RESUMEN
BACKGROUND: Lymphoplasmacytic plaque (LPP) is a recently described rare skin disease characterized by a dense dermal lymphohistiocytic infiltrate with polyclonal plasma cells. The clinical picture is distinct with reddish to brownish plaque with a predilection for the lower leg. LPP typically affects children. OBJECTIVE: To define clinical and histologic criteria of LPP and to develop a diagnostic flow chart. METHODS: We investigated six of our own LPP cases. Immunoglobulin light chains, IgG, IgG4, CD31, CD163 as a histiocytic marker were examined by immunohistochemistry. PCR-based molecular studies were conducted for borrelia sp., mycobacterial and leishmania sp. Moreover, 10 cases, which have been reported in the literature, were checked for the same features. RESULTS: We could differentiate three main histological patterns (superficial band-like only, [deep] dermal only and mixed). Acanthosis and interface dermatitis are key features in cases with a superficial band-like or mixed infiltrate. Granulomas and giant cells could be only found in about 30% of the cases. The number of plasma cells was variable accounting for 5-40% of the infiltrate. The number of blood vessels was increased in the majority of the cases. 'Free-floating' collagen bundles surrounded by histiocytes (pseudorosettes) were identified as a new histological feature. An infectious agent could be excluded in all cases. CONCLUSIONS: LPP is a long-standing skin disease, which may also occur in adults and in other body regions than the lower leg. Reproducible clinical and histological criteria allow delineating a diagnostic work-up for LPP.
Asunto(s)
Borrelia burgdorferi/aislamiento & purificación , ADN Bacteriano/análisis , ADN Protozoario/análisis , Leishmania/aislamiento & purificación , Mycobacterium/aislamiento & purificación , Enfermedades de la Piel/metabolismo , Enfermedades de la Piel/patología , Piel/química , Adolescente , Anciano , Antígenos CD/análisis , Antígenos de Diferenciación Mielomonocítica/análisis , Vasos Sanguíneos/patología , Borrelia burgdorferi/genética , Niño , Preescolar , Colágeno/ultraestructura , Femenino , Humanos , Inmunoglobulina G/análisis , Cadenas Ligeras de Inmunoglobulina/análisis , Leishmania/genética , Masculino , Persona de Mediana Edad , Mycobacterium/genética , Células Plasmáticas/patología , Molécula-1 de Adhesión Celular Endotelial de Plaqueta/análisis , Receptores de Superficie Celular/análisis , Piel/irrigación sanguínea , Adulto JovenRESUMEN
Bazex syndrome is a paraneoplastic acrokeratosis appearing mostly at the same time as the underlying neoplasm. We report a typical case with a squamous cell carcinoma of the tongue and classical cutaneous symptoms disappearing with the treatment of the tumour.
Asunto(s)
Carcinoma de Células Escamosas/patología , Queratosis/patología , Síndromes Paraneoplásicos/patología , Neoplasias de la Lengua/patología , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapéutico , Carcinoma de Células Escamosas/tratamiento farmacológico , Cisplatino/administración & dosificación , Oído Externo , Fluorouracilo/administración & dosificación , Pie , Mano , Humanos , Queratosis/tratamiento farmacológico , Queratosis/radioterapia , Masculino , Persona de Mediana Edad , Nariz , Síndromes Paraneoplásicos/tratamiento farmacológico , Síndromes Paraneoplásicos/radioterapia , Neoplasias de la Lengua/tratamiento farmacológicoRESUMEN
A 51-year old patient with congenital basal cell naevus syndrome was treated with etretinate (50-100 mg/daily) over a period of 5 years (cumulative dose 110 g). Clinically he developed a stiff back with pronounced disability of both hips. Radiologically, axial involvement of the spine with severe diffuse idiopathic skeletal hyperostosis especially of the lumbar spine, and ossifications of both hips were documented. Rheumatological implications of long-term therapy with etretinate are discussed.