It usually involves the lip vermilion and the vermilion border, but the surrounding skin and the oral mucosa may also be affected. The underlying cause of the condition is not completely understood. Cheilitis granulomatosa or granulomatous cheilitis is a chronic swelling of the lip due to granulomatous inflammation. Cheilitis granulomatosa of miescher is characterized by swelling restricted to the lips. Granulomatous cheilitis is a rare disorder of the lips and orofacial mucosa that was first described by meischer 1 in 1945 as persistent or recurrent orofacial swelling secondary to lymphatic obstruction by granulomatous proliferation. This inflammation may include the perioral skin the skin around the mouth, the vermilion border, or the labial mucosa. Cheilitis granulomatosa of mieschermelkerssonrosenthal, cheilitis granulomatosis of mieschermelkersson rosenthal, macrocheilia, facial palsy and edema syndrome, melkerson rosenthal syndrome, melkersonrosenthal syndrome, melkersson rosenthal syndrome, melkersson syndrome, melkerssonrosenthal syndrome, melkerssons syndrome, rosenthal melkerson syndrome, rosenthal melkersson.
It is part of the spectrum of orofacial granulomatosis including mieschermelkerssonrosenthal syndrome granulomatous cheilitis, facial palsy and fissuring of the tongue. Swabs for infections including candida and a biopsy may be required. Cheilitis venenata article about cheilitis venenata by. Granulomatous cheilitis refers to an uncommon condition in which there is lumpy swelling of the lips. The final histopathologic diagnosis was cheilitis granulomatosa. It is a monosymptomatic form or an incomplete variant of melkerssonrosenthal syndrome. The enlargement does not cause any pain, but the best treatment and the prognosis are uncertain. Occasionally, similar edematous swellings involve other areas, including the periocular region and genitalia. It is a rare inflammatory disorder first described by miescher in 1945. Cheilitis is an acute or chronic inflammation of the lips. Surgical treatment of persistent macrocheilia in patients with. The outbreaks of swelling may also be accompanied by headaches, fever or vision problems.
Cheilitis granulomatosa miescher granulomatous macrocheilitis with trisomy 21 bhushan sevakram madke mbbs md, deepti ghia mbbs, reshma gadkari mbbs, chitra nayak mbbs md dermatology online journal 18 6. Histologically, lymphedema, perivascular lymphocytic infiltration, and to a varying degree nonnecrotizing granulomas are seen. Swelling of the lips, plicated tongue lingua plicata, and recurring facial paralysis characterize the melkerssonrosenthal syndrome mrs. Successful treatment of granulomatous cheilitis with. Cheilitis is usually diagnosed by taking a careful history and by clinical examination. It is also known as cheilitis granulomatosa or miescher cheilitis. It often has been described as a monosymptomatic form of melkerssonrosenthal syndrome mrs. Granulomatous cheilitis is a rare condition characterised by persistent, diffuse, softtofirm swelling of one or both lips. The mechanism of the enlargement is granulomatous inflammation. Key points uncommon skin condition affecting the lips can be brought on by various conditions, such as allergies, crohn disease and sarcoidosis consists of a lumpyfeeling swelling of the lips granulomatous cheilitis is an uncommon skin condition which affects the.
Today, because of a great similarity in clinical and microscopic features, most authors consider the disease as part of the melkerssonrosenthal syndrome. It is characterized by persistent, diffuse, nontender, softtofirm swelling of one or both lips. This means that cheilitis granulomatosa, or a subtype of cheilitis granulomatosa, affects less than 200,000 people in the us population. Cheilitis granulomatosa miescher melkerssonrosenthal syndrome. Miescher cheilitis definition of miescher cheilitis by. Cheilitis granulomatosa of miescher is a rare, painless and. Cg may precede the presentation of crohns disease after longterm followup. The study of cheilitis granulomatosa has been mentioned in research publications which can be found using our bioinformatics tool below. Cheilitis is the medical term used to describe chapped or cracked lips. It is thought to be a subset of orofacial granulomatosa and is frequently used in the literature to describe the monosymptomatic presentation of miescher cheilitis. The results of patch tests, done with both regular and dental series to exclude an allergic cause, were negative. The aim of this presentation is to describe a rare case of. Cheilitis granulomatosa cg is a rare chronic disease characterized by a recurrent firm swelling of one or both lips, and, histologically, by a. It is one manifestation of orofacial granulomatosis ofg, which is a clinical entity describing facial and oral swelling in the setting of noncaseating granulomatous inflammation and in the absence of systemic disease such as crohns disease and sarcoidosis.
The term cheilitis refers to an inflammatory condition of the lip and. Melkerssonrosenthal syndrome mrs, also known as cheilitis granulomatosa or mieschermelkerssonrosenthal syndrome, is a rare condition of unknown etiology characterized by. Melkerssonrosenthal syndrome in a patient with hashimoto. Cheilitis granulomatosa is a monosymptomatic variant of this condition. Disease bioinformatics research of cheilitis granulomatosa has been linked to melkerssonrosenthal syndrome, edema, granuloma, crohn disease, fissured tongue. A potential salivary biomarker for orofacial granulomatosis and crohns disease. Granulomatous cheilitis has been recognized as an early manifestation of crohns disease. Cheilitis granulomatosa miescher melkersson rosenthal. Miescher cheilitis is the term used when the granulomatous changes are confined to the lip.
