Tetracycline and niacinamide control bullous pemphigoid but not pemphigus foliaceus when these conditions coexist

Pemphigus and pemphigoid are different types of autoimmune bullous disease and can occur in the same patient. We report a female patient with this condition. At first, we diagnosed her with bullous pemphigoid, and we treated her with tetracycline, niacinamide and a topical steroid. Tense bullas disappeared shortly after that, but crusted erythemas mainly on her head and trunk persisted. We examined BP180 and desmoglein 1 enzyme‐linked immunosorbent assays, and also histological features, which showed coexistence of bullous pemphigoid and pemphigus foliaceus concurrently. Therefore, we tried prednisolone, which could control both conditions. This case showed that tetracycline and niacinamide could control bullous pemphigoid, but could not control pemphigus foliaceus, and that prednisolone was effective for both conditions.     

Systemic lupus erythematosus presenting as a bullous eruption in a child

An 8-year-old girl presented with a generalized bullous eruption clinically resembling bullous pemphigoid or chronic bullous disease of childhood. Further study revealed immunopathologic findings seen in patients with epidermolysis bullosa acquisita or bullous systemic lupus erythematosus (SLE). Although she did not fulfill the American Rheumatism Association (Atlanta) criteria for SLE at her presentation, one year later she went on to do so. As well as being the youngest patient reported with bullous SLE, our patient is noteworthy because the bullous eruption was the initial manifestation of her SLE. Bullous SLE should be considered in the differential diagnosis of children presenting with generalized bullous eruptions.     

Childhood cicatricial pemphigoid confined to the vulva

Cicatricial pemphigoid or benign mucous membrane pemphigoid is an autoimmune bullous disease predominantly affecting the mucosal surfaces and healing with scar formation. Localized cicatricial pemphigoid of the vulva in children is rare. We present a child with this rare condition who was initially investigated on suspicion of her being subjected to child sexual abuse, and discuss its management.     

Adverse Reaction to Prednisone in a Patient with Systemic Lupus Erythematosus

Oral corticosteroids are the main therapeutic choice for systemic lupus erythematosus (SLE). Adverse reactions to systemic corticosteroids rarely occur and the etiology is unclear in most cases. A 14-year-old girl with newly diagnosed SLE developed a pruritic bullous eruption while on prednisone. The patient had been treated successfully in the hospital with intravenous methylprednisolone. In preparation for discharge, the steroid preparation was changed to prednisone to which the patient reacted with a development of new crops of bullous lesions. Skin biopsy specimens of lesional areas showed a bullous eruption consistent with erythema multiforme. The patient underwent immediate and delayed hypersensitivity tests. Intradermal and patch tests to liquid prednisone were positive. The patient was discharged on oral methylprednisolone and has not had recurrence of the skin lesions. In conclusion, a case of prednisone sensitivity in a patient with SLE is presented here. An alternative preparation, methylprednisolone, was used to successfully treat her underlying condition.     

Bullous Pemphigoid in Infancy: Case Report and Literature Review

Abstract: We report a 2-month-old girl who developed bullous pemphigoid on her hands and feet shortly after receiving her routine immunizations. Infantile bullous pemphigoid has a clinical presentation distinct from bullous pemphigoid seen in older children and should be included in the differential diagnosis of blisters involving the hands and feet. Our patient responded well to topical corticosteroid therapy.     

Extragenital Bullous Lichen Sclerosus in a Pediatric Patient: A Case Report and Literature Review

A 14‐year‐old girl presented with a 1‐year history of a pruritic, bullous lesion on her posterior neck. A biopsy revealed bullous lichen sclerosus. Although unusual, this bullous variant of lichen sclerosus is well recognized in the adult literature, but extragenital bullous and hemorrhagic lesions are rare in children. A review of this case and the literature describes the clinical features, pathophysiology, and treatment options for this extragenital bullous variant in an effort to raise awareness of this rare clinical presentation.     

Childhood bullous pemphigoid: report of a case with life-threatening course during homeopathy treatment

Bullous pemphigoid (BP) is an autoimmune blistering disorder that may very rarely occur in childhood. We describe a 9-month-old child who developed bullous pemphigoid while she was being treated for presumptive atopic eczema with a homeopathic regimen comprising sulfur, mercury, cantharides, and Rhus (Toxicodendron). She had generalized bullae and a progressive worsening of her general condition with asthenia, dehydration, malnutrition. While the role of homeopathy in triggering the disease remains unclear, our observation attests to the potential life-threatening course of childhood BP in instances where appropriate treatment is withheld.     

