1/16. esophageal stenosis in childhood: dystrophic epidermolysis bullosa without skin blistering due to collagen VII mutations.We report a 9-year-old girl who experienced recurrent dysphagia since infancy. Crohn's disease was suspected because she had aphthous ulcers of the mouth and anal dermatitis with hematochezia. After bougienages of esophageal stenoses and medication for inflammatory bowel disease proved unsuccessful, interdisciplinary re-examination revealed the cause of the symptoms to be an extracutaneous form of dystrophic epidermolysis bullosa, a genetic skin fragility disorder. Dystrophic epidermolysis bullosa is caused by mutations in the COL7A1 gene encoding collagen VII, a protein of the epidermal attachment complex, and typically manifests with trauma-induced skin blistering, scarring, nail dystrophy, and, in some cases, mucosal involvement. The present proband never developed skin blisters but had nail dystrophy and erosions of the oral, esophageal, and genitoanal mucosa, which healed with slight scarring. mutation analysis disclosed compound heterozygosity for recessive mutations in the COL7A1 gene. The paternal mutation 425 A-->G caused abnormal splicing resulting in a premature stop codon. The maternal mutation G2775S led to the substitution of a glycine by a serine in the triple helical domain of collagen VII. This case shows that mucosal disease and esophageal strictures in childhood are not always acquired, but can also represent a genetic defect of dermal-epidermal adhesion, even in the absence of skin blistering.- - - - - - - - - - ranking = 1keywords = dermatitis (Clic here for more details about this article) |
2/16. Papular-purpuric gloves-and-socks syndrome with bloody bullae.Papular-purpuric gloves-and-socks syndrome (PPGSS) is a disease characterized by itchy, painful acral erythema with edema, confluent papules, and purpura in a gloves-and-socks distribution and is associated with fever and mucosal lesions. parvovirus B19 and other viral infections have been proven to be causative agents of this syndrome. Its histological findings have been the non-specific ones of interface dermatitis. Here, we report a case of PPGSS in a 44-year-old man that we believe to be the first such case in japan. He developed, within one day, a painful edematous eruption with confluent papules and purpura on his hands and feet accompanied by high fever. A unique clinical manifestation in this case was multiple bloody bullae on the toes, which have not been previously described. Serological tests were negative for parvovirus B19, cytomegalovirus, and measles virus.- - - - - - - - - - ranking = 1keywords = dermatitis (Clic here for more details about this article) |
3/16. A subepidermal blistering dermatosis associated with coexistent IgG and IgA anti-dermal basement membrane zone antibodies; demonstration of IgG antibodies reactive against a 200-kDa dermal antigen.We report a 66-year-old woman presenting with an annular erythematous and bullous eruption. Her clinical and histological findings were similar to those of linear IgA bullous dermatosis or dermatitis herpetiformis. Direct immunofluorescence revealed linear deposition of IgA, IgG and C3 along the basement membrane zone (BMZ). Indirect immunofluorescence detected IgG and IgA antibodies against the BMZ. Salt-split skin technique demonstrated that IgG antibodies bound exclusively to the dermal side, while IgA antibodies bound not only to the dermal side, but also to the epidermal side with relatively weak intensity. On immunoblot analysis, the patient's IgG antibodies exclusively reacted with a band of 200-kDa, while the antigenic target of IgA antibodies was not identified. The present case is thought to be a unique bullous dermatosis mediated by both the IgG antibodies to a novel 200-kDa antigen and IgA antibodies against undetermined antigens.- - - - - - - - - - ranking = 1keywords = dermatitis (Clic here for more details about this article) |
4/16. Severe subacute cutaneous lupus erythematosus presenting with generalized erythroderma and bullae.A 31-year-old woman presented with progressive generalized erythroderma and bullae. Histologic evaluation revealed dyskeratosis and interface dermatitis with a paucity of infiltrate. Serologic evaluation revealed markedly elevated titer of Ro/SS-A and La/SS-B antibodies. Further workup revealed leukopenia. The generalized eruption cleared with prednisone. The patient later had the classic discrete lesions of subacute cutaneous lupus erythematosus develop. The erythrodermic and bullous presentation of subacute cutaneous lupus erythematosus is rare and requires a high index of suspicion.- - - - - - - - - - ranking = 1keywords = dermatitis (Clic here for more details about this article) |
