Subclass distribution of IgG autoantibodies in bullous pemphigoid

The distribution of IgG subclasses in the antibasement membrane zone autoantibody of pemphigoid in skin and serum was analyzed by use of monoclonal antibodies to human IgG subclasses. The predominant subclass was IgG4 which was present in 23 of 24 skin biopsies, IgG1 was next and IgG3 was found only occasionally. In 3 of 24 biopsies IgG4 was the only IgG subclass detected, C3 was absent in 2 of these, the third contained IgM and C3. Serum autoantibodies were similarly analyzed by indirect immunofluorescence (IIF) when again IgG4 autoantibody was the dominant subclass. No IgE autoantibody was detected by IIF.     

Steroid allergy in patients with inflammatory bowel disease

Background: Contact allergy to a steroid enema leading to worsening of inflammatory bowel disease (IBD) has recently been reported. This study was designed to look for evidence of steroid allergy in patients with IBD. OBJECTIVES: To look for the presence of steroid allergy in general, and steroid enema allergy in particular, in a cohort of IBD patients prepared to attend for patch testing. METHODS: Patients with IBD in two gastroenterology units at Dublin and Cork were asked to take part in the study. Those who agreed to take part were tested to the British Contact Dermatitis Society standard and steroid batteries. Patients with positive tests to steroids were subsequently asked to attend for intradermal testing with prednisolone, and patch testing to the two commercially available steroid enemas in Ireland and an extended battery of steroids. Reactions were read at days 2 and 4. RESULTS: In total, 44 patients from the two units were patch tested. Four patients had positive patch tests to one or more steroids in these batteries: budesonide (n = 2), triamcinolone acetonide (n = 1), tixocortol pivalate (n = 1) and prednisolone (n = 1). Of these, three underwent further testing. All three had positive reactions to intradermal prednisolone and one had a positive test to steroid enema. CONCLUSIONS: Four (9%) of our patients were found to have steroid allergy. This has important implications for the local and systemic treatment of their IBD.     

The epidermolysis bullosa acquisita antigen (type VII collagen) is present in human colon and patients with crohn's disease have autoantibodies to type VII collagen

Epidermolysis bullosa acquisita is an autoimmune blistering disease of the skin characterized by IgG autoantibodies against type VII collagen. Systemic diseases are often associated with epidermolysis bullosa acquisita, Crohn's disease being the most frequent. This study sought to determine if type VII collagen, the epidermolysis bullosa acquisita autoantigen, was present in normal human colon by western blotting and immunofluorescence. The 290 kDa type VII collagen alpha chain was demonstrated by western blotting in four normal intraoperative colon specimens. Antibodies to type VII collagen labeled the junction between the intestinal epithelium and the lamina propria. We also used an enzyme-linked immunosorbent assay to test sera from patients with Crohn's disease (n = 19), ulcerative colitis (n = 31), celiac disease (n = 17), rheumatoid arthritis (n = 15), and normal controls (n = 16). It was found that 13 of 19 patients with Crohn's disease and four of 31 patients with ulcerative colitis demonstrated reactivity to type VII collagen. Sera from control subjects, patients with celiac disease or rheumatoid arthritis were negative. The sera from Crohn's disease patients also reacted with type VII collagen by immunoblot analysis. It was concluded that patients with inflammatory bowel disease may have IgG autoantibodies to type VII collagen, which exists in both the skin and the gut.     

Treatment of severe acne with isotretinoin in patients with inflammatory bowel disease

Four patients with inflammatory bowel disease and severe cystic acne were treated with isotretinoin. Two patients had a successful course of treatment without any gastrointestinal side-effects. One patient had two episodes of profuse rectal bleeding that were probably related to pre-existing haemorrhoids. The fourth patient had a flare-up of his Crohn's disease after starting isotretinoin.     

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.     

A case of nonscarring subepidermal blistering disease associated with autoantibodies reactive with both type VII collagen and laminin 5

A 35-year-old Japanese woman had recurrent, pruritic, vesicular lesions on the face, neck and upper back as well as erosive lesions of the oral cavity and genitalia. The skin and mucosal lesions healed without scarring upon the systemic administration of corticosteroid and azathioprine. Direct immunofluorescence revealed linear deposits of IgG, IgA and C3 at the cutaneous basement membrane zone. Indirect immunofluorescence on 1 M NaCl-split human skin sections demonstrated that the patient's IgG antibodies reacted with the dermal side of the split, while IgA antibodies weakly reacted with the epidermal side. By immunoblot analyses, the patient's serum reacted with the NC1 domain of type VII collagen as well as both the alpha3- and beta3-subunits of laminin 5. We regarded our case as a nonscarring subepidermal blistering disease with autoantibodies to both type VII collagen and two different subunits of laminin 5. Such a case has not been previously reported.     

