Oral Ulcer—A Disabling Manifestation in a Patient With Lipoid Proteinosis

Abstract

Eight of 18 patients (44%) were morbidly obese, of whom 6 had undergone weight loss surgery (all by Roux-en-Y gastric bypass). Additional frequent comorbidities were alcoholism (8 [44%]), hepatic cirrhosis (8 [44%]), hepatitis C virus infection (5 [28%]), or a large, chronic, nonhealing postsurgical wound or ulcer (4 [22%]).Results of skin biopsies demonstrated the classic epidermal pallor in 7 of 18 patients (39%), whereas the remaining 11 lacked this finding, demonstrating a predominantly spongiotic, psoriasiform, or atrophic epidermal pattern with overlying confluent parakeratosis and intracorneal splitting. Coincidence of zinc, albumin, and/or niacin deficiency and hepatitis C virus infection was common, with 13 patients (72%) harboring at least 2 abnormalities and 5 (28%) with at least 3. Combined deficiency in zinc and protein was most common (11 of 18 [61%]). Three patients (17%) were found to have concomitant thiamine deficiency. Follow-up data were available on all patients with mean (SD) follow-up of 16.8 (21.5) months and median of 7 months (range, 1-71 months). All-cause mortality was 72% (13 patients), with an estimated survival time of 1 to 54 months (mean, 14 months; median, 5 months; 95% CI, 4.7-23.8 months). Detailed review of the final clinical course preceding 7 of 13 deaths unveiled a common picture characterized by an irreversible, nonseptic, distributive, cardiovascular shock with anasarca and multiple-organ failure. Comparative Kaplan-Meier survival analysis using the log-rank test revealed that hypoalbuminemia of less than 3.4 g/dL (to convert to grams per liter, multiply by 10) was associated with a median survival time of 4.0 months (compared with 54.5 months for albumin level of ≥3.4 g/dL [P = .03]). Being 50 years or younger at time of dermatologic diagnosis was also associated with lower median survival of 2 months (compared with 34 months for those aged >50 years [P = .03]) (Figure).

A Sporadic Family of Lipoid Proteinosis with Novel ECM1 Gene Mutations

“…The second stage is characterized by the gradual emergence of verrucous lesions, on the extensor surfaces of the elbows, hands, and knees, as well as diffuse skin thickening, hyperkeratosis and nodules. 8 Classical skin signs of LP consist of multiple beaded papules along the eyelid margins and inner canthus. 9 Furthermore, various systemic manifestations have been described such as seizures, psychiatric disorders, memory loss, gingival hyperplasia, stiff tongue and slurred speech.…”

The role of systemic steroids in oral ulcers associated with lipoid proteinosis

“…Although the exact etiology of oral ulceration in patients with LP is unclear, the involvement of salivary glands, xerostomia, and poor oral hygiene are considered to be triggering factors. 2 High levels of ASO also support the role of infection in the etiology of oral ulcers. To date, only a few cases of LP accompanied by oral ulceration have been reported.…”