5/16. Bullous lesions in acrodermatitis enteropathica. Histopathologic findings regarding two patients.acrodermatitis enteropathica (AE) is an autosomic recessive disorder affecting early infancy. Two cases of infantile AE with low plasma zinc levels are reported in which unusually prominent bullous and vesicobullous lesions were seen on the hands and feet, in addition to the more typical erythematous and scaly patches. Both psoriasiform and bullous lesions responded dramatically to oral zinc-sulfate supplementation. The histopathologic features of the bullous lesions of AE have not previously been fully examined. Histologically, the bullous lesions were characterized by intraepidermal vacuolar changes with massive ballooning, leading to intraepidermal vesiculation and blistering, with prominent epidermal necrosis and with no acantholysis. The bullous lesions did not arise on erythematous patchy lesions, but developed ex novo on unaffected skin. The histopathologic differential diagnosis with other bullous conditions is discussed.- - - - - - - - - - ranking = 5keywords = dermatitis (Clic here for more details about this article) |
6/16. Angina bullosa haemorrhagica: a case report and a concise review.Various differential diagnoses pertaining to angina bullosa haemorrhagica (ABH) have been reported in the dental literature; these differential diagnoses include mucous membrane pemphigoid, bullous pemphigoid, bullous lichen planus, epidermolysis bullosa, dermatitis herpetiformis, linear IgA disease, and oral amyloidosis. ABH is characterized by a solitary blood blister in the palate and may worsen progressively, leading to multiple lesions in other areas. The bullae usually rupture spontaneously and the sites heal uneventfully. Possible etiological factors include trauma, long-term use of steroids, diabetes, and hereditary predilection. Pathological studies have yielded nonspecific findings and the underlying etiopathology remains ill-defined. This article describes a case of ABH following the use of a 0.12% chlorhexidine gluconate mouthrinse and presents a concise review of the literature pertaining to ABH and differential diagnoses.- - - - - - - - - - ranking = 1keywords = dermatitis (Clic here for more details about this article) |
7/16. tinea manuum bullosa.We report a case of tinea manuum bullosa in a 36-year-old male, a crop and livestock farmer by trade. The lesion, resembling contact dermatitis, was located in the palm of the right hand. We isolated trichophyton verrucosum. No other skin lesion was detected. blood chemistry and immunology test results were normal. Treatment with terbinafine 250 mg day(-1) led to clinical and mycological healing.- - - - - - - - - - ranking = 1keywords = dermatitis (Clic here for more details about this article) |
8/16. Assessing phytophotodermatitis: boy with erythema and blisters on both hands.Phytophotodermatitis (PPD) is a phototoxic reaction which occurs when the skin comes in contact with a photosensitizer and is subsequently exposed to radiation. PPD is often seen in people handling furocoumarin-containing products, such as agricultural workers, bartenders, florists, and gardeners. It may also be seen in beachgoers, athletes, and children. The pattern of the lesions usually resembles streaks; the hands and mouth are most commonly affected due to eating and handling of the offending furocoumarin-containing agents.- - - - - - - - - - ranking = 5keywords = dermatitis (Clic here for more details about this article) |
9/16. Early congenital syphilis and erythema multiforme-like bullous targetoid lesions in a 1-day-old newborn: detection of treponema pallidum genomic DNA from the targetoid plaque using nested polymerase chain reaction.A 1-day-old male newborn was born with respiratory distress, low birth weight, hepatosplenomegaly, and bullous targetoid skin lesions over the face, back, buttocks, and extremities. A diagnosis of early congenital syphilis was made based on a treponemal serologic test. Pathologic examination of the skin lesion showed scattered dyskeratotic cells in the epidermis and interface dermatitis consistent with erythema multiforme. No spirochete could be found in the skin sections staining with Warthin-Starry stain. Using nested polymerase chain reaction, treponemal genomic DNA fragments encoding dna polymerase i were detected.- - - - - - - - - - ranking = 1keywords = dermatitis (Clic here for more details about this article) |
10/16. Bullous systemic lupus erythematosus: an unusual clinical course and detectable circulating autoantibodies to the epidermolysis bullosa acquisita antigen.Bullous systemic lupus erythematosus is a newly recognized form of systemic lupus erythematosus characterized by a skin eruption clinically and histologically resembling dermatitis herpetiformis and responsive to dapsone. We report on a patient with bullous systemic lupus erythematosus who initially presented with lesions clinically resembling erythema multiforme, experienced exacerbation of her disease with dapsone, and had detectable circulating autoantibodies to the epidermolysis bullosa acquisita antigen.- - - - - - - - - - ranking = 1keywords = dermatitis (Clic here for more details about this article) |
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