verrucous thickening and brown pigmentation of the areola or/and the nipple. It may be presented as isolated nevoid form or associated with other skin diseases. We described a 21-year-old woman with characteristic lesions of nevoid hyperkeratosis in both areolas but with histopathological findings resembling mycosis fungoides. Cutaneous lesions have remained unchanged after two years of follow-up.- - - - - - - - - - ranking = 1keywords = skin disease (Clic here for more details about this article) |
4/19. alopecia mucinosa and lymphoma. Report of two cases and review of literature.A total of 90 cases of alopecia mucinosa (follicular mucinosis) have been reported in the world literature since Pinkus described the entity in 1957. alopecia mucinosa appears morphologically in any variety of three basic clinical types: grouped folliculopapules, plaque forms of folliculopapules, and as boggy nodular masses. Characteristic histochemical changes of follicular mucinosis have been demonstrated in alopecia mucinosa as well as in selected cases of mycosis fungoides and lymphoma. The majority of case reports of alopecia mucinosa have been in children and young adults (58%), and the course of the dermatosis was usually uneventful. Beyond age 40, the disease appears to behave differently in that it follows a prolonged chronic course, and of 28 cases in this category four (15%) developed into either mycosis fungoides or lymphoblastoma.- - - - - - - - - - ranking = 0.87144526940589keywords = dermatosis (Clic here for more details about this article) |
5/19. Composite mycosis fungoides and B-cell chronic lymphocytic leukemia.Concordant or composite mycosis fungoides and B-cell chronic lymphocytic leukemia (B-CLL) is exceedingly rare, with only 10 cases previously described to our knowledge. We report a case of a 64-year-old woman who developed generalized erythroderma 5 years after the diagnosis of early stage B-CLL. Over the next 6 years of her clinical course multiple sequential samples of skin, peripheral blood, and one enlarged lymph node were studied in detail by flow cytometry, immunohistochemistry, molecular diagnostics, and electron microscopy. The progressive cutaneous infiltrates were initially characterized as leukemia cutis, infiltration by B-CLL. Three years later, when she developed worsening skin disease and lymphadenopathy, the cutaneous infiltrates were characterized as cutaneous T-cell lymphoma. At that point, a biopsy of an enlarged lymph node revealed a composite lymphoma of both B-CLL and cutaneous T-cell lymphoma, and the peripheral blood also contained circulating cells of both neoplasms. Herein we summarize the literature on concordant cutaneous T-cell lymphoma and B-CLL, and the literature on concordant T- and B-cell neoplasms in general, with a review of the postulated relationships between these neoplasms.- - - - - - - - - - ranking = 1keywords = skin disease (Clic here for more details about this article) |
6/19. Cytotoxic mycosis fungoides evolving from pityriasis lichenoides chronica in a seventeen-year-old girl. Report of a case.pityriasis lichenoides chronica and its acute form, pityriasis lichenoides et varioliformis acuta, are skin diseases of unknown origin, that probably represent a hypersensitivity reaction to an infective agent. pityriasis lichenoides is often a benign disorder but, because of the presence of a clonal T cell population detected both in the chronic and acute forms, some authors have suggested that it may belong to the group of primary cutaneous T cell lymphomas. Although various studies have clearly documented no significant association between pityriasis lichenoides and malignant lymphomas, cases of long-standing pityriasis lichenoides evolving into mycosis fungoides have been described. Herein we report the case of a girl suffering from pityriasis lichenoides since the age of 11 years, subsequently developing a CD45RO , CD8 , TIA-1 mycosis fungoides.- - - - - - - - - - ranking = 1keywords = skin disease (Clic here for more details about this article) |
7/19. Low-dose oral bexarotene in combination with low-dose interferon alfa in the treatment of cutaneous T-cell lymphoma: clinical synergism and possible immunologic mechanisms.BACKGROUND: For nearly 2 decades clinicians have been treating cutaneous T-cell lymphoma (CTCL) with regimens that combine interferon alfa with retinoid compounds. In December 1999 a new retinoid, bexarotene, was approved by the US food and Drug Administration for the treatment of CTCL. At the manufacturer's recommended dose of bexarotene (300 mg/m(2) of body surface area), it has proven to be a highly effective therapy for all stages of CTCL. Nevertheless, this dose is typically associated with adverse effects including severe hyperlipidemia. Furthermore, there appears to be no standardization of dosing among physicians who treat CTCL. OBSERVATIONS: We present 3 representative patients, 2 with erythrodermic CTCL and 1 with follicular mycosis fungoides, who experienced the rapid clearing of skin disease while being treated with a combination of low-dose bexarotene and low-dose recombinant interferon alfa. CONCLUSIONS: Combining low-dose bexarotene with low-dose interferon alfa was well tolerated and led to rapid improvement in our patients. We review the clinical and biologic basis for this approach.- - - - - - - - - - ranking = 1keywords = skin disease (Clic here for more details about this article) |
8/19. Clinical case of the month. A young man with a persistent skin eruption. Mycosis fungoides.There are many types of skin disease that fit into the classification of cutaneous lymphoma, but mycosis fungoides is by far the most common of this group. It is a non-Hodgkin's lymphoma of T-cell origin that presents in the skin. mycosis fungoides often evolves for years without a specific diagnosis because it can present as an eczematous or psoriasiform eruption. patients identified in the early stages and treated appropriately have a normal life expectancy.- - - - - - - - - - ranking = 1keywords = skin disease (Clic here for more details about this article) |
9/19. mycosis fungoides-type cutaneous T-cell lymphoma and neutrophilic dermatosis.BACKGROUND: mycosis fungoides (MF) is the most common type of cutaneous T-cell lymphoma (CTCL). patients with limited patch and/or plaque disease have a normal life expectancy. Neutrophilic dermatosis (ND) may be associated with various hematologic disorders. However, its association with CTCL is exceptional and has been reported only twice with leukemic forms of CTCL. OBSERVATIONS: Three patients with MF developed ND resistant to conventional therapies and responsible for an impaired quality of life due to constitutional symptoms and painful cutaneous lesions. All patients underwent an aggressive treatment course despite their varying initial clinical stages of MF, and all experienced a fatal outcome less than 18 months after the onset of ND. CONCLUSIONS: The association of MF with ND is exceptional and carries a poor prognosis, but the pathophysiologic nature of this association remains unclear. It may involve neutrophil chemoattractant cytokine production by tumor cells. A triggering role of interferon alfa is also possible.- - - - - - - - - - ranking = 4.3572263470294keywords = dermatosis (Clic here for more details about this article) |
10/19. Idiopathic guttate hypomelanosis: idiopathic or ultraviolet induced?Idiopathic guttate hypomelanosis (IGH) is a common but often unrecognized skin disease with obscure etiopathogenesis. As the lesions mostly develop on the exposed parts of the extremities, ultraviolet has been hypothesized to induce these lesions. However, previous statistical studies did not confirm any association between ultraviolet and IGH. We describe a 72-year-old woman with mycosis fungoides in whom widespread IGH occurred during narrow band ultraviolet B (UVB) therapy. The lesion development in the trunk following narrow band UVB therapy suggests that these lesions may not be idiopathic and they may be associated with UV exposure although controversy exist in the literature. This case is particular as it reveals strong evidence on this relationship.- - - - - - - - - - ranking = 1keywords = skin disease (Clic here for more details about this article) |
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