Cases reported "Pityriasis Lichenoides"

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11/27. Febrile ulceronecrotic Mucha-Habermann disease with extensive skin necrosis in intertriginous areas.

    Febrile ulceronecrotic Mucha-Habermann disease (FUMHD) is a severe variant of pityriasis lichenoides et varioliformis acuta characterized by high fever and papulonecrotic skin lesions. Here we report a case of a 14-year-old boy with typical features of FUMHD and unusual manifestation of extensive skin necrosis in intertriginous regions including axillae, neck, inguinal and antecubital areas. Systemic administration of corticosteroid and erythromycin led to rapid healing of ulcerations without residual scar formation. review of the literature showed male-predominance and favorable outcome in pediatric cases of FUMHD.
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12/27. pityriasis lichenoides et varioliformis acuta immunohistopathologic study.

    immunoglobulin m and complement (Clq, C3 and C9) were found to be deposited together in the walls of affected blood vessels and also in the basement membrane zone of skin lesions from two patients with pityriasis lichenoides et varioliformis acuta (PLVA). The fluorescence appeared to be most intensely distributed in blood vessels which were affected by inflammatory infiltrates and frequently showed hyaline or fibrinoid changes of their walls. Deposits of C3 activator, fibrin and albumin were not observed. These findings suggest that the disease process in PLVA is the result of a vasculitis mediated by an immune complex and possibly by activation of the classical complement pathway. In addition, serum IgM was slightly increased in both patients.
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13/27. Febrile ulceronecrotic Mucha-Habermann disease with clonality: a cutaneous T-cell lymphoma entity?

    Febrile ulceronecrotic Mucha-Habermann disease (FUMHD) is a severe variant of pityriasis lichenoides et varioliformis acuta (PLEVA). PLEVA patients only very rarely have systemic signs; the cutaneous lesions are usually asymptomatic, but may be pruritic and may heal with scarring. FUMHD often starts out as classic PLEVA, but goes on to develop widespread ulceronecrotic lesions and is associated with a high mortality rate. Whether pityriasis lichenoides chronica (PLC) and PLEVA form a spectrum rather than single entities of clonal lymphoproliferative diseases has been discussed. Recently, it has been proposed that FUMHD, too, is a clonal lymphoproliferative disorder. Here, we report two cases of FUMHD with monoclonal T-cell population, as detected by Southern blot analysis. We propose that clonal FUMHD represents a cutaneous T-cell lymphoma entity.
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14/27. pityriasis lichenoides chronica.

    A 19-year-old woman presented with a five-year history of guttate macules and yellow to skin-colored papules with collarette of fine scale on the trunk and the upper and lower extremities. Guttate pityriasis lichenoides chronica is an uncommon presentation of this T-cell-mediated disease. It is of unknown etiology; however, an infectious agent has been suspected. It is considered in a spectrum with pityriasis lichenoides et varioloformis acuta and rarely mycosis fungoides and CD30 lymphomas. Improvement has been shown after treatment with oral tetracyclines, ultraviolet B and UVA1 phototherapy, and PUVA photochemotherapy.
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ranking = 2
keywords = pityriasis
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15/27. Transition of pityriasis lichenoides et varioliformis acuta to febrile ulceronecrotic Mucha-Habermann disease is associated with elevated serum tumour necrosis factor-alpha.

    Febrile ulceronecrotic Mucha-Habermann disease (FUMHD) represents a fulminant and potentially lethal variant of pityriasis lichenoides. Only 24 cases have been described so far. We report a 9-year-old boy who initially presented with classical pityriasis lichenoides et varioliformis acuta (PLEVA) following a mild enteritis. Three weeks later, his skin lesions started to ulcerate progressively, involving > 90% of his body surface, accompanied by high fever, normal c-reactive protein, but highly elevated serum levels of tumour necrosis factor (TNF)-alpha. methotrexate 10 mg m(-2) weekly was required to halt disease progression, while oral steroids (initial dose 2.8 mg kg(-1) daily) alone proved insufficient. Sequential histology revealed progressively dense perivascular and intramural lymphocytic inflammation as well as keratinocyte necrosis. Our case demonstrates the clinical and histological continuum between 'classical' PLEVA and FUMHD and points to the potentially pathogenic significance of TNF-alpha. We hypothesize that in future cases, treatment with TNF-alpha antagonists might represent a reasonable alternative to high-dose immunosuppressive therapy.
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ranking = 6
keywords = pityriasis
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16/27. Paraneoplastic pityriasis lichenoides in cutaneous lymphoma: case report and review of the literature on paraneoplastic reactions of the skin in lymphoma and leukaemia.

