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P H Hoeger - One of the best experts on this subject based on the ideXlab platform.

  • transition of Pityriasis lichenoides et varioliformis acuta to febrile ulceronecrotic mucha habermann disease is associated with elevated serum tumour necrosis factor α
    British Journal of Dermatology, 2005
    Co-Authors: Athanasios Tsianakas, P H Hoeger
    Abstract:

    Summary 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)-α. 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-α. We hypothesize that in future cases, treatment with TNF-α antagonists might represent a reasonable alternative to high-dose immunosuppressive therapy.

Jimenez J Joaquin - One of the best experts on this subject based on the ideXlab platform.

  • phototherapy for Pityriasis lichenoides in the pediatric population a review of the published literature
    American Journal of Clinical Dermatology, 2016
    Co-Authors: Eric L Maranda, Austin Huy Nguyen, Megan Smith, Lawrence A. Schachner, V. Patel, Jimenez J Joaquin
    Abstract:

    Background Pityriasis lichenoides (PL) is a dermatologic disorder that manifests in either the acute (Pityriasis lichenoides et varioliformis acuta) or the chronic form (Pityriasis lichenoides chronica, also known as parapsoriasis chronica). Traditional first-line therapy consists of corticosteroids or antibiotics; however, these treatments are often accompanied with multiple side effects and may be ineffective.

  • phototherapy for Pityriasis lichenoides in the pediatric population a review of the published literature
    American Journal of Clinical Dermatology, 2016
    Co-Authors: Eric L Maranda, Austin Huy Nguyen, Megan Smith, Lawrence A. Schachner, Vivek N Patel, Jimenez J Joaquin
    Abstract:

    Pityriasis lichenoides (PL) is a dermatologic disorder that manifests in either the acute (Pityriasis lichenoides et varioliformis acuta) or the chronic form (Pityriasis lichenoides chronica, also known as parapsoriasis chronica). Traditional first-line therapy consists of corticosteroids or antibiotics; however, these treatments are often accompanied with multiple side effects and may be ineffective. The goal of this study was to review the use of phototherapy for treating PL in the pediatric population. We performed a systematic review of the literature in the National Library of Medicine’s PubMed database and the SCOPUS database discussing phototherapy for treatment of PL in the pediatric population. The following search terms were used: ‘Pityriasis lichenoides’, ‘Pityriasis lichenoides chronica’, ‘Pityriasis lichenoides et varioliformis acuta’, and ‘febrile ulceronecrotic Mucha-Habermann disease’. The systematic search and screening of articles resulted in 14 articles including a total of 64 patients with PL treated with phototherapy. Three different modalities were utilized, with five studies using broadband ultraviolet B (BB-UVB) radiation, nine studies utilizing narrowband UVB (NB-UVB), and two studies employing psoralen with ultraviolet A (PUVA) therapy. Overall, the use of BB-UVB had an initial clearance rate of 89.6 % with 23.1 % recurrence, whereas NB-UVB cleared 73 % of the lesions with no recurrence, and PUVA therapy initially cleared 83 % of the lesions with 60 % recurrence. The side-effect profiles were similar and revealed limited toxicity. Phototherapy shows promising results and a favorable side-effect profile in the treatment of PL. Ultimately, large randomized controlled trials are needed to determine optimal treatments.

Regina Finkpuches - One of the best experts on this subject based on the ideXlab platform.

  • Pityriasis lichenoides atypical Pityriasis lichenoides and related conditions a study of 66 cases
    The American Journal of Surgical Pathology, 2018
    Co-Authors: Tiziana Borra, Ana Custrin, Carlo Cota, William Vermi, A Saggini, Fabio Facchetti, Regina Finkpuches, Lorenzo Cerroni
    Abstract:

    Pityriasis lichenoides (PLs) is an uncommon skin disease of unknown etiology. In recent years, an atypical form of PL has been described, showing overlapping features with mycosis fungoides (MF) and lymphomatoid papulosis. We studied 66 patients with an initial histopathologic diagnosis of PL (M:F=3

  • febrile ulceronecrotic Pityriasis lichenoides et varioliformis acuta
    Journal of The American Academy of Dermatology, 1994
    Co-Authors: Regina Finkpuches, Peter H Soyer, Helmut Kerl
    Abstract:

    An unusually severe form of Pityriasis lichenoides et varioliformis acuta (PLEVA) with a fatal outcome in an 82-year-old woman is reported. After a period of a mild eruption, extensive polymorphous, papular and ulcerohemorrhagic skin lesions developed, associated with intermittent high temperature and constitutional symptoms. Skin biopsies showed the typical histopathological changes of PLEVA. Early recognition of this severe variant of PLEVA is important, since the fulminating course can lead to death.

