Progressive Nodular Histiocytosis -a rare variant of the Histiocytoses

Although PNH is characterized by a progressive course and does not tend towards spontaneous resolution, patients usually remain in good general health without visceral involvement. The diagnosis of the condition relies heavily on experienced pathologists who actively look for the features of the disease. It is the hope, that with academic awareness, more of these patients will be diagnosed earlier, and thereby greatly improving treatment outcomes. If dermatologists and physicians are made aware of the condition, further investigations will be done and pathologists will be asked to look for the disease features and ask for special stains. With this, there may be many patients who are either misdiagnosed or without diagnosis. This will improve treatment outcomes. This is an extremely rare variant of the Histiocytosis group of conditions, and our patient is the first reported case in Africa. Our patient did not exhibit any systemic symptoms, but regular follow up is mandatory. It is with hope, that additional study of the immunologic and other host factors responsible for these proliferations may someday allow more targeted therapy of these currently resistant forms.

Autorentext

Dr Lushen Pillay is a Specialist Dermatologist and Dermatologic surgeon at Helen Joseph Hospital. He is the Head of Dept at Helen Joseph Hospital. He finished his undergraduate degree at the University of Pretoria, and then completed the Fellowship in Dermatology FC Derm(SA), as well as a Masters in Dermatology through Wits.