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.     

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.     

Metastatic Crohn's disease

Metastatic Crohn's disease is a term used to describe a granulomatous reaction occurring in flexures, and separated from the affected areas of the gastro-intestinal tract by normal skin. Previous reports refer to such lesions affecting the groins, the male genitalia, a flexure on the anterior abdominal wall of an obese patient, and submammary regions. A further case, in which the retro-auricular areas are involved, is presented.     

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.     

Metastatic Vulvar Crohn's Disease—A Rare Case Report and Short Review of Literature

Metastatic Crohn''s disease (CD), a type of extraintestinal CD may present with gynecological manifestation which causes diagnostic dilemma and needs multidisciplinary approach. Vulvar lesions occur in very small number of cases with CD of which asymmetrical labial swelling and edema is the most common presentation. We report a case of hypertrophic exophytic variety of vulvar CD because of its rarity.     

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.     

Melkersson-Rosenthal syndrome caused by saprodontia: a case report

A 24-year-old female patient with Melkersson-Rosenthal syndrome (MRS) in association with saprodontia is reported. She presented with lower labial swelling and left facial edema. Histological examination of the involved oral mucosa showed a noncaseating epithelioid granuloma. Results from the laboratory and imaging examinations were normal or negative. Her orofacial swelling disappeared after treatment of the saprodontia of the left first molar.     

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     

Crohn's Disease with Cutaneous Polyarteritis Nodosa in a Child: A Case Report

A 10-year-old boy presented with a 1-day history of multiple painful erythematous skin lesions on his upper and lower extremities. He was admitted to the Department of Pediatrics with persistent right lower abdominal pain and diarrhea. Punch biopsy of a skin lesion on his lower leg showed necrotizing granulomatous vasculitis with septal panniculitis consistent with polyarteritis nodosa, and our differential diagnosis included cutaneous manifestations of Croh''s disease. Abdominal ultrasonography revealed distended colonic loops suggestive of inflammatory bowel disease. Upper and lower gastrointestinal endoscopy revealed lesions involving the duodenum, cecum, colon, and rectum. He developed multiple perianal fistulas during hospitalization. Additional laboratory tests revealed positive results for anti-saccharomyces cerevisiae and antinuclear antibodies. Based on his clinical presentation and laboratory findings, he was diagnosed with Crohn''s disease associated with cutaneous polyarteritis nodosa. We report a rare case of a child who presented with cutaneous polyarteritis nodosa as an extraintestinal manifestation of Crohn''s disease.     

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.     

Orofacial granulomatosis treated with intralesional triamcinolone

Orofacial granulomatosis (OFG) is an uncommon disease, usually presenting as recurrent or persistent swelling of the soft tissues in the orofacial region, predominantly lips (cheilitis granulomatosa). The cause of this illness is unknown. OFG may also be part of the triad of Melkersson-Rosenthal syndrome (MRS) and some consider it a monosymptomatic form of MRS. We describe a case of a Croatian male patient with recurrent swelling limited to the upper lip for the past 6 years. After establishing the diagnosis, we performed intralesional triamcinolone injections (16 mg, twice on a weekly schedule), resulting in complete remission. OFG differential diagnosis and treatment modalities are discussed.     

Metastatic Crohn's disease in a boy presenting with genital swelling

Cutaneous manifestations of Crohn's disease are uncommon and occasionally can affect the genitals with varying symptomatology. When cutaneous features precede or occur in the absence of GI symptoms, this condition may remain undiagnosed. We report the case of a 14-year-old boy who presented for evaluation of persistent penile edema with clinicopathological features consistent with a diagnosis of genital metastatic Crohn's disease, successfully treated with infliximab.     

Acquired Zinc Deficiency in an Adult Female

Acrodermatitis enteropathica is an autosomal recessive inherited disorder of zinc absorption. Acquired cases are reported occasionally in patients with eating disorders or Crohn''s disease. We report a 24-year-old housewife with acquired isolated severe zinc deficiency with no other comorbidities to highlight the rare occurrence of isolated nutritional zinc deficiency in an otherwise normal patient.     

Morphea Developing at the Site of Healed Herpes Zoster

Wolf''s isotopic response describes the occurrence of a new, unrelated disease that appears at the same location as a previously healed skin disease, and the most common primary skin disease of this phenomenon is herpes zoster. Several cutaneous lesions have been described to occur at the site of healed herpes zoster, and granulomatous dermatitis and granuloma annulare have been reported to be the most common second diseases. The pathogenesis of the isotopic response is still unclear. Morphea can develop at the site of regressed herpes zoster and a few such cases have been reported. We present here an additional case of morphea that developed at the site of previously healed herpes zoster, and we review the relevant literature.     

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.     

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.     

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.     

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.