Early oral presentation of Crohn's disease

Inflammatory changes of the oral mucosa in children can have a variety of infectious and non‐infectious etiologies. We report on a 10‐year‐old boy with progressive cobblestone‐like changes and erosions of oral mucosa over six months, which turned out to be early oral manifestations of Crohn 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.     

Cutaneous Crohn's disease with superimposed psoriasis: A unique case with overlapping histology

Crohn's disease (CD) is an idiopathic, chronic inflammatory disorder of the gastrointestinal tract. We recently encountered a unique case in which a patient with longstanding CD presented with skin lesions with histopathologic features of both psoriasis and granulomatous inflammation suggestive of cutaneous CD. To our knowledge, this has not been described concomitantly in the same patient, in the same lesions. Review of the literature suggests that the intersection of these 2 histopathological reaction patterns may not be pure coincidence. Clinical‐pathologic correlation of this case will be discussed, along with a review of the potential mechanisms of this unique disease presentation.     

Pediatric patients with orofacial granulomatosis likely to subsequently develop intestinal Crohn's disease: Brief Report

Orofacial granulomatosis (OFG) is an uncommon chronic granulomatous condition presenting as perioral inflammation in the absence of systemic disease. There is continued debate regarding whether OFG is a distinct clinical disorder or a manifestation of orofacial Crohn's disease. Our retrospective review identified 7 patients diagnosed with OFG between 2000 and 2018 at a tertiary pediatric hospital. Four of the 7 patients subsequently developed Crohn's disease with a median delay of 3.1 years (range 0.4-6.9 years). This indicates that gastroenterology evaluation with long-term monitoring for intestinal Crohn's disease is warranted.     

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.     

Kikuchi's disease (histiocytic necrotizing lymphadenitis) with cutaneous involvement

We report a case of Kikuchi's disease (histiocytic necrotizing lymphadenitis) in a 22-year-old Japanese man who presented with cervical and axillary lymphadenopathy and who developed edema of lips with desquamation and erosions and transient indurated erythematous papules on the face, neck and upper extremities. A lymph node biopsy specimen showed the typical necrotizing lymphadenitis of Kikuchi's disease. A skin biopsy specimen showed a dense, lymphohistiocytic, superficial, deep perivascular, and interstitial infiltrate, papillary dermal edema, and abundant nuclear debris with a conspicuous absence of neutrophils, paralleling the nodal histology of Kikuchi's disease. CD68 immunohistochemistry showed many plasmacytoid monocytes. Oral corticosteroid (prednisolone 40 mg) therapy improved the skin lesions and other clinical symptoms. Lip involvement by Kikuchi's disease has not been reported, and should be included as one of the possible cutaneous manifestations of Kikuchi'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.     

Diagnosis and management of vulvar ulcers

The diagnosis and management of the following non-infectious vulvar ulcers are reviewed: vulvar aphthae in adult and pediatric patients, aphthae associated with Beh?et's disease, vulvar ulcers resulting from Crohn's disease, and vulvar ulcers associated with human immunodeficiency virus infection. There are many resources providing excellent reviews of infectious ulcers; therefore this topic will not be covered here.     

Manifestation of Crohn's disease in a young woman during the course of treatment for severe form of chronic plaque psoriasis with etanercept

With the growing number of patients with immune‐modulated diseases treated with tumor necrosis factor (TNF) alpha inhibitors, we are more frequently encountering the occurrence of so‐called paradoxical drug reactions. These are basically situations where during the course of the treatment of one disease, the manifestation of another with similar etiopathogenesis occurs, although under normal conditions this newly developed disease responds well to treatment with TNF alpha inhibitors and is indicated for this treatment. Skin reactions are most frequently recorded in the form of induced psoriasis and psoriasiform exanthems. A less common paradoxical reaction is the induction of Crohn's disease, which is most often described in association with the treatment of inflammatory joint diseases with etanercept. We present a case of induction of Crohn's disease during therapy with etanercept, where the primary disease being treated was psoriasis. In the literature, similar cases have only been described sporadically.     

