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21/58. Bullous congenital ichthyosiform erythroderma masquerading as dystrophic epidermolysis bullosa.

    A black baby, born at full term of a normal healthy mother, showed multiple erosions and ulcers. No history of skin diseases was known in the family. A skin biopsy taken shortly after birth from an arm lesion showed subepidermal edema and cavity formation. Electron microscopy showed clumping of microfibrils and anchoring fibril-like fibers at the dermo-epidermal junction. In more severely affected areas, absence of anchoring fibrils and collagenolysis were found and the diagnosis of dystrophic epidermolysis bullosa was made. Two weeks later, the lesions healed without leaving scars and a second biopsy from the chest area revealed typical histopathology of epidermolytic hyperkeratosis.
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ranking = 1
keywords = epidermolysis bullosa, epidermolysis, bullosa
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22/58. Trauma-induced bullous pemphigoid.

    We describe three patients with bullous pemphigoid who presented with bullae at sites of trauma and with little spread of the condition outside such areas. The diagnosis was confirmed by histology and direct and/or indirect immunofluorescence; electron microscopy was performed in two cases to rule out the diagnosis of epidermolysis bullosa acquisita (EBA). Treatment was with either systemic steroids or ACTH in all three cases. Trauma-induced bullous pemphigoid should be included in the differential diagnosis of localized blistering eruptions.
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ranking = 1.4061737367396
keywords = epidermolysis bullosa acquisita, bullosa acquisita, epidermolysis bullosa, acquisita, epidermolysis, bullosa
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23/58. Subepidermal vesicular dermatosis and sensory peripheral neuropathy caused by pyridoxine abuse.

    A woman who had ingested 2 gm of pyridoxine (vitamin B6) daily for 2 years for menstrual water retention developed a subepidermal vesicular eruption on the dorsa of the hands and toes, as well as a sensory peripheral neuropathy. The cutaneous and neurologic manifestations subsided about 2 months after discontinuation of the pyridoxine. The possible relationship of subepidermal vesicular eruptions caused by pyridoxine abuse to epidermolysis bullosa acquisita is discussed.
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ranking = 1.4061737367396
keywords = epidermolysis bullosa acquisita, bullosa acquisita, epidermolysis bullosa, acquisita, epidermolysis, bullosa
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24/58. Pemphigoid and ulcerative colitis.

    We report eight patients with ulcerative colitis who have subsequently developed pemphigoid. Four patients were investigated to determine the pattern of pemphigoid antibody staining on whole and chemically split skin and oral mucosa to distinguish between pemphigoid and epidermolysis bullosa acquisita. The findings were suggestive of pemphigoid with a predominantly epidermal pattern. Three patients were studied for the presence of antibodies against the colon (which were absent). The relationship between pemphigoid and ulcerative colitis is discussed in relation to a case-control study.
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ranking = 1.4061737367396
keywords = epidermolysis bullosa acquisita, bullosa acquisita, epidermolysis bullosa, acquisita, epidermolysis, bullosa
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25/58. Epidermolysis bullosa aquisita with basal epidermal cytoplasmic antibodies.

    A 45-year-old woman with epidermolysis bullosa aquisita is presented. The clinical, histological, and immunopathological features were in keeping with the previous reports of this disease. The patient also had anti-basal cell cytoplasmic antibodies at a significant titer, which is considered an unusual finding associated with this disorder. Treatment with a moderate dose of corticosteroid was effective in controlling the bullous lesions.
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ranking = 0.27645144202236
keywords = epidermolysis bullosa, epidermolysis, bullosa
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26/58. Oral non-dystrophic bullous eruption mainly limited to the gingivae: a mechano bullous response. A variant of cicatricial mucous membrane pemphigoid?

