Hemiatrofia facial progresiva y morfea en golpe de sable: ¿entidades diferentes o un espectro de la misma enfermedad?

La hemiatrofia facial progresiva (HFP) o sindrome de Parry-Romberg se caracteriza por la presencia de lesiones atroficas que comprometen unilateralmente la piel de la cara. La morfea en golpe de sable (MGS) es un subtipo de esclerodermia localizada de disposicion lineal en cara o cuero cabelludo. Ambas comparten multiples caracteristicas, por lo que diferenciarlas suele ser un gran desafio. Existen controversias acerca de si son procesos independientes o un espectro de una misma enfermedad. Objetivos. Describir las caracteristicas clinicas, hallazgos histologicos y de laboratorio de los pacientes con diagnostico de HFP, MGS o ambas entidades evaluados en nuestro servicio. Comparar ambas patologias y revisar la bibliografia publicada para intentar esclarecer si son entidades independientes o un espectro de la misma enfermedad. Material y metodos. Se realizo un estudio retrospectivo y descriptivo. Se incluyeron todos los pacientes con diagnostico de HFP, MGS o ambas entidades evaluados desde enero de 2002 hasta julio de 2014. Resultados. Se encontraron 83 pacientes con esclerodermia localizada. Se seleccionaron 12 pacientes: 4 con MGS, 4 con HFP y 4 con ambas entidades. Se observo la coexistencia con morfea en area extracefalica en un 25%, sin predominio por ningun grupo. En relacion con las manifestaciones extracutaneas, el 41,66% presento alteraciones oftalmologicas y/o otorrinolaringologicas, principalmente en el grupo con HFP o ambas patologias. Ningun paciente presento alteraciones neurologicas. Solo 2 pacientes, una con diagnostico de HFP y otra con HFP + MGS, presentaron FAN reactivo. Todos los pacientes presentaron estudio histopatologico compatible. Se pudieron revisar 6/12 biopsias cutaneas. Los principales hallazgos histopatologicos independientemente del diagnostico clinico fueron: esclerosis con hialinizacion del colageno e infiltrado inflamatorio mononuclear. Conclusion. Los hallazgos del presente estudio respecto de la clinica, la histologia y los resultados de laboratorio nos permiten sugerir que ambas entidades podrian ser consideradas como un espectro de una misma enfermedad. Los resultados aqui comentados son similares a los revisados en las escasas publicaciones de series de casos en la literatura nacional y extranjera (Dermatol. Argent., 2014, 20 (6): 391-399). Palabras clave: hemiatrofia facial progresiva, sindrome de Parry-Romberg, morfea en golpe de sable Progressive facial hemiatrophy and morphea en coup de sabre: different entities or a spectrum of the same disease? Abstract Progressive facial hemiatrophy (PFH) is characterized by the presence of unilateral atrophic lesions in the face. Morphea en coup de sabre (MCS) is a subtype of linear scleroderma localized in face or scalp. As they share many characteristics, differentiate them is usually a challenge. There is controversy about whether they are independent processes or a spectrum of the same disease. Objectives. To describe the clinical, histological and laboratory findings in patients with PFH, MCS or both entities evaluated in our service. Compare both pathologies and review the published literature in order to try to clarify whether they are independent entities or a spectrum of the same disease. Material and methods. A retrospective analysis was performed. All patients with PFH, MCS or both entities evaluated from January 2002 to July 2014 were included. Results. 83 patients with localized scleroderma were found. 12 patients were included: 4 with MCS, 4 with PFH and 4 with both entities. 25% had concomitant localized scleroderma in extracefalic areas, with no predominance of either group. Regarding to the extracutaneous manifestations, 41.66% had ophthalmologic and/or otorhinolaryngological disorders, mainly in the group with HFP or both diseases. No patient had neurological abnormalities. Only 2 patients with PFH had reactive FAN. All the patients had histopathology consistent. 6/12 skin biopsies were reviewed. The main histopathological findings were sclerosis with hyalinization of collagen and mononuclear inflammatory infiltrate. Conclusion. Based on the present study, the clinical, histological and laboratory findings allow us to suggest that both entities could be considered as a spectrum of the same disease. These results are similar to the few publications in national and international literature (Dermatol. Argent., 2014, 20 (6): 391-399). Keywords: progressive facial hemiatrophy, morphea en coup de sabre, Parry-Romberg syndrome.