Granulomatous cheilitis of Miescher: a rare entity.

BACKGROUND: Granulomatous cheilitis (GC) is a rare entity of unknown etiology. It is a chronic inflammatory disorder with a predilection for young females. It is characterized by asymptomatic unrelenting swelling of lips. It is a monosymptomatic form or an incomplete variant of Melkersson-Rosenthal syndrome (a triad of recurrent orofacial swelling, relapsing facial paralysis, and fissuring of the tongue). CASE PRESENTATION: We herewith report a case of a 27-year-old female, presenting with persistent upper lip swelling, for 3 months. Biopsy from the lesion revealed features of granulomatous cheilitis. CONCLUSION: GC should be considered in the differential diagnosis of unrelenting swelling in the lip. Spontaneous remission is rare, and recurrences are common. Corticosteroids used for treatment provide temporary improvement.

Granulomatous Cheilitis Preceding Gastrointestinal Manifestations of Crohn's Disease.

ABSTRACT: We describe a 20-year-old male with no significant medical history who presented with a 1-month history of painless upper and lower lip edema initially treated with antibiotics for suspected cellulitis before arriving to the clinic. After a failed response to that treatment, a lip biopsy was eventually performed and consistent with a diagnosis of granulomatous cheilitis. In addition to oral and topical corticosteroids and tacrolimus, the patient adopted the cinnamon- and benzoate-free diet with some improvement of his lip swelling. Persistent mild tachycardia led to a cardiology referral for further evaluation and sarcoidosis workup. A gastroenterology consult was placed to correlate his presentation with Crohn's disease. The cardiology workup was noncontributory, and the patient was ultimately diagnosed with Crohn's disease after evaluation with laboratory studies and colonoscopy. This case highlights the need to evaluate for Crohn's disease in patients who present with granulomatous cheilitis, even in the absence of gastrointestinal symptoms, and the potential benefit of incorporating a cinnamon- and benzoate-free diet in treatment.

Pathological and Immunohistochemical Characteristics of Granuloma and Lymphatics in Cheilitis Granulomatosa.

ABSTRACT: Cheilitis granulomatosa (CG) is an idiopathic, rare, and chronic granulomatous disorder involving the lips. We characterized the pathological and immunohistopathological findings of these granulomas and their relationship with the lymphatic vessels. Pathologically confirmed cases of primary CG from 2001 to 2016 were collected. Cases of inflammatory cheilitis without the presence of granuloma were included in the control group. Demographic data, clinical presentation, response to therapy, and pathological differences were compared. Periodic acid-Schiff and acid-fast stains excluded patients having infections. CD68, CD163, and D2-40 stains demonstrated features of granuloma, macrophage polarization, and the relationship between granuloma and lymphatic vessels. Thirteen patients diagnosed with CG were enrolled. Thirteen people were enrolled in the control group. The granulomas were either mononuclear or sarcoidal. They were predominantly positive for CD68 but negative for CD163. Perilymphatic granulomas were found in all patients. Intralymphatic histiocytosis and lymphatic dilatation were more commonly observed in patients diagnosed with CG than those in controls (54% vs. 15%, P = 0.03 and 92% vs. 23%, P < 0.01). TH1 immune response due to CD68+ M1 macrophages results in CG. Perilymphatic aggregation of macrophages and intralymphatic histiocytosis were important pathological clues for diagnosis.

An uncommon case of lip swelling: Granulomatous cheilitis associated with Crohn's disease.

Unbeknownst to most otolaryngologists, there is quite a range of oral manifestations which commonly manifest in the context of inflammatory bowel disease. As providers who will encounter such patients in consultation it is beneficial to be aware of that association. Lip swelling (granulomatous cheilitis) is just one such presentation, which is often otherwise mistaken for angioneurotic edema and can lead to unwarranted testing and misdirected treatment. We present such a case to highlight the educational value of this patient encounter.

The histopathological spectrum of Melkersson-Rosenthal syndrome: Analysis of 47 cases.