    Fourteen patients with recurrent blistering entirely restricted to the mouth have been observed for up to 7 years. Their average age was 52 years, and there was a predilection for females (ratio 2-5:1). patients presented with thick-roofed blisters and denuded, red, boggy areas of mucosa. The area of maximal involvement with the labial gingiva, and whilst the blisters would arise spontaneously, mechanical trauma was the obvious provocative factor in all patients. Gentle friction on normal looking mucosa would produce a bulla which became blood filled. re-epithelialization usually occurred within 1-3 weeks, with no scarring. Biopsies showed subepithelial bullae, and direct immunofluorescence was positive in the basement membrane zone of 2 of the 5 cases examined. The most striking feature was the extreme fragility of the epithelial attachment to the underlying corium, as shown by a useful clinical test with a probe. In 10 patients, the condition gradually remitted and the probe test became difficult to perform. The term acquired oral non-dystrophic epidermolysis bullosa was considered for the diagnosis, although a forme fruste of cicatricial mucous membrane pemphigoid remained an alternative.
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ranking = 0.2
keywords = epidermolysis bullosa, epidermolysis, bullosa
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27/58. 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.
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ranking = 1.4061737367396
keywords = epidermolysis bullosa acquisita, bullosa acquisita, epidermolysis bullosa, acquisita, epidermolysis, bullosa
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28/58. Disseminated cicatricial pemphigoid in a child and in an adult. Ultrastructural diagnostic criteria and differential diagnosis with special reference to acquired epidermolysis bullosa.

    The first case of an infant affected with a rare, disseminated variant of benign cicatricial pemphigoid is described, showing the same ultrastructural features of initial blister formation as an adult patient. These consist in edematous changes within the superficial dermis caused by vesiculation or dissolution of cellular and noncellular connective tissue elements, coalescing into subepidermal blisters. Differential diagnosis excludes other nonhereditary bullous disorders because of the ultrastructure of the dermo-epidermal junction in nascent blisters and in perilesional skin. In spite of evident clinical, histological, and immunohistological similarities as well as controversial and confusing immunological studies, acquired epidermolysis bullosa can be clearly separated from our case by a diagnostic hallmark on the electron-microscopical level, i.e., band-like IgG depositions beneath the basal lamina. This is demonstrated in comparing the two cases of disseminated cicatricial pemphigoid with three patients suffering from acquired epidermolysis bullosa, thus providing evidence that disseminated cicatricial pemphigoid and acquired epidermolysis bullosa are two distinct nosologic entities.
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ranking = 1.4
keywords = epidermolysis bullosa, epidermolysis, bullosa
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29/58. ichthyosis bullosa of siemens: a unique type of epidermolytic hyperkeratosis.

    We report the second family of ichthyosis bullosa, an entity that was first described by Siemens in 1937 and since then has fallen into oblivion. Clinically, ichthyosis bullosa is characterized by blistering resembling epidermolysis bullosa simplex and by generalized, yet circumscribed dark gray hyperkeratoses covering mainly the arms and the legs. Lichenification and superficially denuded areas (mauserung) are further prominent features. histology disclosed intracorneal blister formation corresponding to the mauserung phenomenon and epidermolytic hyperkeratosis that was confined to the granular layer and to the uppermost layers of the prickle cells. On electron microscopic examination the keratinocytes of these layers displayed structural alterations of tonofilaments as usually observed in epidermolytic hyperkeratosis. Thus ichthyosis bullosa shares with bullous ichthyosiform erythroderma blistering and epidermolytic hyperkeratosis, but can be distinguished from this wellknown disease by the lack of erythroderma, by the mauserung phenomenon, by the confinement of acanthokeratolysis to the superficial layers of the epidermis, and by intracorneal blistering.
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ranking = 0.33379002353912
keywords = epidermolysis bullosa, epidermolysis, bullosa
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30/58. Autosomal recessive pachyonychia congenita.

    We report the second and third cases of pachyonychia congenita inherited as an autosomal recessive disorder. Our cases were unusual, with the fingernails showing a striking leukonychia and appearing clinically as Terry's nails. These patients were originally diagnosed as having epidermolysis bullosa simplex because of a history of a life-long blistering disorder. The clinical features and inheritance of pachyonychia congenita, as well as the reasons for the long delay in diagnosis of our cases, are discussed.
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ranking = 0.2
keywords = epidermolysis bullosa, epidermolysis, bullosa
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