ABSTRACT
Report on a patient suffering from keratosis follicularis spinulosa decalvans (Siemens I syndrome). This ia a very rare clinical picture, at least in its complete form. The patient's father and brother are suffering from an abortive Siemens I syndrome. The condition is a form of hereditary parakeratosis, a spinulose keratosis with typical localization in the eyes and on the skin. Causal therapy is not possible; attempts at conservative and surgical treatment are described. Prognostically, the condition will probably come to a standstill at puberty. The description of this case is intended to illustrate the necessity of close cooperation between dermatologist and ophthalmologist.
Subject(s)
Darier Disease/pathology , Eye Diseases/pathology , Adolescent , Adult , Darier Disease/genetics , Darier Disease/surgery , Female , Humans , Male , SyndromeABSTRACT
A report is given on an eight months old child with a juvenile xanthogranuloma of the iris and a secondary glaucoma. After radiotherapy with 6 X 0,5 Gy under conventional deep therapy conditions, a soon regression of the increased intraocular pressure was observed. In case of a juvenile xanthogranuloma of the iris, this secondary glaucoma represents in any case an acute danger to the eye, so it must be treated immediately. Radiotherapy has to be considered as the method of choice; it is also discussed with regard to the risk and the avoidance of a radiation cataract which, however, has to be tolerated in certain cases in order to prevent greater troubles.
Subject(s)
Eye Neoplasms/radiotherapy , Iris Diseases/radiotherapy , Xanthogranuloma, Juvenile/radiotherapy , Cataract/etiology , Female , Glaucoma/etiology , Humans , Infant , Iris Diseases/complications , Risk , Xanthogranuloma, Juvenile/complicationsABSTRACT
With reference to a case of juvenile xanthogranuloma localized in the anterior uvea the authors describe the clinical signs, differential diagnosis, morphology and treatment of this disease. If the juvenile xanthogranuloma is located in the anterior uvea, the eye may be severely affected. Spontaneous hyphema in infants is characteristic. At present radiation is the most successful treatment for preserving the bulbus and visual function. The earlier the disease is discovered the better the chances of successful treatment.
Subject(s)
Uveal Diseases/diagnosis , Xanthogranuloma, Juvenile/diagnosis , Diagnosis, Differential , Humans , Infant , Male , Uveal Diseases/radiotherapy , Xanthogranuloma, Juvenile/radiotherapyABSTRACT
The authors describe two cases of the rare disease lymphangioma hemifaciale. Every ophthalmologist should know this disease in view of the complications such as hemorrhage or erysipelas. Treatment methods are discussed. Furthermore, lymphangioma must be taken into consideration in the differential diagnosis of ptosis, protrusio bulbi, protrusio conjunctivae and congenital hemifacial hypertrophy.
Subject(s)
Facial Neoplasms/pathology , Lymphangioma/pathology , Child , Diagnosis, Differential , Eye Neoplasms/pathology , Eye Neoplasms/surgery , Facial Neoplasms/etiology , Female , Humans , Infant , Lymphangioma/etiology , Lymphangioma/surgeryABSTRACT
The nevus of Ota (naevus fusco-coeruleus-ophthalmo-maxillaris) is demonstrated by two clinical cases. The authors describe the disease, the morphology and the problem of malignancy.
