Introduction

Sarcoidosis is a relatively uncommon multi-systemic immunological disorder of unknown cause(s) with variable clinical manifestations and outcomes. Many of the earliest investigators are associated with sarcoidosis in eponymous perpetuity. Jonathan Hutchinson, a British dermatologist, first reported and illustrated a case of sarcoidosis in 1877 under the title of “Case of livid papillary psoriasis”. In 1895 he presented the case of a 64-year-old woman with facial and upper extremity skin lesions, which were neither tuberculosis nor lupus and named it Mortimer's malady. Yet Mrs Mortimer was not willing to undergo a skin biopsy and only in 1897 did Caesar Boeck manage to biopsy a 34-year-old policeman with cutaneous lesions.

The term “sarcoid” derives from Boeck's initial histological description of the tumoral non-necrotizing granulomas as sarkoid (Greek for flesh- or sarcoma-like). Dr Arthur Conan Doyle, a London contemporary of Dr Hutchinson, was probably the first to describe familial sarcoid and made cutaneous sarcoid an integral part of the plot of The Adventures of the Blanched Soldier. Visceral involvement, including the first description of lung disease, was reported during the First World War. Swedish physicians Jorgen Schaumann and Sven Löfgren applied chest radiography to sarcoid patients and defined hilar and mediastinal nodal disease in the first two decades of the twentieth century; years before Löfgren described the syndrome of erythema nodosum, polyarthritis, hilar adenopathy and fever that now bears his name. Morton Ansgar Kveim noted that intradermal inoculation of sarcoid lymph node tissue elicited a skin reaction in virtually all sarcoid patients. Louis Siltzbach modified this test using splenic tissue from affected individuals and organized an international study: hence the Kveim-Siltzbach test.