PMC4724758_01
(A) Detail of patient's photograph en face showing multiple pigmentary disorders of her light skin. The lips also show pigmentary disorders but the lip contour is not affected. The disease cannot be estimated from the external evaluation. (B) Oral inspection reveals clear mucous drooling after light compression of the upper lip (C) Close-up view of the oral aspect shown in (B) reveals the extended orifice of the labial minor salivary gland. (D) Expulsion of clear saliva after slight pressure on the lower lip from minor salivary glands of the lower lip vermilion during oral investigation for recurrent cheilitis glandularis. (E) After incision of the lower lip, berry-like enlarged minor salivary glands come to light.
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F1
External examination showed no alteration of the lips (Figure 1A (Fig. 1)). Enlarged ostia were visible that secreted a clear tenacious mucus (Figures 1B (Fig. 1) and 1C (Fig. 1)). After incision, berry-like tumours were visible that lay beneath the mucous membrane and did not infiltrate the lip muscles (Figure 1E (Fig. 1)). The physical appearance of the lesion was always the same throughout the observation period of almost six years (Figure 1D (Fig. 1)).
(A) Minor salivary gland with interstitial lymphoid infiltrates penetrating atrophic acinar residues; excretory ducts (left) showing moderate ectasia and mucous retention. (B) Closer look at the duct system showing epithelial hyperplasia and apocrine metaplasia (A and B: haematoxylin-eosin staining). (C) Low magnification of the ectatic ductal tree and the irregular atrophia of acinar tissues with some fibrosis and lymphoid infiltrates in between. (D) Closer look at the lymphoid infiltrates penetrating the hyperplastic ductal epithelium (C and D: periodic acid-Schiff reaction).
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F2
There were neither eosinophil granulocytes, granuloma nor cellular atypia (Figure 2 (Fig. 2)).
Immunohistochemical decoration of the interstitial lymphoid infiltrate (A: CD45, B: CD138). IgG4 staining showing a scattered pattern in (C), and very focal clustering in (D).
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F3
Stainings were done for CD45 (Figure 3A (Fig. 3)), CD138 (Figure 3B (Fig. 3)), and IgG4 (Figures 3C (Fig. 3) and 3D (Fig. 3)), and revealed lymphoplasmacellular infiltrates (see Figure 3A (Fig. 3)), mainly located in broadened interstitial spaces with some exocytosis in the ductal and acinar compartments. Immunoglobulin G subclass 4 (IgG4)-positive cells were mostly scattered, yet also showed some clustering (Figure 3B (Fig. 3)).