坏疽性脓皮病合并炎症性肠病

报告1例坏疽性脓皮病合并炎症性肠病。患者男,30岁。腹痛、腹泻及稀薄脓血便1个月余;躯干及四肢出现散在的红色丘疹、脓疱疹,溃疡伴疼痛12 d入院。皮损组织病理检查:表皮角化过度,棘层增厚,真皮全层及皮下脂肪间大量中性粒细胞浸润,可见红细胞外渗及血管壁纤维蛋白样变性。电子肠镜诊断为炎症性肠病。抗核周型中性粒细胞胞质抗体(anti perinuclear antineutrophil cytoplasmic antibody,p-ANCA)阳性。予糖皮质激素、美沙拉嗪及支持治疗后,患者病情明显好转。     

Erythema nodosum-like eruption as a manifestation of azathioprine hypersensitivity in patients with inflammatory bowel disease

BACKGROUND: Clinical manifestations of hypersensitivity to azathioprine may mimic symptoms of the initial disease. We report 5 cases of peculiar skin hypersensitivity reactions to azathioprine in patients with inflammatory bowel disease. OBSERVATIONS: In 5 patients with a recent azathioprine regimen, manifestations appeared between 8 and 18 days after drug introduction. All patients had a high fever. Three patients initially had erythema nodosum; 2 patients had sterile pustules. All had elevated neutrophil counts and serum C-reactive protein levels, whereas eosinophil counts were normal, ruling out drug-induced rash with eosinophilia and systemic symptoms. In 3 patients who were rechallenged with azathioprine or with 6-mercaptopurine, dermatological lesions recurred within hours. CONCLUSIONS: Erythema nodosum and pustules are rarely reported manifestations of azathioprine hypersensitivity. Both skin lesions may be related to the clinical activity of inflammatory bowel disease. Relapse of such lesions shortly after thiopurine rechallenge should raise the hypothesis of hypersensitivity rather than pharmacological manifestations.     

Skin manifestations of inflammatory bowel disease

Ulcerative colitis (UC) is an inflammatory disorder of the colon that is associated with several extraintestinal manifestations in multiple organs. Several mucous membrane and skin disorders occur in patients with UC. These disorders are not unique to UC and often occur secondary to other causes or in the absence of an apparent cause. One or more such disorders may occur together in association with UC. Mucous membrane and skin disorders may antedate, occur with, or postdate the onset of UC. The dermatologist plays an important role in suspecting the diagnosis of UC that presents with associated mucous membrane or skin disorders. This review covers the clinical presentation, differential diagnosis, workup, and management of selected mucocutaneous manifestations in UC.     

Subclass characteristics of IgG autoantibodies in bullous pemphigoid and pemphigus

Immunoglobulin (Ig) G subclasses in anti-basement membrane zone (BMZ) autoantibodies found in the sera of bullous pemphigoid (BP) and in anti-intercellular substance (ICS) autoantibodies of pemphigus were investigated using immunofluorescent (IF) staining. In BP, IgG4, IgG1, and IgG2 were detected in 13, 5 and 6 of 15 patients, respectively; IgG3 was not detected. In pemphigus, IgG4 was detected in all of 10 patients, IgG1 in 7, IgG2 in one, and IgG3 in one patient, respectively. In both BP and pemphigus, the most prominent subclass in intensity of IF staining was IgG4. Although one BP and one PV patient had only IgG4 autoantibodies, C3 deposition was detected. The quantification of IgG subclasses in the sera of the patients was performed by enzyme-linked immunosorbent assays (ELISA). Serum levels of IgG4 in both BP and pemphigus were elevated approximately 3-fold over those in normal controls; those of whole IgG and IgG1-3 were not significantly elevated. Using direct IF staining, the deposition of C3 at the BMZ and at the ICS was demonstrated in 9 of 10 BP and in 3 of 8 pemphigus patients, respectively. The prominent IgG subclasses of anti-BMZ and anti-ICS antibody were IgG4, a noncomplement-fixing antibody, suggesting that the deposition of C3 in the lesional skin occurred via the alternative pathway, or that small amounts of IgG1-3 subclass autoantibodies activated the classical pathway.     

Leucocytoclastic vasculitis and angio-oedema associated with inflammatory bowel disease

Two cases are reported in which purpuric skin plaques due to leucocytoclastic vasculitis and angio-oedema were the presenting signs of inflammatory bowel disease. The aetiological implications are discussed. One case was controlled with oral colchicine, the other with oral Salazopyrine.