Lupus pernio: a clinico-radiological study of thirty-five cases.

Thirty-five patients with lupus pernio were observed in a series of 818 patients with clinical and histological evidence of sarcoidosis. This analysis provides the natural history of lupus pernio and its associated clinico-radiographic features. There was intrathoracic involvement in 74% of patients, upper respiratory tract disease in 54%, reticulo-endothelial involvement in 54%, bone cysts in 43% and ocular lesions in 37%. It predominated in West Indies-born women. Histological confirmation was easily obtained by nasal mucosal biopsy and this could be repeated serially to evaluate the response to various treatments. Nasal bone radiographs showed destruction of the nasal bones, porosis and alteration in bone texture. Peripheral bone cysts were evident in 50% of patients who were radiographed, and in about 50% of these patients, the cysts were found in both hands and feet.

Sarcoidosis of the upper respiratory tract (SURT).

A series of eight hundred and eighteen patients with histologically-confirmed multisystem sarcoidosis was analysed; within this series were 53 (6 per cent) patients with sarcoidosis of the upper respiratory tract (SURT). Two-thirds presented with predominantly upper respiratory tract symptoms when the systemic disorder was minimal or unrecognized. Women were affected twice as often as men. SURT is commonly associated with lupus pernio and other manifestations of chronic fibrotic sarcoidosis, and it follows the same indolent course. When granulomas are found on biopsy of the upper respiratory tract, there should be a management plan to determine whether they are due to multisystem sarcoidosis or other granulomatous disorders including tuberculosis, Wegener's granulomatosis and leprosy. About one-half of patients with SURT will need oral steroids, either alone or in combination with topical steroids or chloroquine.

Sarcoidosis of bone.

Sarcoidosis of bone has been observed in 29 patients for up to 43 years. It was present in the hands and/or feet in 26 patients, in the nasal bone in three and once each in the hard palate and temporal bones. There were three types of bone lesion: (a) lytic in 25 patients; rounded cortical or medullary lesions ranging in size from 1 mm to 1 cm in diameter, which on healing left a residual punched-out 'cyst'; (b) permeative in nine patients; these showed progressive cortical 'tunnelling' with remodelling of trabecular and cortical architecture; (c) destructive in three patients: rapidly progressive with pathological fractures and secondary joint surface involvement. Soft tissue swelling preceded the radiological abnormality for up to four yearts in 10 patients, accompanied it twice, followed it once and was absent on 16 (55 percent) occasions. Bone involvement was usually an incidental finding when sarcoidosis presented elsewhere. Other features included intrathoracic sarcoid (86 per cent), lupus pernio (48 per cent), skin plaques (41 per cent), ocular inflammation (48 per cent), nasal mucosal disease (24 per cent), lymphadenopathy (24 per cent), hepatomegaly (13 per cent), splenomegaly (10 per cent), and parotid enlargement (10 per cent). Pulmonary infiltration with or without lymphadenopathy was observed in three fifths and hilar adenopathy alone in one third of patients. Abnormalities in chest radiographs of patients with bone sarcoid resolved in only 20 per cent. Hypercalciuria was noted in one and hypercalcaemia in the other two patients with bone distruction.

Sarcoidosis of the upper respiratory tract and its association with lupus pernio.

In a series of 34 patients with sarcoidosis affecting the upper respiratory tract and nose, 26 had lupus pernio (LP) and 17 had sarcoidosis of the upper respiratory tract (SURT). In nine patients these features coexisted. A patient presenting with SURT carried a 50% risk of developing LP although one feature could be present without the other. Both were disorders of women of the child-bearing years of life. SURT, like LP, was an indicator of chronic fibrotic sarcoidosis, developing insidiously and progressing indolently over the years. It was complicated by ulceration, septal perforation, and LP. Three patients had nasal septal perforations, in two instances following submucous resection. This operation is contraindicated in patients with active sarcoidosis, particularly when granulomas are found on nasal biopsy. The Kveim-Siltzbach skin test was positive in all patients with SURT, making it invaluable in the differential diagnosis of granuloma of the nasal cavity.

Bone sarcoidosis.

Sarcoidosis of the bone has been observed in 24 patients for up to 43 yr. It was always present in hands and/or feet and also elsewhere in 4 other patients. Soft-tissue swelling preceded the radiological abnormality for up to 4 yr in 9 patients accompanied it once, followed it once, and was absent in 13 (55%). Bone involvement was usually an incidental finding when sarcoidosis presented elsewhere, including lupus pernio (50%) and other chronic skin lesions (41%), lungs (75%), eyes (51%), lymph nodes (21%), liver (17%), spleen (13%), parotids (13%), facial palsy in 2 patients and erythema nodosum in 1. Pulmonary infiltration, with or without lymphadenopathy, was evident in three-fifths of the patients and hilar adenopathy alone in one-fifth. Sarcoid tissue was observed in the bone, but not in the surrounding soft-tissue swelling of 2 amputated fingers. There were 3 types of bone lesions: a) Lytic in 20 patients; minute 1-mm cortical defects or larger, usually rounded defects that on healing become corticated leading to punched-out "cysts". b) Permeative in 8 patients; these showed progressive cortical tunnelling with remodelling of trabecular and cortical architecture. c) Destructive in 3 patients, with secondary joint-surface involvement and in one a periosteal reaction.