Infliximab for treatment of resistant pyoderma gangrenosum associated with Crohn's disease

We present the case of an 18-year-old woman with Crohn's disease manifested by diffuse abdominal pain, bloody diarrhea accompanied by arthralgia, and swelling of large joints. On the lateral aspect of her right ankle there was an hemorrhagic, necrotic bullous lesion measuring 3 x 4 cm, surrounded by cutaneous inflammation and erythema. Biopsy showed a neutrophilic abscess-like ulcerative skin inflammation, which was diagnosed as pyoderma gangrenosum. The patient was treated with high doses of parenteral methylprednisolone, but her condition failed to improve and infliximab, a TNF-alpha blocking agent, was instituted. An immediate response of Crohn's disease was observed and, over the next 5 weeks, the ulcer on her right ankle also healed completely.     

大疱性肥大细胞增生症

报告1例以头皮大疱为主要特点的大疱性肥大细胞增生症.患儿女,11个月.头皮反复起水疱、大疱3个月,每次发作前,面部潮红,头部出现风团样皮损,而后出现水疱、大疱,皮肤划痕征阳性.皮损组织病理和Giemsa染色证实为大疱性肥大细胞增生症.     

表现为嗜酸性粒细胞增多性皮炎的大疱性类天疱疮一例

患者,女,73岁。全身散在红斑水疱、弥漫性色素斑伴瘙痒1年。查体:躯干弥漫性条索状和漩涡状黑褐色色素沉着斑及散在绿豆至蚕豆大小不等的水疱,四肢有丘疹、结节样皮损,腹股沟淋巴结肿大。嗜酸粒细胞计数21.63×10~9/L,病理支持大疱性类天疱疮诊断。给予糖皮质激素、甲氨蝶呤同时联合干扰素α治疗后,病情得到明显缓解。     

Interferon-alpha-associated development of bullous lesions in mycosis fungoides

We report a 67-year-old woman with mycosis fungoides (MF) who was receiving subcutaneous injections of recombinant interferon alpha-2b (IFN-alpha). After 2 months of IFN-alpha treatment, bullous lesions appeared on her trunk and extremities. A skin biopsy specimen from the trunk revealed histopathologic features of bullous MF. Bullous lesions with specific infiltrates of MF are very rare; to the best of our knowledge, this is the first case showing specific bullous lesions of MF developed under IFN-alpha treatment. Copyright (R) 2000 S.Karger AG, Basel     

大疱性肥大细胞增生症1例

患儿女,13个月。躯干反复出现水疱和大疱3月、风团伴瘙痒1月。皮损组织病理示:表皮下大疱形成,真皮中上部弥漫性疑似肥大细胞浸润。甲苯胺蓝染色示:细胞质内有紫红色异染性颗粒。诊断:大疱性肥大细胞增生症。     

DNA-based prenatal exclusion of bullous congenital ichthyosiform erythroderma at the early stage, 10 to 11 weeks' of pregnancy, in two consequent siblings

The proband was a Japanese woman with bullous congenital ichthyosiform erythroderma harboring a keratin 10 gene mutation M150T. DNA-based prenatal exclusion of bullous congenital ichthyosiform erythroderma was successfully performed in her two consequent pregnancies using chorionic villus samples at 10 to 11 weeks' gestation, several weeks earlier than the previously reported cases.     

Dowling-Meara dominant epidermolysis bullosa. An intraepidermal epidermolysis bullosa which hides its prognosis well

The epidermolysis bullosa simplex (intraepidermal) disorders represent a heterogeneous group of bullous diseases all inherited in an autosomal dominant mode. The prognosis is usually good and the bullous lesions heal without scarring. We present here three patients affected with intraepidermal epidermolysis bullosa of the Dowling-Meara type with varying prognoses. Case n. 1. This 4-year-old girl was first seen at the age of 15 months for numerous bullous lesions distributed over her entire skin surface and on her oral mucosa. The blisters, first noted shortly after birth, showed an herpetiform distribution and a thick and hyperkeratotic roof. A yellowish palmoplantar keratoderma was also present. At the age of four the bullous eruption remained extremely severe. The family history revealed no similar cutaneous disorders. Histology showed focal intraepidermal separation and ultrastructural examination revealed that the split occurred above the dermoepidermal junction within the basal cell cytoplasm. Tonofilament clumping was observed. The dermoepidermal junction was normal with hemidesmosomes and anchoring fibrils showing no significant abnormalities. Case n. 2. This 8-year-old boy presented at the age of 5 with numerous bullous lesions involving most of the skin surface and mucosa. The family history was unremarkable. The blisters, present since birth, were numerous and were often circinate with central healing. Palmoplantar keratoderma was noted. Electron microscopy showed intraepidermal separation occurring in the basal cell layer with tonofilament clumping.(ABSTRACT TRUNCATED AT 250 WORDS)     

Successful treatment of one case of pemphigoid gestationis complicating with polycystic ovary syndrome

Pemphigoid gestationis (PG) is a rare, autoimmune, subepidermal bullous dermatosis associated with pregnancy. It is mainly caused by autoantibodies against hemidesmosomal proteins‐bullous pemphigoid 180, and usually presents in the second or third trimester of pregnancy sometimes exacerbates spontaneously after delivery. Here we reported a case of PG with polycystic ovary syndrome (PCOS). This patient received assisted reproduction technology and suffered PG in her third trimester of pregnancy. To the best of our knowledge, there is no similar case has been reported.     

