Phrynoderma: A Manifestation of Vitamin A Deficiency? . . . the Rest of the Story

Phrynoderma is a distinctive form of follicular hyperkeratosis associated with nutritional deficiency. Although originally thought to represent vitamin A deficiency, several studies have demonstrated multiple etiologies. Characteristic skin lesions are hyperkeratotic papules that first appear on the extensor surfaces of the extremities, shoulders, and buttocks. We report a 14-month-old boy with malnourishment and hyperkeratotic papules and plaques with histologic changes typical of phrynoderma. Despite an extensive evaluation, a specific nutritional deficiency was not identified. Phrynoderma is believed to be a manifestation of severe malnutrition, not necessarily accompanying low vitamin A levels. While the literature supports a link between phrynoderma and vitamins E, B, A, and essential fatty acids general malnutrition seems to be the strongest association. The clinical picture typically improves with enhanced nutritional status. Phrynoderma must be considered in the differential diagnosis in patients with extensor surface hyperkeratotic papules and plaques in the setting of malnourishment and should prompt the clinician to evaluate cell markers of nutritional status, not just vitamin A. We believe this patient exemplifies the conundrum that faces clinicians in evaluating patients with extensor surface predominant hyperkeratotic papules and plaques in the setting of malnourishment.     

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.     

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.     

Crohn's Disease Presenting as a Neutrophilic Dermatosis in a 5‐Month‐Old Boy

A 5‐month‐old boy with a previous history of failure to thrive and poor feeding was admitted to the hospital with failure to thrive, oral ulcers, and a generalized vesiculopustular rash that demonstrated a subcorneal pustule and neutrophilic infiltrate on histology. Esophagogastroduodenoscopy and flexible sigmoidoscopy biopsies demonstrated chronic active colitis with granulomas, consistent with the diagnosis of Crohn's disease. Our case represents, to our knowledge, the youngest person reported with this condition in association with 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.     

Malnutrition and skin disease in Far East prisoners‐of‐war in World War II

During the Second World War, thousands of captured British and Commonwealth troops were interned in prisoner‐of‐war (POW) camps in the Far East. Imprisonment was extremely harsh, and prisoners developed multiple pathologies induced by physical hardship, tropical infections and starvation. Immediately after the war, several POW doctors published their clinical experiences, including reports of skin disease caused by malnutrition. The most notable deficiency dermatoses seen in Far East POWs were ariboflavinosis (vitamin B2 or riboflavin deficiency) and pellagra (vitamin B3 or niacin deficiency). A lack of vitamin B2 produces a striking inflammatory disorder of scrotal skin. Reports of pellagra in POWs documented a novel widespread eruption, developing into exfoliative dermatitis, in addition to the usual photosensitive dermatosis. A review of the literature from 70 years ago provides a reminder of the skin's response to malnutrition.     

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.     

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.     

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.     

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.     

Phrynoderma and acquired acrodermatitis enteropathica in breastfeeding women after bariatric surgery

Background and objectives: Women who have undergone bariatric surgery are susceptible to nutritional deficiencies in subsequent pregnancies. We highlight the importance of dermatologists in the early recognition of cutaneous signs of malnutrition occurring in this specific clinical setting. Patients and methods: We compare clinical characteristics of two young women with dermatological signs of combined post‐gestational nutritional deficiencies following Roux‐en‐Y gastric bypass surgery. Results: Patient 1 exhibited follicular papules on the extremities, perianal eczema, perlèche, alopecia, and depigmentation of hair. Patient 2 showed erythematous plaques in genitoanal and acral areas, perlèche, diffuse alopecia, and depigmentation of hair. Based on clinical and histopathological findings, decreased vitamin A (patient 1) and zinc levels (patient 2), we diagnosed phrynoderma and acquired acrodermatitis enteropathica, respectively. Comparison of the two patients revealed that both (i) were lacking follow‐up after gastric bypass surgery, (ii) developed skin lesions as primary symptoms with (iii) mixed clinical manifestations due to combined deficiencies, and (iv) experienced initial symptoms during lactation suggesting a causal relationship. Conclusions: Our observations highlight the potentially increased risk of women to develop post‐gestational dermatological manifestations of malnutrition following bariatric surgery. The awareness of dermatologists with respect to this emerging, susceptible patient group may help avert damage to mother and child.     

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.     

Adolescent “Saxophone Penis” Secondary to Crohn's Disease: Misdiagnosed as Being Posttraumatic

We describe a patient with a “saxophone penis” mistakenly diagnosed as having posttraumatic changes. A careful history and attention to blood tests at presentation may have alerted clinicians that there was a nontraumatic cause for our patient's disease. It is essential that clinicians consider “metastatic” anogenital Crohn's disease as a possible cause of “saxophone penis”.     

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.     

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.     

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.     

Nomalike Necrotizing Stomatitis in a Child with Crohn's Disease

Noma is an opportunistic infection characterized by devastating gangrenous stomatitis leading to severe tissue destruction that predominantly affects malnourished children in sub‐Saharan Africa. Only a few cases have been reported in immunocompromised patients from developed countries. We present an unusual case of nomalike necrotizing stomatitis in a previously healthy child with Crohn's disease in Korea.     

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 vasculitis in Crohn's disease: a clinicopathologic correlate of two unusual cases

Cutaneous complications occur not uncommonly in patients with Crohn's disease (CD). Gastrointestinal CD often shows non‐caseating granulomas and a rare cutaneous finding in CD is a sterile granulomatous infiltrate not contiguous with the GI tract, termed extraintestinal CD (ECD). The clinical presentation of ECD is diverse. The most common histopathological presentation is a superficial and deep granulomatous infiltrate that often accompanies a mixed perivascular infiltrate. Here we report two patients with CD and skin lesions characterized on microscopy by granulomatous vasculitis. A 29‐year‐old female presented with papules and ulcerated nodules above the ankle. The biopsy showed dermal and superficial subcutaneous involvement by a vasocentric infiltrate of mononuclear and multinucleated histiocytes as well as mural fibrin deposition. A 35‐year‐old male presented with two tender indurated erythematous plaques with punched‐out centers on the lower leg. Histopathologically, a granulomatous vasculitis of small and medium‐sized vessels in the dermis and subcutis was evident. These two cases represent the rarely described phenomenon of cutaneous granulomatous vasculitis in CD. Previously reported examples of this entity are reviewed. Burns AM, Walsh N, Green PJ. Granulomatous vasculitis in Crohn's disease: a clinicopathologic correlate of two unusual cases.