ABSTRACT
Patient was a 69 year old white female who presented with diffuse brownish-reddish and normal skin colored nodules of a diameter ranging from 0.5-4 cm. Histologic examination showed histiocytosis X. Despite the retinoid-PUVA, and combination of glucocorticoid and chemotherapy the disease progressed and in 8 months after the appearance of the skin lesions the patient expired. The authors briefly summarise the clinical, histological features of histiocytosis X and the therapeutic possibilities.
Subject(s)
Histiocytosis, Langerhans-Cell/diagnosis , Aged , Diagnosis, Differential , Fatal Outcome , Female , Histiocytosis, Langerhans-Cell/pathology , HumansABSTRACT
In the past 10 years the authors have observed 27 people (13 men and 14 women) suffering from Darier's disease in Somogy county. Out of these 22 cases were familiar and 5 isolated. The praevalence of dysceratosis follicularis vegetans was found 1 to 16,000 in the area. In the 3 familiar cases the dysceratotic papules of Darier's disease followed the naevus-lines, drawing out the Arnosan-triangle in the meantime. In the case of one of the nonfamiliar occurrences the Darier's diseased papules running stripelike also wrote out the naevus-lines, strictly localized to one side of the body. The stripes composed by the dysceratotic papules ran bipolarly in these cases too; heading from a point of the lumbosacral part of the median line of the back towards the navel. According to the authors the direction of the development of the epidermal elements of the skin is expressed in the early phase of embryonic life by the dysceratotic papules running bipolarly and following naevus-lines as embryological rudiments.