  • febrile ulceronecrotic Pityriasis lichenoides et varioliformis acuta
    Journal of The American Academy of Dermatology, 1994
    Co-Authors: Regina Finkpuches, Peter H Soyer, Helmut Kerl
    Abstract:

    Pityriasis lichenoides et varioliformis acuta (PLEVA; Mucha-Habermann disease) is a disease of unknown cause with an acute or subacute course characterized by hemorrhagic and papulonecrotic lesions. The eruption is accompanied by only minimal constitutional symptoms, and the general health of the patient is usually not affected. 1 A rare ulceronecrotic type of PLEVA associated with high fever has been described by Degos et a1.2; to our knowledge, seven patients with this variant have been reported to date. We describe a patient with the Degos type of PLEVA in whom large ulceronecrotic skin lesions and a fulminating course developed. Treatment with systemic corticosteroids was ineffective, but administration of methotrexate (MTX) produced rapid clearing without recurrence.

Helmut Kerl - One of the best experts on this subject based on the ideXlab platform.

  • febrile ulceronecrotic Pityriasis lichenoides et varioliformis acuta
    Journal of The American Academy of Dermatology, 1994
    Co-Authors: Regina Finkpuches, Peter H Soyer, Helmut Kerl
    Abstract:

    An unusually severe form of Pityriasis lichenoides et varioliformis acuta (PLEVA) with a fatal outcome in an 82-year-old woman is reported. After a period of a mild eruption, extensive polymorphous, papular and ulcerohemorrhagic skin lesions developed, associated with intermittent high temperature and constitutional symptoms. Skin biopsies showed the typical histopathological changes of PLEVA. Early recognition of this severe variant of PLEVA is important, since the fulminating course can lead to death.

  • febrile ulceronecrotic Pityriasis lichenoides et varioliformis acuta
    Journal of The American Academy of Dermatology, 1994
    Co-Authors: Regina Finkpuches, Peter H Soyer, Helmut Kerl
    Abstract:

    Pityriasis lichenoides et varioliformis acuta (PLEVA; Mucha-Habermann disease) is a disease of unknown cause with an acute or subacute course characterized by hemorrhagic and papulonecrotic lesions. The eruption is accompanied by only minimal constitutional symptoms, and the general health of the patient is usually not affected. 1 A rare ulceronecrotic type of PLEVA associated with high fever has been described by Degos et a1.2; to our knowledge, seven patients with this variant have been reported to date. We describe a patient with the Degos type of PLEVA in whom large ulceronecrotic skin lesions and a fulminating course developed. Treatment with systemic corticosteroids was ineffective, but administration of methotrexate (MTX) produced rapid clearing without recurrence.

Erin M. Warshaw - One of the best experts on this subject based on the ideXlab platform.

  • Pityriasis lichenoides and its subtypes
    Journal of The American Academy of Dermatology, 2006
    Co-Authors: Sacharitha Bowers, Erin M. Warshaw
    Abstract:

    Pityriasis lichenoides represents a unique group of inflammatory skin disorders that include Pityriasis lichenoides et varioliformis acuta (PLEVA), febrile ulceronecrotic Mucha-Habermann disease (a subtype of PLEVA), and Pityriasis lichenoides chronica. The history, epidemiology, clinical features, pathophysiology, and treatment of this group of conditions are reviewed in this manuscript. Learning objective At the completion of this learning activity, participants should be familiar with the clinical manifestations, histopathological findings, proposed mechanisms for pathogenesis, methods of treatment, and potential outcomes of Pityriasis lichenoides and its subtypes.

  • Pityriasis lichenoides and its subtypes.
    Journal of the American Academy of Dermatology, 2006
    Co-Authors: Sacharitha Bowers, Erin M. Warshaw
    Abstract:

    Pityriasis lichenoides represents a unique group of inflammatory skin disorders that include Pityriasis lichenoides et varioliformis acuta (PLEVA), febrile ulceronecrotic Mucha-Habermann disease (a subtype of PLEVA), and Pityriasis lichenoides chronica. The history, epidemiology, clinical features, pathophysiology, and treatment of this group of conditions are reviewed in this manuscript.