Vulval papules: A case for diagnosis

You are asked to examine a 68‐year‐old woman with a 3‐year history of multiple bilateral and largely asymptomatic white papules on her labia majora. Based on the history and photographs, what is your diagnosis?     

Vulval disease in pre-pubertal girls

Children present with vulval complaints less frequently than do adults; although there are many similarities between paediatric and adult groups of patients with vulval disease, there are also important differences. In both groups, dermatitis, psoriasis and lichen sclerosus are the most frequently seen dermatoses. Birthmarks and congenital abnormalities presenting for the first time are more of an issue in children than in adults. Fusion of the labia and streptococcal vulvovaginitis are conditions seen only in the paediatric group. Sexually transmitted diseases such as genital warts and genital herpes are not common in this group and should always raise the possibility of child sexual abuse. Chronic vulvovaginal candidiasis, although a very common problem in adult patients, is not seen in the prepubertal group.     

Vulval Crohn disease: A case series of 26 patients

Vulval Crohn disease is rare and likely under-reported, leading to difficulty in diagnosis and delay in treatment. In this case series, we report the clinical features and discuss therapy in 26 consecutive patients with vulval Crohn disease, with or without documented gastrointestinal disease, presenting between January 2016 and July 2018. We highlight the need for treatment that is in some cases more aggressive than the requirement to manage the patient's gastrointestinal involvement.     

Metastatic Crohn's disease: a review

Metastatic Crohn's disease (MCD) indicates the presence of non-caseating granuloma of the skin at sites separated from the gastrointestinal tract by normal tissue and is the least common dermatologic manifestation of CD. In adults, MCD usually appears after the initial diagnosis of CD in 70% of cases, whereas in children, it appears at the same time as CD in almost half of the cases. The most frequent skin lesions in adults are nodules, plaques with or without ulceration on the extremities and ulcers on the genitals. In children, genital swelling with or without erythema is the most frequent presentation of MCD. Simultaneous presence of perianal CD affects more females (60%) and particularly children. Associated gastrointestinal symptoms are present in one third of the cases in adults and in half of the cases in children. Treatment is often unsatisfactory. Randomised controlled trials are lacking. Various chemotherapeutic agents have been used such as oral metronidazole, topical and/or oral steroids, azathioprine, cyclosporine, sulfasalazine, tetracyclines, topical or systemic tacrolimus, infliximab alone or with methotrexate, and surgical treatment with oral zinc sulphate. MCD represents another 'great imitator'. This reviews the most relevant characteristics of this disease, in order to increase awareness and to avoid delay in diagnosis and improve management of the whole CD complex.     

Metastatic Crohn's disease

Crohn's disease is a granulomatous inflammatory disease of the gastrointestinal tract. It can involve other sites of the body also. When such lesion are separated from Crohn's lesion of the intestine by normal skin, they are referred to as metastatic Crohn's disease. Two such cases are reported.     

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.     

Metastatic Crohn's disease

Cutaneous granuloma formation distant from the gastrointestinal tract in patients with Crohn's disease of the bowel has been called metastatic Crohn's disease. We report two patients with this entity, including the first to present with an erysipelas-like eruption of the face. A review of the world's literature reveals that all patients with metastatic Crohn's disease have had gastrointestinal disease involving the colon or rectum. Clinical features of the nine previously reported cases are reviewed.     

Perianal Crohn's disease

A 13-year-old girl with a history of 4 months of perianal skin lesions is described. Physical examination revealed three 0.5 I 1-cm red, swollen, fleshy, skin tags extending from the perianal area to the perineum (Fig. 1). The patient reported intermittent fever, diarrhea, and abdominal pain, and her body weight was below the third percentile for her age. Laboratory studies showed an erythrocyte sedimentation rate of 101 mm/h; hematocrit of 26%; white blood cell count of 9800/mm3; serum iron of 15 mg/L (normal value (NV), 60-160 mg/L); ferritin of 43.4 microg/L (NV, 12-150 microg/L); transferrin of 203 mg/100 mL (NV, 200-400 mg/100 mL); transferrin saturation of 6% (NV, 20-50%); hypoalbuminemia; negative purified protein derivative (PPD), cytomegalovirus (CMV), human immunodeficiency virus (HIV), venereal disease research laboratory (VDRL), and antinuclear antibody tests; and Toxoplasma titers of 1/16, Van de Kamer 1.67 g/day. A barium examination revealed marked irregularity of the descending colon, and a colonoscopy showed uneven areas of mucosal edema and pseudopolyps in the transverse and descending colon, associated with irregular thickening and stenosis. Histopathologically, large intestine and skin lesions consisted of noncaseating epithelioid and giant cell granulomas (Fig. 2). Cultures for acid-fast bacilli and fungi were negative, and under polarized light no foreign bodies were seen. Treatment with metronidazole (250 mg three times a day), prednisone (0.5 mg/kg/day), and acetylsalicylic acid (75 mg/kg/day) was moderately effective. Vitamin, folic acid, and iron supplements were also added.     

Vulval varicosities in pregnancy

Vulval varicosities reportedly occurs in pregnant women. We report a case of vulval varicosities with associated varicosities of the breast and saphenous veins, which regressed completely post partum with conservative management.     

Metastatic Crohn's disease in a child

Metastatic Crohn's disease is a rare complication of Crohn's disease that has not been frequently reported in the literature. Although Crohn's disease is common in children, cutaneous manifestations are rarely a presenting sign. We report the case of a young boy who initially presented with unusual skin lesions and whose condition was diagnosed later as Crohn's disease.     

Extensive necrobiosis in metastatic Crohn's disease

This report is on a 33-year-old female patient with Crohn's disease of the intestine and nonhealing leg ulcers which histologically showed the typical noncaseating epithelioid granulomas of sarcoid structure defining the so-called 'metastatic Crohn's disease'. Furthermore, as a very rare pattern, extensive necrobiotic areas surrounded by sarcoid granulomas were observed.     

Erythema induratum (nodular vasculitis) associated with Crohn's disease: a rare type of metastatic Crohn's disease

We herein report a patient with erythema induratum/nodular vasculitis (EI/NV) associated with Crohn's disease (CD), which is considered to be a rare type of metastatic CD. A 54-year-old woman, who had a history of CD, presented with erythematous nodules on her legs. The histopathological features of the skin biopsy revealed a granulomatous, mixed septal and lobular panniculitis, which was characterized by many discrete epithelioid cell granulomas (necrobiotic/necrotizing-type and sarcoidal type), necrosis of the adipocytes, and granulomatous phlebitis in the muscular wall of a subcutaneous vein. A review of the pertinent literature and the presented case suggested the following: (1) panniculitis associated with CD may be either an erythema nodosum type or an EI/NV type; (2) so far, the reported cases of metastatic CD or granulomatous vasculitis in CD rarely presented with granulomatous panniculitis without dermal involvement, and most cases showed histopathological features that were similar to or indistinguishable from those of EI/NV; and (3) the finding of granulomatous vasculitis (especially the presence of discrete epithelioid cell granulomas involving the veins or venules) may be a characteristic feature of EI/NV associated with CD, in contrast to the finding of acute vasculitis, which is typically present in patients with EI/NV due to causative factors other than CD.