Claude Huriez and his syndrome.

Claude Huriez (1907-1984) was a well-known French dermatologist. In the 1960s, Huriez and colleagues reported two families from northern France with a rare congenital genodermatosis-later known as Huriez syndrome. This syndrome is characterized by scleroatrophy of the hands and feet, nail hypoplasia, mild palmoplantar keratoderma, and hypohidrosis and is a cancer-prone genodermatosis. This report sheds light on Huriez and the syndrome that bears his name.

El primer artículo con fotografías de la revista Actas Dermo-Sifiliográficas: Hiperkeratosis universal congénita maligna. (Ictiosis fetal; keratoma maligno congénito, etc.), por Juan de Azúa / The First Article in the Journal Actas Dermo-Sifiliográficas Published With Photographs: Malignant Keratosis Diffusa Fetalis (Fetal Ichthyosis, Congenital Maligna Keratoma, etc. )

Se realiza un breve comentario del primer artículo publicado en la revista Actas Dermo-Sifiliográficas con documentación gráfica del caso clínico y de los hallazgos histológicos. Fue redactado por nuestro fundador, el Dr. Juan de Azúa, en el año 1909 (AU)

Brief comment is made on the first article published in the journal Actas Dermo-Sifiliográf icas with photographic documentation of the case history and histological findings. It was written by the founder of the journal, Dr. Juan de Azúa, in 1909 (AU)

Diagnóstico y tratamiento de la uretritis crónica en la sanidad militar con el cambio de siglo (XIX-XX) / Diagnosis and treatment of chronic urethritis in the Military health-care system with the change of the Century (XIX-XX)

OBJETIVO: Podemos afirmar que en los Hospitales Militares, instituciones pioneras dentro de la organización sanitaria del país, aproximadamente un 25% de la patología durante la segunda mitad del siglo XIX, correspondería a las Enfermedades Venéreas, existiendo salas dedicadas a este tipo de afecciones que inicialmente se asociaban a las Enfermedades del Aparato Genito-urinario. La "Gota Militar" o Uretritis Blenorrágica fue un padecimiento venéreo con gran incidencia y prevalencia en la era pre-antibiótica. En el presente trabajo revisaremos los métodos diagnósticos y terapéuticos realizados por los Médicos Militares Españoles durante el periodo indicado. MÉTODOS: Se realiza una introducción, interrelacionando los conocimientos de la época con el medio en que los Médicos Militares desarrollaban su actividad profesional. Se hace referencia a las Revistas de Sanidad Militar, vehículo de las inquietudes y demostración del nivel de conocimiento teórico y práctico que les distinguía. En base a sus Hojas de Servicio y a sus publicaciones, hacemos mención de los Urólogos originarios de la Sanidad Militar que más interés demostraron en el campo de la patología de la uretra, comentando la metodología que utilizaban en el diagnóstico y tratamiento de la Uretritis Blenorrágica. RESULTADOS Y CONCLUSIONES: La Sanidad Militar de finales del siglo XVIII y principios del XIX reunió a un grupo de profesionales que demostraron ingenio y perseverancia en el tratamiento de la "Gota Militar". Conocer los conceptos en el diagnóstico y manejo de las uretritis, mejora nuestra perspectiva en el conocimiento de estas patologías y nos confirma en la valoración de los avances de que hoy disponemos, gracias a la suma de esfuerzos de nuestros predecesores

BJECTIVES: We can say that in military hospitals, pioneer institutions in health-care in our country, approximately 25% of the pathology over the second half of the 19th century would correspond to sexually transmitted diseases (STD), with hospital wards dedicated to these diseases, initially associated with genitourinary diseases. The «military drip» or blennorrhagic urethritis was a venereal disease with great incidence and prevalence in the pre-antibiotic era. This article reviews the diagnostic and therapeutic methods employed by Spanish military doctors during such period. METHODS: In the introduction we state the relationship between the knowledge of the time and the environment in which military doctors developed their professional activity. We make reference to military health-care journals, vehicle for their worries and demonstration of the level of theoretical and practical knowledge they were distinguished for. Based on their service records and publications we refer the urologists from the military health-care system that showed a greater interest in the field of urethral diseases, talking about the methodology they used for diagnosis and treatment of blennorrhagic urethritis. RESULTS AND CONCLUSIONS: Military health-care at the end of 18th century and the beginning of the 19th century joined a group of professionals who demonstrated talent and perseverance in the treatment of "military drip". To know the concepts for the diagnosis and treatment of urethritis improves our perspective in the knowledge of these pathologies, and confirms us in the evaluation of the advances available for us today, thanks to the addition of efforts of our predecessors (AU)

Keratosis lichenoides chronica: proposal of a concept.

It has been a subject of controversy whether keratosis lichenoides chronica (KLC) is a distinctive inflammatory disease of the skin or whether it represents a manifestation of another well-known disease, such as lichen planus, lupus erythematosus, or lichen simplex chronicus. In search of clear criteria for diagnosis of KLC the entire literature pertinent to the subject was studied and findings clinical and histopathologic as they were telegraphed in them were compared with a patient of my own experience. Review of the literature reveals more than 60 patients in whom the diagnosis of KLC was made. Three categories emerge based on whether the findings presented in a particular article (1) do not permit any diagnosis to be rendered; (2) do allow a diagnosis specific to be made, such as of lichen simplex, lichen planus, or lupus erythematosus; or (3) do not correspond to any disease well defined, such as lichen simplex, lichen planus, lupus erythematosus, but seem to show attributes morphologic, clinically and histopathologically, that are repeatable. Patients diagnosed as having KLC obviously represent a potpourri of different diseases, the most common of them being lichen simplex chronicus, lichen planus, and lupus erythematosus. Fewer than 25 patients reported on, however, presented themselves with lesions very similar to one another clinically, namely, an eruption that involved the face in a manner reminiscent of seborrheic dermatitis and with tiny papules on the trunk and extremities, which assumed linear and reticulate shapes by way of confluence of lesions. Individual papules were infundibulocentric and acrosyringocentric. Findings histopathologic were those of a lichenoid interface dermatitis affiliated with numerous necrotic keratocytes and covered by parakeratosis housing neutrophils in staggered fashion. These patients seem to have an authentic and distinctive condition that is exceedingly rare. In conclusion, the diagnosis of KLC should be made only for patients who present themselves with features clinical and findings histopathologic that resemble closely those of what is summarized in this article under category 3.

Keratosis lichenoides chronica: the centenary of another Kaposi's disease.

In 1895, Kaposi used the term 'lichen ruber acuminatus verrucosus et reticularis' to describe the case of a 27-year-old woman with a linear, warty lichenoid eruption. We have reviewed the literature and found 50 cases, of which only 40 had common features reminiscent of the original description of Kaposi. These 'authentic' cases of keratosis lichenoides chronica (KLC) showed strong clinical and histological similarity, a uniformity indicating that KLC is an entity and is distinct from lichen planus. KLC may be associated with internal diseases such as glomerulonephritis and lymphoproliferative disorders.

Cholesteatoma of the external auditory canal and keratosis obturans.

Keratosis obturans and external auditory canal cholesteatoma have often been regarded as a single entity. However, these are two distinct disorders with their own clinical presentations, physical and pathologic findings, and treatment. Keratosis obturans is an accumulation of obstructive desquamated keratin in the external auditory meatus. External auditory canal cholesteatoma is an invasion and erosion of squamous epithelium into a localized area of the bony ear canal. The origin of both entities remains obscure. The clinical symptoms, pathologic processes, and treatment are outlined and compared. Case reports are presented to illustrate the features of these two diseases.