RESUMO
BACKGROUND: Gingival lesions in patients with dystrophic epidermolysis bullosa (DEB) are a common manifestation. However, their clinical features, frequency and severity are currently unknown. METHODS: Forty-five DEB patients were assessed by an oral medicine specialist, who analysed the presence/absence of four clinical signs (erythema, erosion/ulcer, atrophy, blister) on free and attached gingiva, using the Epidermolysis Bullosa Oropharyngeal Severity score. RESULTS: Twenty-eight (62.2%) out of 45 DEB patients showed different types of gingival lesions, whose presence/absence and total frequency/distribution were not significantly different between males and females (p=0.087 and p=0.091, respectively). Erythema was the most prevalent lesion (66.2%) and the recessive DEB severe generalized (RDEB-sev gen) reached the highest median disease activity score. A significant correlation was observed between the DEB subtypes and the disease activity median score (p<0.001), but not between age and total disease activity score in each group of DEB (p>0.05). Lastly, logistic regression showed that only gender (p=0.031) and RDEB-sev gen (p=0.001) were risks factors for the presence of gingival lesions. CONCLUSIONS: Gingival lesions in DEB patients are a relatively common entity and may have multiple clinical aspects, emphasizing the need for thorough attention and awareness among dentists.
Assuntos
Epidermólise Bolhosa Distrófica/patologia , Doenças da Gengiva/patologia , Adolescente , Adulto , Vesícula/patologia , Criança , Pré-Escolar , Estudos Transversais , Epidermólise Bolhosa Distrófica/classificação , Eritema/patologia , Feminino , Doenças da Gengiva/classificação , Humanos , Lactente , Masculino , Pessoa de Meia-Idade , Úlceras Orais/patologia , Atrofia Periodontal/patologia , Prevalência , Fatores de Risco , Fatores Sexuais , Adulto JovemRESUMO
Pasini's albopapuloid epidermolysis bullosa is a very rare subtype of generalized dystrophic dominant epidermolyis bullosa. A 30 year-old white female patient presented since her childhood disseminated small blisters and papules. Light microscopy of a blister showed dermal-epidermal cleavage; moreover, focal areas of dermal-epidermal splitting were also observed. Transmission electron microscopy also identified focal areas of cleavage, which were seen below the lamina densa. It is important to recognize this condition as a variant of epidermolysis bullosa, since the most important cutaneous findings are generalized papules and not blisters and erosions as in other forms of epidermolysis bullosa.
Assuntos
Epidermólise Bolhosa Distrófica/patologia , Adulto , Epidermólise Bolhosa Distrófica/classificação , Feminino , Humanos , Microscopia Eletrônica de TransmissãoRESUMO
Pasini's albopapuloid epidermolysis bullosa is a very rare subtype of generalized dystrophic dominant epidermolyis bullosa. A 30 year-old white female patient presented since her childhood disseminated small blisters and papules. Light microscopy of a blister showed dermal-epidermal cleavage; moreover, focal areas of dermal-epidermal splitting were also observed. Transmission electron microscopy also identified focal areas of cleavage, which were seen below the lamina densa. It is important to recognize this condition as a variant of epidermolysis bullosa, since the most important cutaneous findings are generalized papules and not blisters and erosions as in other forms of epidermolysis bullosa.
A epidermólise bolhosa albo-papulóide de Pasini é uma variante rara da forma generalizada de epidermólise bolhosa distrófica dominante. Uma paciente de 30 anos apresenta desde a infância pápulas e bolhas disseminadas. A microscopia óptica de uma bolha demonstrou clivagem dermo-epidérmica; além disso áreas focais de desprendimento dermo-epidérmico foram encontradas. A microscopia eletrônica de transmissão identificou a clivagem abaixo da lâmina densa. É importante que se reconheça essa variante de epidermólise bolhosa, já que o aspecto clínico predominante são pápulas disseminadas e não bolhas como nas outras formas de epidermólise bolhosa.
Assuntos
Adulto , Feminino , Humanos , Epidermólise Bolhosa Distrófica/patologia , Epidermólise Bolhosa Distrófica/classificação , Microscopia Eletrônica de TransmissãoRESUMO
Esophageal strictures may complice Dystrophic Recessive + Epidermolysis Bullosa (DREB). We report six consecutive cases of DREB with this type of lesion as the main gastrointestinal manifestation. In three children the stenosis was unique and located in the upper third one in the middle third, and the last two children showed a double stenosis (Upper and middle third) of the esophagus.