    Paraneoplastic dermatoses are non-neoplastic skin disorders which occur in the context of an underlying malignant neoplasm. The classic paraneoplastic dermatoses are mostly associated with solid internal malignancies. They only rarely occur in the context of nodal or primary cutaneous lymphomas. Apart from these classic paraneoplastic dermatoses, there are additional skin disorders reported to occur in close association with haematological and lymphoproliferative disorders which can thus be regarded as paraneoplastic manifestations. We report for the first time two patients with pityriasis lichenoides et varioliformis acuta in association with mycosis fungoides. In addition, we review the literature on paraneoplastic dermatoses of the skin which have been described in patients with leukaemias and primary cutaneous lymphomas.
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keywords = pityriasis
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17/27. A case report of pityriasis lichenoides in a patient with chronic hepatitis c.

    The authors describe a case of chronic hepatitis c associated with pityriasis lichenoides. The association and evolution during antiviral treatment of these two diseases in this patient point to a possible pathogenetic link between chronic C virus infection and pityriasis lichenoides.
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ranking = 6
keywords = pityriasis
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18/27. Septic, CD-30 positive febrile ulceronecrotic pityriasis lichenoides et varioliformis acuta.

    We report life-threatening febrile ulceronecrotic pityriasis lichenoides et varioliformis acuta in an 8-year-old girl. Hemorrhagic-crusted papules and plaques covered over 90% of the patient's body, leaving her susceptible to pseudomonas aeruginosa and staphylococcus epidermidis bacteremia as well as candida parapsilosis fungemia. sepsis delayed definitive treatment of the underlying cutaneous disease for 2 weeks. Combined therapy with methotrexate and cyclosporin caused remission of the process. Although immunohistochemistry revealed CD-30 positive cells, suggesting the diagnosis of lymphomatoid papulosis, the histopathology was most compatible with pityriasis lichenoides et varioliformis acuta. A partial loss of CD2 and CD5 in the predominant CD3 T-cell lymphocytes suggested a clonal proliferation. Elevated soluble interleukin-2 receptor levels reflected marked T-cell activation, and the downward trend of the levels during treatment coincided with clinical regression of this inflammatory dermatosis.
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ranking = 6
keywords = pityriasis
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19/27. The role of cytotoxic skin-homing CD8 lymphocytes in cutaneous cytotoxic T-cell lymphoma and pityriasis lichenoides.

    BACKGROUND: pityriasis lichenoides (PL) is a rare cutaneous lymphoproliferative disorder of unknown origin. Malignant transitions of PL have been described, but are very rare. We recently observed the fatal course of a 26-year-old patient who presented with a clinical picture resembling PL but had cytotoxic CD8 T-cell lymphoma of the skin (CxCTL). This case prompted us to reinvestigate the role of cytotoxic T lymphocytes in PL and its relationship to antiviral immunity. methods: skin biopsy specimens of 11 patients with PL and two biopsy specimens of CxCTL were included. In all, 5 biopsy specimens taken from healthy skin and 5 samples of varicella-zoster virus (VZV) skin lesions were analyzed for control purposes. The inflammatory infiltrate was characterized by immunohistochemistry using monoclonal antibodies against CD3, CD4, CD8, CD20, cutaneous lymphocyte-associated antigen (CLA), CCR4, CXCR3, Granzyme B, Tia-1, and MxA. flow cytometry was used to analyze the expression of chemokine receptors on peripheral blood mononuclear cells in CxCTL. RESULTS: The CxCTL skin lesions were dominated by a dense infiltration of CD8 cytotoxic lymphocytes with a skin-homing CLA CCR4 phenotype. PL and VZV skin lesions were also characterized by a predominantly CD8 T cellular infiltrate with strong expression of the cytotoxic molecules Granzyme B and Tia-1 and the skin-homing molecules CLA and CCR4. Coexpression analyses confirmed that skin CLA CD8 cytotoxic T cells are present in CxCTL, VZV, and PL skin lesions. Strong lesional production of the antiviral protein MxA, which is specifically induced by type I interferons, could be found in all investigated disorders. The study was based on histologic, immunohistologic, and flow cytometric analyses in a limited number of patients, because of the rareness of the investigated diseases. CONCLUSION: Our results revealed a striking similarity between the immunohistologic picture of malignant CxCTL, benign PL, and VZV skin lesions. Strong expression of the antiviral protein MxA in all disorders supports the view that a common antiviral immune response pattern leads to aberrant skin recruitment of CLA CCR4 cytotoxic T lymphocytes in PL and CxCTL.
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ranking = 4
keywords = pityriasis
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20/27. pityriasis lichenoides and idiopathic thrombocytopenic purpura in a young girl.

    pityriasis lichenoides is an inflammatory skin disorder characterized by erythematous, desquamative papules and plaques. An acute form, pityriasis lichenoides et varioliformis acuta, and a chronic form, pityriasis lichenoides chronica, represent the two ends of the spectrum of this disorder. Most commonly seen in children and young adults, its etiology is unknown. We describe a young patient with concurrent pityriasis lichenoides and idiopathic thrombocytopenia purpura, a previously unreported association.
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ranking = 3
keywords = pityriasis
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