Orofacial Granulomatosis Associated with Crohn's Disease

Orofacial granulomatosis (OFG) is a term used to describe swelling of the orofacial area, mainly in the lips, secondary to an underlying granulomatous inflammatory process. OFG has been reported in association with systemic conditions such as sarcoidosis and Crohn''s disease (CD). OFG may precede gastrointestinal disease, such as CD, by several years and may be the only obvious focus of the disease. Herein, we report a patient with OFG and non-symptomatic ulcerations of the ileocecal valve. The patient received intralesional triamcinolone injections every 2 weeks. After 6 weeks, all oral lesions showed marked improvement. The favourable treatment response of this patient suggests that intralesional triamcinolone can be used as a treatment option for patients with CD that have oral lesions. In addition, patients presenting with OFG should be carefully evaluated for gastrointestinal signs and symptoms.     

Orofacial granulomatosis: three case reports illustrating the spectrum of disease and overlap with Crohn's disease

We report three cases of orofacial granulomatosis (OFG) to illustrate the spectrum of this disease, and to discuss the appropriate management steps, consider its overlap with Crohn's disease (CD) and raise its awareness among paediatric dermatologists. The term ‘orofacial granulomatosis’ was first used in 1985 to describe granulomas in the orofacial region in the absence of any recognized systemic condition. It is uncommon but becoming increasingly recognized in children. The clinical features of the disease may vary greatly, and often present with subtle changes that can be missed. There is a debate about whether OFG exists as a separate condition or whether it is an oral feature of CD, as some patients go on to develop CD several years later. Identifying those most at risk is important, as ongoing investigations may be necessary. The three cases presented in this series illustrate the range of disease signs and symptoms, and the investigations required.     

Cutaneous manifestations of metastatic Crohn's disease

Metastatic Crohn's disease is a rare cutaneous complication of primary Crohn's disease. It is a granulomatous inflammatory process, similar to the pathogenic mechanism of Crohn's disease, that occurs in sites discontiguous from the gastrointestinal tract. Metastatic Crohn's disease can precede the development of Crohn's disease by months to years, and children are more likely to present with metastatic Crohn's disease in the absence of gastrointestinal symptoms. Given that approximately 30% of individuals with Crohn's disease present in childhood, early recognition of extraintestinal manifestations of Crohn's disease such as metastatic Crohn's disease can aid in timely diagnosis and management of bowel disease. We present data from two pediatric cases of metastatic Crohn's disease recently seen at our institution in addition to the 61 reported cases of pediatric metastatic Crohn's disease in the literature. This review article will focus on the epidemiology, pathogenesis, clinical features, and histology of and treatment options for pediatric metastatic Crohn's disease.     

Cutaneous Crohn's disease treated with infliximab and 4 years of follow up

Metastatic Crohn's disease (MCD) is a rare, non‐contiguous cutaneous manifestation of Crohn's disease. To date, there have been only four reports in the literature of an effective treatment of this condition with infliximab and there are no long‐term follow‐up studies on adult MCD patients treated with infliximab. We present a case of MCD treated with infliximab with 4.5 years of follow up.     

Granulomatous cheilitis and Crohn''s disease

A case of granulomatous cheilitis is reported in whom investigations showed intestinal involvement compatible with Crohn's disease, although the patient had no gastrointestinal symptoms. Granulomatous cheilitis (GC) is an uncommon disorder that is characterized by persistent swelling of the lips and a granulomatous histology. Since the report of a case of GC that occurred several years before intestinal lesions appeared,¹ GC has been recognized to be an early manifestation of Crohn's disease. However, to our knowledge only four cases have been reported that initially presented with GC and then developed Crohn's disease.^1–4 Whether GC represents an early manifestation of Crohn's disease or a distinct clinico-pathological entity remains controversial.⁵ We describe a patient with GC who had intestinal lesions compatible with the diagnosis of Crohn's disease, although she had no gastrointestinal symptoms.     

Childhood and adolescent orofacial granulomatosis is strongly associated with Crohn's disease and responds to intralesional corticosteroids

We present seven cases of orofacial granulomatosis occurring in paediatric patients aged 6–16 years. All patients were investigated for Crohn's disease and a strong association was found. All patients were treated with intralesional corticosteroid injections with excellent clinical responses. We review the literature and discuss the epidemiological association between childhood orofacial granulomatosis and Crohn's disease, as well as various treatment options, and propose a treatment protocol that was efficacious and well tolerated in all our patients.     