Extramammary Paget's Disease with Distant Skin Metastasis

We report a 68-year-old Japanese man with invasive extramammary Paget's disease (EPD) which started in the left inguinal area and enlarged to include the scrotal skin, penis, and pubic area, resulting in lymph edema of the entire left leg. Histopathological examination of one nodule revealed an intraepidermal and intradermal arrangement of solid tumor cells with characteristic pale cytoplasm. The tumor was found to have disseminated to regional and systemic lymph nodes, lungs, bones, and the left adrenal gland. In addition, the patient had a metastatic cutaneous nodule on the scalp. Histology of the skin metastasis showed that the proliferation of Paget cells was confined to the dermis. This type of metastatic spread of EPD is very rare; only two other apparent cases in Japan have been reported.     

Prevalence of inflammatory bowel disease in Japanese psoriatic patients

Psoriatic patients reportedly have a higher prevalence of inflammatory bowel disease (IBD); however, there have been few research studies of Japanese psoriatic patients. To elucidate the prevalence of IBD in Japanese psoriatic patients, a cross‐sectional study was performed. Information was collected regarding psoriatic patients with current or prior history of Crohn's disease (CD) or ulcerative colitis (UC) who were treated at Fukuoka University Hospital from 2010 to 2018. Among 681 psoriatic patients (449 men and 232 women), eight (1.2%, six men, two women) had UC and two (0.3%, one man, one woman) had CD. Diagnosis of IBD preceded psoriasis in five patients, while diagnosis of psoriasis preceded IBD in two; the remaining patients’ records did not have sufficient information. Seven of 10 UC‐positive patients had mild psoriasis, two had moderate psoriasis and one had severe psoriasis. When UC‐positive psoriatic patients were compared with IBD‐negative psoriatic patients, there were no differences in age at onset of psoriasis, age at first visit or complications (e.g. psoriatic arthritis, hypertension, hyperlipidemia, hyperuricemia and diabetes). However, UC‐positive patients had significantly higher body mass index (BMI) (26.7 vs 23.7; P = 0.021), compared with patients without IBD. The CD/UC ratio in this cohort was 0.25, while the prevalence of IBD was 1.2%; these values were both lower than those in previous reports involving Caucasian patients. Patients with psoriasis and UC may have higher BMI and milder skin symptoms than those with psoriasis alone. These observations must be further confirmed by controlled domestic studies with larger samples.     

The potential role of serology in diagnosing chronic lymphogranuloma venereum (LGV): a case of LGV mimicking Crohn's disease

We present the case of a 26 year old HIV positive homosexual man who was managed for suspected Crohn's disease for over 1 year before lymphogranuloma venereum (LGV) was clinically diagnosed. He had presented with constipation, secondary to acute haemorrhagic proctitis, and subsequently had two chlamydia negative rectal smears, using direct fluorescent antibody (DFA) Chlamydia trachomatis staining. Positive chlamydial serology guided retrospective testing of an early rectal biopsy, which was found to have C trachomatis by polymerase chain reaction (Roche Cobas) and identified as LGV serovar L2 by the Sexually Transmitted Bacteria Reference Laboratory (STBRL), Health Protection Agency (HPA), Colindale, London. Chlamydial serology may have a role in identifying late stage LGV infection. Although no standardised test currently exists, consideration should be given to evaluating the role of chlamydial serology in establishing a diagnosis of LGV.     

Linear IgA Dermatosis associated with ulcerative colitis: complete and sustained remission after total colectomy

Linear IgA dermatosis has been increasingly associated with inflammatory bowel diseases, particularly ulcerative colitis. A 13-year-old male patient with an 11-month history of ulcerative colitis developed vesicles, pustules and erosions on the skin of the face, trunk and buttocks and in the oral mucosa. The work-up revealed a neutrophil-rich sub-epidermal bullous disease and linear deposition of IgA along the dermoepidermal junction, establishing the diagnosis of linear IgA dermatosis. The patient experienced unsatisfactory partial control of skin and intestinal symptoms despite the use of adalimumab, mesalazine, prednisone and dapsone for some months. After total colectomy, he presented complete remission of skin lesions, with no need of medications during two years of follow-up. A review of previously reported cases of the association is provided here and the role of ulcerative colitis in triggering linear IgA dermatosis is discussed.     