BACKGROUND: Melkersson-Rosenthal syndrome (MRS) is a rare disease characterized by the triad of recurrent orofacial edema, relapsing facial paralysis and plicated tongue. Histopathological features of MRS have not been extensively analyzed. METHODS: This study investigated the histopathological aspects of oral lesions from 47 MRS patients. RESULTS: The most common biopsy site was the upper lip, followed by the lower lip, gingiva and palate. The most important findings were ill-defined and well-formed granulomas. Lymphoplasmacytic inflammatory infiltrate was seen in early and late stages of MRS. Edema, fibrosis, vasodilatation and congestion were the most common finding in the lamina propria. Gingival and palate exams also demonstrated granulomatous infiltrates. Regarding the evolution time of the disease, we demonstrated that, in initial phases, there is a lymphoplasmacytic inflammatory infiltrates, followed by a granulomatous infiltrate and, subsequently, fibrosis. CONCLUSION: Histopathological examination of oral lesions is helpful for the diagnosis of MRS; the absence of granulomatous inflammation does not exclude the diagnosis of syndrome. Clinical and histopathological analysis of the rare gingival and palate lesions is important, since all histopathological findings of the disease were detected in these sites.

Periocular intralymphatic histiocytosis or localized Melkersson-Rosenthal syndrome?

We describe three cases of periocular edema with histopathologic features of intralymphatic histiocytosis without extravascular granulomas. All were elderly males with no other significant medical problems. Previous reports of periocular Melkersson-Rosenthal syndrome are identical clinically, and some reports show illustrations of intralymphatic histiocytosis histopathologically, in addition to other features typical of the syndrome. Given the lack of associated diseases or other features of the Melkersson-Rosenthal triad, some of these cases may be better defined as periocular intralymphatic histiocytosis.

Melkersson-Rosenthal syndrome with isolated unilateral eyelid edema: an immunopathologic study.

Lymphedema is caused by defective drainage of the lymphatic system. In Melkersson-Rosenthal syndrome, involvement is predominantly of the lumens with blockage of lymphatic channels by histiocytic-epithelioid cell clusters accompanied by dermal granulomas and lymphocytes. It is a localized, painless, nonitching, and nonpitting form of lymphedema. Besides the eyelids, the disease can cause lip edema, facial palsy, and/or fissured tongue. It is rare and has received little attention in the ophthalmic literature, either in its complete triadic form, or more frequently, in its monosymptomatic forms. Pathogenesis is not well understood, and there is no effective therapy. The authors describe a case of Melkesson-Rosenthal syndrome in a 45-year-old Hispanic man with isolated unilateral upper eyelid edema. Histopathological and immunohistochemical evaluations of an eyelid biopsy specimen revealed intravascular and extravascular clusters of histiocytic-epithelioid cells that were CD68/163-positive. Variable numbers of mostly T-lymphocytes were found in the epidermis, dermis, and orbicularis muscle and by virtue of the associated granulomas established the diagnosis of Melkersson-Rosenthal syndrome. CD4 helper and CD8 suppressor T-lymphocytes were equally represented. CD20 B-lymphocytes were exceedingly sparse. Conspicuous CD1a-positive Langerhans' cells were present in the epidermis, sometimes formed subepithelial loose aggregates and were also incorporated in the granulomas. The differential diagnosis includes the far more common condition of acne rosacea. Management of Melkersson-Rosenthal syndrome, and of angioedema in general, is reviewed.

[Cheilitis granulomatosa and Melkersson-Rosenthal syndrome. Intralymphatic histiocytosis as valuable diagnostic indication]. / Cheilitis granulomatosa und Melkersson-Rosenthal-Syndrom. Intralymphatische Histiozytose als wertvoller diagnostischer Hinweis.

Intravascular and/or intralymphatic cell aggregations can occur in malignant tumors as well as in benign reactive conditions, such as chronic inflammatory diseases. This article discusses the valuable histomorphological aspects of intralymphatic histiocytosis (or granuloma) in the differential diagnostics to other entities exemplified by a case report on juvenile cheilitis granulomatosa. In addition other benign entities sometimes presenting with the phenomenon of intravascular histiocytosis (e.g. rheumatoid arthritis, orthopedic metal implants and Rosai-Dorfman disease) are discussed.

Edematous lip in a HIV patient on highly active antiretroviral therapy.

A 59-year-old HIV-positive, hepatitis C positive man on highly active antiretroviral therapy presented with a 2-year history of extreme swelling of the lower lip. Granulomatous cheilitis was diagnosed.