Granulomatous cheilitis is a rare, idiopathic, inflammatory disorder which usually affects young adults. Melkerssonrosenthal syndrome cheilitis granulomatosa. Satisfactory results have been obtained combining medical therapy and surgical approach. Successful treatment of two recalcitrant cases with combination drug therapy. Cheilitis granulomatosa cg is a rare idiopathic condition with painless lip swelling, characterized by nonnecrotizing granulomatous inflammation in the absence of other identifiable causes such as crohns disease, sarcoidosis, foreign body reaction, or infection. It may follow, coincide with or precede the onset of crohns disease. Rare causes are infections, cancers and genetic disorders. The latest version of icd10 is updated each year on october 1. Cheilitis is caused by multiple reasons such as change in weather, constant licking of lips, side effects of certain medications, nutrient deficiency etc. Granulomatous cheilitis is a chronic swelling of the lip due to granulomatous inflammation.
Cheilitis actinica definition of cheilitis actinica by. Cheilitis granulomatosa cg is a cosmetically disturbing and persistent idiopathic lip swelling. Cheilitis granulomatosa associated with melkerssonrosenthal syndrome. Cheilitis granulomatosa is a disorder that periodically appears and that most often involves the swelling of the lips, but may also include swelling of other areas of the head.
Pdf cheilitis granulomatosa associated with lupus erythematosus. Therefore, a final diagnosis of idiopathic ofg was made. Cheilitis granulomatosa is listed as a rare disease by the office of rare diseases ord of the national institutes of health nih. However, factitial injury to the lower lip vermilion border and mucosa due to chronic lip biting, lip drying, or habitual lip licking, combined with suninduced lip damage, are extremely commonplace.
Granulomatous cheilitis definition of granulomatous. It is characterised by lips that are dry, cracked, and scaly with one or more fissures. We report two cases of granulomatous cheilitis treated with a. The condition is characterized by reddening, edema, burning, itching, and formation of blisters, ulcers, cracks, scales, and serousbloody crusts. Granulomatous cheilitis is considered a monosymptomatic form of melkerssonrosenthal syndrome by some clinicians. Cheilitis granulomatosa cg is a chronic swelling of the lip due to granulomatous inflammation. Cheilitis granulomatosa is usually seen in orofacial granulomatosis ofg as an episodic nontender swelling and enlargement of one or both lips. Cheilitis granulomatosa associated with melkersson.
Melkerssonrosenthal syndrome mrs is a rare syndrome that is characterized by a triad of facial paralysis, chronic edema of the lip, and a fissured tongue. Abstract background cheilitis granulomatosa, often regarded as a subtype of orofacial granulomatosis, is characterized by recurrent or. However, soothing emollient lip balms and sunscreens are usually recommended. Cheilitis inflammation of the skin, red border, and mucosa of the lips. Gada md walter reed national military medical center, 8901 rockville pike, bethesda, md 208895600, u. Oral manifestations of crohns disease, an analysis of 79 cases. The results of the other investigations were also negative. Cheilitis granulomatosa is clinically characterized by painless soft to firm swelling of one or both lips. Cheilitis granulomatosa an uncommon clinicopathological entity.
Cheilitis glandularis is an uncommon condition in the united states. Granulomatous cheilitis associated with exacerbations of. The etiology of this disease is unclear, but the condition has been linked to an abnormal immune reaction. Pdf we present the rare case of a 47yearold patient, suffering from cheilitis granulomatosa and lupus erythematosus discoid. Orofacial granulomatosis ofg is a condition characterized by persistent enlargement of the soft tissues of the mouth, lips and the area around the mouth on the face. Unsuccessful treatment of cheilitis granulomatosa with.
There are many different causes, such as allergy, crohn disease, sarcoidosis and orofacial granulomatosis. Cheilitis granulomatosa melkerssonrosenthal syndrome. Cheilitis granulomatosa cg is a rare granulomatous disorder of unknown origin, initially described by german. The onset is usually in childhood and relapses are common. Meischer syndrome cheilitis granulomatosa a chronic swelling of the lips due to granulomatous inflammation, which some. I medical doctor, ent specialist, adjunct professor of the ophthalmology, otorhinolaryngology and speech therapy.
Dermis cheilitis granulomatosa of miescher information. Cheilitis granulomatosa is a rare disease characterised by the recurrent labial swelling of one or both lips with the possibility of the condition to remain on a permanent basis. The skin and the vermilion border are more commonly involved, as the mucosa is less affected by inflammatory and allergic reactions it is a general term, and there are many recognized types and different causes. An overview on oral manifestations of gastrointestinal.
Fissured tongue and recurrent cheilitis of the upper lip were observed. Crohns disease is a disease involving the whole gastrointestinal tract from the mouth to the anus. Oral lesions are considered to be an important extraintestinal manifestation. Cheilitis granulomatosa or mieshers cheilitis is a rare inflammatory disorder with.
In cheilitis glandularis cases in which a history of chronic sun exposure exists especially if the individual is fair skinned or the everted lip surface is chronically eroded, ulcerated, or crusted, biopsy is strongly recommended to rule out actinic cheilitis or carcinoma. Pdf granulomatous cheilitis is a rare, idiopathic, inflammatory disorder which usually affects young adults. Ofg with the subentities mrs and cheilitis granulomatosa is. Initially mieschers cheilitis granulomatosa was considered as a separate entity. Article pdf available in inflammatory bowel diseases 258. Common symptoms include erythema, dryness, scaling, fissuring, edema, itching, and burning. Cheilitis granulomatosa amy e rose md, marie leger md phd, julie chu md, shane meehan md dermatology online journal 17 10. Cheilitis granulomatosa or granulomatous cheilitis is characterized by persistent idiopathic swelling of the lip due to granulomatous inflammation. Criticisms and controversies in the diagnosis of cheilitis mdpi. It is a commonly encountered condition and heals within a few days. Source national institutes of health nih cheilitis granulomatosa as a. In spite of the best treatment, recurrence of the disease is very common. One variant, cheilitis granulomatosa of miescher cg, is generally. An idiopathic syndrome characterized by one or more of the following.