Further evidence for an association between psoriasis, Crohn's disease and ulcerative colitis

To test the hypothesis that psoriasis is associated with Crohn's disease and ulcerative colitis, 204 patients with inflammatory bowel disease (116 with Crohn's disease and 88 with ulcerative colitis) and 204 age and sex matched controls were interviewed and examined. The prevalence of psoriasis in Crohn's disease (11.2%) and in ulcerative colitis (5.7%), was significantly greater than in the control group (1.5%). The prevalence of psoriasis in first degree relatives of patients with inflammatory bowel disease was also increased. It is suggested that there is a relationship between psoriasis, ankylosing spondylitis, sacroiliitis, peripheral arthropathy and inflammatory bowel disease, which may be explained by common genetic factors.     

Azathioprine‐induced Sweet's syndrome and published work review

Hypersensitivity to azathioprine can manifest with a wide clinical spectrum. Azathioprine‐induced Sweet's syndrome (SS) is rare and usually overlooked because it can mimic disease exacerbation and sepsis. This study aims to characterize the clinical findings of azathioprine‐induced SS. A retrospective analysis of the records of three patients diagnosed with azathioprine‐induced SS and a review of the relevant English‐language published work was performed. Twelve (71%) of the 17 patients were male, ranging 9–89 years in age (mean, 47.2). The time of onset after starting azathioprine was 5–28 days (mean, 13.3). The most common associated disease was inflammatory bowel disease including ulcerative colitis and Crohn's disease (76%). The clinical features typically consisted of fever and classic rash of SS with pustules and vesicles. The lesions occurred most commonly on the face and trunk. Systemic involvement was rare and no hypotension or shock was reported as seen in azathioprine hypersensitivity syndrome. Thiopurine methyltransferase activity is not predictive of this type of adverse effect. Most patients dramatically responded to systemic corticosteroids. Azathioprine‐induced SS may be underdiagnosed because it can be easily misinterpreted as inflammatory bowel disease‐associated skin eruption. Patients with inflammatory bowel disease may be at higher risk of this condition. Early recognition and drug withdrawal can decrease morbidity of the patients.     

A Case of Phrynoderma in a Patient with Crohn's Disease

Phrynoderma is a type of follicular hyperkeratosis associated with nutritional deficiencies. It is rarely seen in developed countries, although cases have been reported in patients with severe malnutrition or malabsorption secondary to various causes. This report describes a 19‐year‐old patient with poorly controlled Crohn's disease and malnutrition who developed the characteristic hyperkeratotic papules and plaques on his trunk and extremities in the setting of low serum vitamin A levels. To our knowledge, there are no reports of phrynoderma associated with Crohn's disease. It is likely that our patient's low vitamin A level and subsequent phrynoderma was the result of increased Crohn's disease activity and malnutrition.     

Vulval Crohn's disease: difficulties in diagnosis

Four patients with chronic vulval inflammation are described. The histological features of non-caseating granulomata and multinucleated giant cells arc compatible with Crohn's disease, but only two patients had proven gastrointestinal involvement. The clinical and histological characteristics of Crohn's disease and other granulomatous inflammations of the vulva arc discussed and the literature is reviewed.     

CROHN''S DISEASE OF THE MOUTH

A patient with histologically proven Crohn's disease of the ileum and caecum presented with unusual ulceration of the mouth 3 years after subtotal colectomy. Worsening of bowel symptoms was associated with exacerbation of oral ulceration on several occasions. The areas of ulceration in the mouth were in relation to a sarcoid reaction typical of Crohn's disease.     

METASTATIC CROHN'S DISEASE OF EXTERNAL GENITALIA

Metastatic Crohn''s disease is an uncommon extraintestinal manifestation of Crohn''s disease. Its hallmark features include the presence of cutaneous noncaseating granulomas that are noncontiguous with the gastrointestinal tract or fistula. We report a rare case of metastatic Crohn''s disease involving the external genitalia in a 14-year-old girl. Diagnosis was based on skin biopsy. Patient had complete recovery on treatment with oral and topical steroids along with azathioprine.     

Disseminated metastatic cutaneous Crohn's disease

Metastatic cutaneous Crohn's disease, in which non-caseating granulomatous infiltration of the skin occurs at sites separated from the gastrointestinal tract by normal tissue, is the least common dermatological manifestation of Crohn's disease. We report the case of an 18-year-old man who developed very widespread metastatic cutaneous Crohn's disease 2 years after he had first developed mild oral and anal lesions. His gastrointestinal symptoms had been satisfactorily controlled for 18 months with sulphasalazine alone prior to his developing skin lesions. The cutaneous lesions responded rapidly to oral prednisolone which was tailed off over 5 months and then withdrawn. He relapsed 6 months later and now requires a low dose of oral prednisolone to control the skin lesions. The only complication of this therapy has been exacerbation of mild acne vulgaris.     