Radiofrequency therapy as an effective treatment for granulomatous cheilitis: A CARE case report.

INTRODUCTION: Granulomatous cheilitis is a rare disorder characterized by recurrent, idiopathic, and painless lip swelling. The diagnosis is proven by histopathological examination. The unknown aetiology and poorly understood underlying mechanism contribute to the difficulty in establishing an effective treatment. This case study proposes the effectiveness of radiofrequency therapy in the management of refractory granulomatous cheilitis. CASE SUMMARY: A 68-year-old patient presented with hypertrophy and swelling of the lower lip, and a biopsy revealed actinic cheilitis. The patient underwent lip shaving and an advancement mucosal flap, and definitive histologic examination confirmed the diagnosis of granulomatous cheilitis. No other signs of orofacial granulomatosis were observed, and the complementary aetiological study was negative for systemic disease. The lip swelling reappeared and persisted, which interfered with the quality of life. Hence, radiofrequency therapy was performed in the submucosal and subdermal layers of the lip, resulting in significant aesthetic and functional improvement and no further relapses after five years. DISCUSSION: The management of granulomatous cheilitis is challenging. The current mainstay treatment is corticotherapy or reduction cheiloplasty in severe cases. Radiofrequency has potential as a treatment option in debilitating macrocheilia, presenting worthy long-lasting functional and aesthetical results, with minimal morbidity.

Cheilitis granulomatosa (Miescher granulomatous macrocheilitis) with trisomy 21.

A 30-year-old male with Down syndrome presented to us with diffuse swelling of the lower lip of seven years duration. On examination, there was gross enlargement of his lower-lip with fissuring at places. A histopathological specimen from the lower lip showed non-caseating granulomas. We treated our patient with intralesional triamcinolone acetonide and oral clofazimine. We report this case because of the rare association of cheilitis granulomatosa with Down syndrome.

Granulomatous cheilitis: a report of 6 cases and a review of the literature.

INTRODUCTION: Granulomatous cheilitis (Miescher cheilitis), a condition characterized by recurrent swelling of the lips, is the most common monosymptomatic form of the Melkersson-Rosenthal syndrome. The aim of this study was to study the characteristics of patients diagnosed with granulomatous cheilitis at the dermatology department of our hospital over a period of 17 years. MATERIAL AND METHODS: We performed a descriptive study of patients diagnosed with granulomatous cheilitis at our hospital between January 1993 and January 2010. RESULTS: The condition was diagnosed in 6 patients (4 women and 2 men), with a mean age of 49 years at the time of diagnosis. All the patients had recurrent swelling of the upper lip and 2 also had swelling in other parts of the face. The mean time from the onset of symptoms to the initial visit was approximately 16 months. There were no cases of facial palsy, and just 1 patient had a fissured tongue. None of the patients developed Crohn disease or any other granulomatous disorders during follow-up. CONCLUSIONS: Granulomatous cheilitis is a rare disease. None of the patients in our series had gastrointestinal or neurologic symptoms. Accordingly, we believe that granulomatous cheilitis is an independent orofacial granulomatous disease which most often presents without accompanying signs or symptoms.

Melkersson Rosenthal syndrome: a histopathologic mystery and dermatologic challenge.

Melkersson Rosenthal syndrome (MRS) is rare disease of unknown etiology characterized by orofacial edema, facial nerve palsy and fissured tongue. Microscopically, it shows epithelioid non-caseous granulomas; however, edema and perivascular lymphocytic infiltrates have been described. Two different clinical forms of MRS are presented in this report. In the complete form (Case 1), the main histopathologic finding was a non-necrotizing granulomatous inflammation with 56% of the total number of cells composed of B cells (CD 20(+) ) principally located in the granuloma's center and 33% being T cells predominating in the surrounding area, of which 48% were CD 4(+) and 16% were CD 8(+) lymphocytes. In the monosymptomatic form (Case 2), the inflammatory cells were dispersed into the connective tissue without granulomatous formation. B cells were scanty, and 78% of the cells were CD 45(+) T cells, with 46% and 34%, CD 8(+) and CD 4(+) phenotype, respectively. These cases showed different clinical, histopathological and immunohistochemical forms of MRS, suggesting different host immune responses.