Bullous amyloidosis: the mechanism of blister formation revealed by electron microscopy

BACKGROUND: Few electron microscopic studies of blister lesions in bullous amyloidosis have been reported, and the mechanism of blister formation remains to be elucidated. This study was designed to examine the nature of bullous amyloidosis ultrastructurally, and clarify the pathogenesis of blister formation. METHODS: We examined a 47-year-old woman with IgD-lambda type myeloma, suffering from bullous lesions on her hands and feet caused by trauma or rubbing. Light and electron microscopic studies were performed. RESULT: Ultrastructurally, amyloid deposits aggregated under the lamina densa. Keratinocyte protrusions penetrated the dermis through the gap in the lamina densa and enfolded amyloid deposits. Amyloid globules were found in enlarged intercellular spaces of keratinocytes. Desmosomes were sparsely distributed in some areas of the epidermis. CONCLUSION: These results indicate that keratinocytes enfold the amyloid globules and take them in the intercellular space of epidermis, and that the breakdown of the lamina densa and widening of the intercellular space between keratinocytes induce skin fragility. Trauma or rubbing of her hands and feet appears to act as the localized precipitating factor of blister formation in bullous amyloidosis.     

Bullous pemphigoid of childhood: immunofluorescent investigation

A four-month-old Japanese girl with bullous pemphigoid of childhood (BPC) was reported. The characteristics of BPC antigen were studied by immunofluorescent technique using the serum of this patient with variously treated normal human skin as substrate. This study showed that her BPC antigen was quite similar to bullous pemphigoid antigen seen in adult patients and that this BPC antigen was differed from epidermolysis bullosa acquisita (EBA) antigen.     

Bullous Pemphigoid and Ulcerative Colitis

ABSTRACT: A 21-year-old white woman, who had ulcerative colitis for 14 years, developed generalized severe bullous pemphigoid. Following the resection of her colon, her skin showed marked clinical improvement, but this was only temporary. Direct immunofluorescence was performed on the surgical specimen and no antibodies (BMZ) to colonic mucosal cells were evident. Anti-basement membrane zone antibodies were found on direct and indirect immunofluorescent studies and have persisted. Sera from 15 patients with ulcerative colitis and 11 patients with Crohn's disease, evaluated for the presence of an anti-basement membrane zone antibody did not contain any demonstrable levels of anti-BMZ antibodies. The co-existence of ulcerative colitis and bullous pemphigoid is more likely incidental rather than etiopathologic.     

Bullous systemic lupus erythematosus responding to dapsone

A 29-year-old woman with a 4-week history of systemic lupus erythematosus presented acutely with a severe generalized tense vesicular and bullous eruption with involvement of mucosal surfaces. At the time of her initial diagnosis of systemic lupus erythematosus, she had declined treatment, preferring to explore complementary medical therapies. Skin biopsy showed subepidermal blister formation with inflammation at the dermoepidermal junction. Direct immunofluorescence revealed strongly positive linear deposition of IgG and IgM, and positive linear granular deposition of IgA along the basement membrane zone. Electron microscopy showed that the level of the basement membrane split was below the lamina densa. A diagnosis of bullous systemic lupus erythematosus was made and dapsone was commenced, with a dramatic improvement in her skin eruption. The patient again declined further treatment of her systemic disease and sought complementary therapies, and subsequently presented with cerebral involvement.     

IgA bullous pemphigoid: a distinct blistering disorder

We report a patient with an eccrine carcinoma who developed localized blistering which clinically resembled pemphigoid, histologically showed subepidermal blistering with features of both dermatitis herpetiformis and bullous pemphigoid, responded to dapsone and exhibited linear IgA deposition on direct immunofluorescence. The nosological position of patients with linear IgA deposition and subepidermal blistering is not clear. A review of the literature reveals that in adults linear IgA deposition may occur in three separate situations: dermatitis herpetiformis, bullous pemphigoid and a third condition of which our case is an example which is best termed IgA bullous pemphigoid. This condition is distinguished from cases of dermatitis herpetiformis with linear IgA by the clinical features and the site of IgA deposition on immunoelectronmicroscopy. It is distinguished from cases of bullous pemphigoid with linear IgA by the absence of circulating IgG antibasement membrane zone antibody, the therapeutic response to dapsone and the frequent occurrence of circulating IgA antibasement membrane zone antibody. IgA bullous pemphigoid has not previously been reported with a carcinoma but the association lends further support to the concept that this eruption represents a variant of pemphigoid.