Vulvar swelling as the first presentation of Crohn's disease in children—A report of three cases

Vulvar swelling is a rare manifestation of metastatic Crohn's disease in children. It often predates gastrointestinal symptoms and can be the first presentation of pediatric Crohn's disease. We report three cases of pediatric Crohn's disease presenting with vulvar swelling. We discuss this rare presentation and its treatment and highlight the importance of recognizing it so that prompt investigation and appropriate management can be initiated.     

Melkersson-Rosenthal Syndrome with Genitalia Involved in a 12-Year-Old Boy

Melkersson–Rosenthal syndrome (MRS) is an uncommon granulomatous disease characterized by the triad of relapsing facial paralysis, orofacial swelling, and fissured tongue. Genital swelling in MRS is rarely reported. We presented the first case of complete MRS with genital swelling in a child. Biopsy examinations of both the child''s lower lip and penis showed noncaseating granuloma and intralymphatic granuloma infiltration. No symptoms or signs of other systemic disease (Crohn''s disease or sarcoidosis) were observed after 2 years of follow-up. Genetic screening for CARD15/NOD2 in this patient showed negative, which further confirmed the diagnosis of MRS. Eleven other cases of suspected complete or incomplete MRS with genitalia involved were reviewed. Our case emphasizes the specific clinical feature of MRS with genitalia involved, which was genetically different from Crohn''s disease and could be an independent entity. Lymphatic obstruction is responsible for localized edema in MRS.     

Red blood cell distribution width is increased in patients with psoriasis vulgaris: A retrospective study on 261 patients

Circulating inflammatory cytokines and markers are increased in patients with psoriasis. Recent studies have shown that a higher red blood cell distribution width (RDW) is associated with disease activity in various disorders. Our objective was to investigate whether RDW is increased in psoriasis patients, and to evaluate its possible association with disease severity. We conducted a retrospective study of psoriasis patients seen in a university hospital in South Korea. Information about demographics, hematological parameters and disease severity were collected. Statistical analysis was performed using Student's t‐test, multivariable logistic regression, Fisher's exact test and Spearman's rank correlation coefficient analysis as appropriate. A total of 261 psoriasis patients and 102 healthy controls were included in our study. The mean RDW value was significantly increased in psoriasis patients compared with healthy control (P = 0.037). Compared with mild psoriasis patients (Psoriasis Area and Severity Index [PASI], <7), moderate to severe patients (PASI, ≥7) showed significantly higher RDW values (P = 0.044). However, RDW did not show significant correlation with PASI (P = 0.358). When patients were divided into two groups according to their RDW value (<14.6% and ≥14.6%), the mean value of PASI was not significantly different (P = 0.219). Patients with psoriasis showed increased RDW values compared with healthy controls. It was also higher in the moderate and severe disease group than the mild group. Though this is only a pilot study, it is possible that RDW value can reflect the inflammatory status of psoriasis patients.     

Granulomatous cheilitis (Miescher)

A case of granulomatous cheilitis (Miescher) is reported. No aetiological factors could be determined and investigation failed to demonstrate evidence of sarcoidosis or of tuberculosis. The histology was not typical of these tatter conditions, but was consistent with that of Crohn's disease although there was no systemic evidence for this disorder. The occurrence of labial Crohn's disease preceding other manifestations has only recently been described (Carr, 1974). Careful assessment of this case and review of the literature has led us to the conclusion that granulomatous cheilitis is a distinct entity, although it can be difficult to distinguish from labial Crohn's disease.     

Orofacial granulomatosis and erythema multiforme in an adolescent with Crohn's disease

The cutaneous manifestations of Crohn's disease are myriad. A 15‐year‐old girl presented with recurrent lip swelling and eventual development of diarrhea and targetoid macules on the palms, feet, and back. She was finally diagnosed with Crohn's disease in the setting of a clinical presentation and histopathology consistent with orofacial granulomatosis and erythema multiforme. We review the literature and summarize reported occurrences of these cutaneous diseases in children with Crohn's disease.     

Metastatic Crohn's disease of the umbilicus

We report a case of metastatic Crohn's disease of the umbilicus which responded to topical corticosteroid treatment, Crohn's disease is a granulomatous disease of the bowel which may a fleet other organs. The skin is commonly involved¹, and the cutaneous manifestations may be non-specific, e.g. pyoderma gangrenosum or erythema nodosum² or specific with epilhelioid granulomas and multinucleated giant cells in the skin. Specific skin lesions may be contiguous with the bowel at sites including the perineum, mouth and adjacent to fistulae, or separated from it by normal skin from the bowel, a rare condition which has been termed ‘metastatic’ Crohn's disease.³