RESUMEN
Cold-associated perniosis of the thighs ("equestrian cold panniculitis") is an unusual and still enigmatic entity. The authors retrieved 6 cases for a re-evaluation of their clinicopathologic features and for an immunohistochemical assessment with antibodies anti-CD3, anti-CD20, and anti-CD123. All patients were women, aged 17-45 years. One of them had elevated antinuclear antibody titers. Available anamnestic data confirmed the triggering role of prolonged/intermittent exposure to cold (not necessarily for equestrian activities). The lesions affected the thighs, with a preferential, although not exclusive involvement of the upper lateral surface. The histopathological pattern was perivascular, superficial, and deep, extending toward the superficial fat lobules, with lymphocytic vasculitis and mucin deposition; clumps of CD123 cells were found in 4 of 6 cases. Cold-associated perniosis of the thighs cannot be considered as a panniculitis. The histopathological features considerably overlap with perniosis at other sites of the body and with chilblain lupus erythematosus.
Asunto(s)
Tejido Adiposo/patología , Eritema Pernio/diagnóstico , Frío/efectos adversos , Dermis/patología , Inmunohistoquímica , Paniculitis/diagnóstico , Paniculitis/patología , Tejido Adiposo/química , Adolescente , Adulto , Antígenos CD20/análisis , Biomarcadores/análisis , Vasos Sanguíneos/patología , Complejo CD3/análisis , Eritema Pernio/clasificación , Eritema Pernio/etiología , Eritema Pernio/patología , Dermis/irrigación sanguínea , Dermis/química , Diagnóstico Diferencial , Femenino , Humanos , Subunidad alfa del Receptor de Interleucina-3/análisis , Lupus Eritematoso Cutáneo/patología , Persona de Mediana Edad , Paniculitis/clasificación , Paniculitis/etiología , Valor Predictivo de las Pruebas , Terminología como Asunto , Muslo , Adulto JovenRESUMEN
The common feature of all the conditions discussed in this article is that they are inflammatory vascular dermatoses that may occur as reactive processes in association with other diseases. The histopathologic characteristics of the lesions range from mild, perivascular dermal infiltration with inflammatory cells and vasodilation to various degrees of vessel damage (endothelial swelling to fibrinoid necrosis). The vessel damage is reflected in varying degrees of secondary changes including extravasation of edema fluid, extravasation of erythrocytes, epidermal necrosis, separation of the dermal-epidermal junction zone, and widespread tissue necrosis. The etiology of most of these conditions is still unknown, although strong evidence indicates that type III (circulating immune-complex-mediated) pathogenesis may be responsible for necrotizing venulitis (leukocytoclastic vasculitis) and that a type I (IgE-mediated) pathogenesis may be involved in some types of urticaria. For many of the reactions described, the patients have serum sickness-like systemic signs and symptoms in addition to cutaneous lesions, and a circulating immune-complex-mediated pathogenesis has been considered. Future investigations must address the types of antigens and antibodies present in the circulating immune complexes, the detection of specific antigen in cutaneous blood vessels, the reproduction of lesions in experimental animals, and the mechanisms responsible for the spectrum of clinicopathologic lesions produced, with special attention to the possibility that vessel damage results from circulating immune complex-induced lymphocytic rather than only leukocytoclastic vasculitis.