Psoriasis,the liver,and the gastrointestinal tract

Psoriasis is a common chronic inflammatory, immune‐mediated skin disease that is frequently associated with comorbidities including psoriatic arthropathy, chronic inflammatory bowel diseases, and cardio‐metabolic disorders. In particular, nonalcoholic fatty liver disease affects about half of patients, Crohn's disease 0.5% and celiac disease 0.2–4.3% of patients with psoriasis. Some shared genetic traits as well as common inflammatory pathways may underlie these associations. The presence of comorbidities has important implications in the global approach to patients. In particular, traditional systemic antipsoriatic agents could negatively affect cardio‐metabolic comorbidities as well as nonalcoholic fatty liver disease and may have important interactions with drugs commonly used by psoriasis patients. Moreover, patients with psoriasis should be encouraged to drastically correct their modifiable cardiovascular and liver risk factors, in particular obesity, alcohol consumption, and smoking habit, because this could positively affect both psoriasis and their life expectance.     

Repeated Paradoxical Aggravation of Preexisting Psoriasis during Infliximab Treatment for Crohn's Disease

As more rheumatologists and dermatologists have begun to use biological agents such as TNF-α blocker, they have confronted an unexpected complication: psoriasis was paradoxically aggravated or induced by the TNF-α blocker. Although it is not a common complication of TNF-α blocker, this aggravation may be more common than previously thought. To our knowledge, most reports about TNF-α blocker-induced psoriasis have been limited to western countries while only a few cases have been reported in Korea and Japan. In addition, new onset of pustular psoriasis by TNF-α blocker has been reported more commonly than worsening of preexisting psoriasis. Now we report a patient whose preexisting psoriasis vulgaris was aggravated repeatedly after using the TNF-α blocker, infliximab, to control Crohn''s disease, which is a rare rheumatologic disease in Korea.     

Hidradenitis suppurativa: viewpoint on clinical phenotyping,pathogenesis and novel treatments

Hidradenitis suppurativa (HS) is an inflammatory, debilitating follicular skin disease with recurring flare‐ups. The painful, deep‐seated, inflamed lesions in the inverse areas of the body cause severe discomfort, and hence, serious psycho‐social and economic costs. HS is common, but often misdiagnosed and mechanistically poorly understood. Furthermore, HS is notoriously difficult to treat resulting in a high unmet medical need. To provoke debate, rational experimentation and initiate strategic studies, we here present a concise viewpoint on seven topics: the diagnosis of HS, the role of mechanical friction, the critical importance of accurate clinical subgrouping, smoking and obesity, the role of bacteria, and our comprehensive view on HS pathogenesis with a central role for keratin clearance, and novel treatment approaches.     

Chronic linear ulcerations of the inguino-crural and buttocks folds

Vulvo-perineal Crohn's disease is a rare condition either when it is isolated or associated with digestive manifestations. In the former condition named metastatic Crohn's disease, it may constitute a diagnostic challenge and may be confused especially with other infectious or inflammatory disorders. We report a case of vulvo-perineal Crohn's disease in a 46-year-old woman. A 46-year-old woman was diagnosed with a vulvo-perineal Crohn's disease without digestive involvement. There was a chronic edema of the vulva with linear ulcerations on the inguino-crural regions and the buttocks fold, of 3 years. Treatment with metronidazole (1 g/day for 6 months) led to almost complete healing of the ulcerations with a sustained result. Physicians must be aware of the diverse manifestations and confusing presentations of vulvo-perineal Crohn's disease.