Life‐threatening laryngeal involvement in systemic lupus erythematosus
Authors: Krasowska Dorota , Schwartz Robert A. , Wojecka‐Gieroba Regina , Lecewicz‐Torun Barbara 
Published: April 2004

A 46‐year‐old woman was admitted in 1998 for the evaluation of muscular pain, erythematous to violaceous, slightly atrophic patches on the dorsal interphalangeal joints, suggestive of Gottron's sign, a heliotrope, and oral mucosal dryness. The patient also had a history of photosensitivity and joint pain. On examination, erythematous patches on the dorsal interphalangeal joints were evident, as was painful digital pulp necrosis (Fig. 1). The skin on the elbows, knees, and medial malleoli was normal in appearance. Hoarseness, altered voice pitch, and slight difficulty in swallowing were also noted. There was no proximal muscle weakness evident. Neither periungual telangiectasia nor cuticular thickening was seen. Capillaroscopic examination was also normal. Laboratory findings included a white blood cell count of 3000 and a creatine phosphokinase (CPK) of 987 U/L (normal, up to 195 U/L). Waalery‐Rose test dye test was positive at 1 : 640 (normal, up to 1 : 80), rheumatoid factor latex agglutination test ++, Schirmer test for tear secretion of 5 and 7 mm Eye Right Left (ERL) (normal, 15 mm each eye), antinuclear antibody (ANA) of 1 : 160, and anticytoplasmic antinuclear antibody which is named anti Ro/SSA positive. Histopathologic examination of the deltoid muscle and electromyographic investigation did not confirm a diagnosis of dermatomyositis. Next, the patient underwent diagnostic investigations to exclude malignancy. All of the following were within normal limits: ultrasonography of the abdomen, contrast roentgenogram of the digestive tract, barium enema, rectoromanoscopy, echocardiogram, mammography, lung roentgenogram, sinus X‐ray, and gynecologic examination with cytology.

Erythematous patches on the lateral and palmar finger with digital pulp necrosis

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One year later, she was again hospitalized. On examination, deep, coin‐sized, necrotic ulcers were observed affecting the aural area of the cheek (Fig. 2) and the lateral aspect of the right hand, persisting for about 2 months. In the oral cavity, there were erosions changing into superficial ulcerations. Moreover, ophthalmologic examination revealed bilateral erosive keratitis. Neurologic examination showed isolated paresis of the fifth finger of the right hand. Laboratory data were as follows: Westergreen erythrocyte sedimentation rate (ESR), 80 and 108 mm/h (normal, 0–20 mm/h); white blood cell count (WBC), (3.0–3.7) × 109/L [normal, (4.1–10.9) × 109/L]; rheumatoid factor latex agglutination test, ++; Waalery‐Rose test, 1 : 640; CPK, 987 U/L; minimal erythema dose (MED), 30 s; Schirmer test, 5 and 7 mm ORL. Staphylococcus aureus was cultured from the nose, but not from the throat. Other testing revealed the following: lupus erythematosus (LE) cells, negative; ANA, 1 : 160; Ro/SSA, positive; gamma globulins, 19.6%.

Deep, oval, necrotic ulcers of the aural area of the cheek

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Direct laryngologic examination showed the larynx and vocal cords to be erythematous and covered with telangiectasia. Both vocal folds were thickened along their entire length, with their upper and middle parts covered with a white deposit. Laryngeal mobility was preserved on both sides. The glottis vera was moderately narrowed. Slimy mucoid secretions and vocal fold ulcerations appeared on removing the white deposit from the vocal folds by means of microlaryngoscopy. Decortication was performed to remove the affected membrane from both vocal folds. As the hoarseness was growing worse and difficulty in breathing ensued, the patient was transferred to the Laryngologic Clinic where she underwent emergency surgery.

Histopathologic examination from the larynx showed extensive ulceration covered with a fibronecrotic mass and nonspecific inflammation of the background with the presence of granulocytes (Fig. 3). Polyp fragments were evident. Staining for fungal elements was negative. The patient did not consent to a biopsy from the vasculitis‐like ulcers of the lip and skin. As on the first admission, all internal organ investigations were normal, except for the chest roentgenogram, which this time showed that the inferior part of the left lung had changed, with the costophrenic angle blurred. It returned to normal after treatment with steroids.

Histopathologic specimen from the larynx showing extensive mucosal ulceration and a dense, nonspecific, chronic inflammatory reaction

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In the third year of treatment for systemic lupus erythematosus, her general condition appeared good, but occasional episodes of joint pain recurred. Slightly erythematous patches on the face (particularly the malar region), neck, and the outside of the hands, as well as hoarseness and dryness of the eye mucosa, still persisted. The Schirmer test results remained unchanged, whereas the erosions cleared. Laryngologic examination showed both vocal folds to be smooth, although slightly reddened and thickened. A broad glottis vera was evident. Follow‐up examination results were as follows: ESR, 45 mm/h; WBC, 5500; CPK, 78 U/L; ANA, 1 : 160; Ro/SSA, positive. Methylprednisolone, 4 mg daily and 80 mg in 2‐week pulse dosages, continues to be administered to this patient. An ANA of 1 : 160 and an Ro/SSA positive serology have persisted, together with the malar eruption and oral erosions. The violaceous, slightly atrophic patches on the dorsal interphalangeal joints have regressed.