Prurigo: review of its pathogenesis, diagnosis, and treatment.

Prurigo is a reactive, hyperplastic skin condition characterized by pruritic papules, plaques, and/or nodules. The temporal classification includes acute/subacute and chronic disease (≥ 6 weeks), with different clinical variants, synonymies, and underlying etiological factors. The immunology of chronic prurigo shows similarities with atopic dermatitis due to the involvement of IL-4 and IL-13, IL-22, and IL-31. Treatment includes antihistamines, topical steroids, dupilumab, and JAK inhibitors. Several conditions manifest clinically as prurigo-like lesions, and the correct clinical diagnosis must precede correct treatment. Furthermore, chronic prurigos represent a recalcitrant and distressing dermatosis, and at least 50% of these patients have atopic diathesis, the treatment of which may induce adverse effects, especially in the elderly. The quality of life is significantly compromised, and topical treatments are often unable to control symptoms and skin lesions. Systemic immunosuppressants, immunobiologicals, and JAK inhibitors, despite the cost and potential adverse effects, may be necessary to achieve clinical improvement and quality of life. This manuscript reviews the main types of prurigo, associated diseases, their immunological bases, diagnosis, and treatment.

Translation, cross-cultural adaptation and validation of the Quality of Life Evaluation in Epidermolysis Bullosa instrument in Brazilian Portuguese.

BACKGROUND: The Quality of Life Evaluation in Epidermolysis Bullosa (QoLEB) questionnaire was developed for use in English-speaking individuals. OBJECTIVES: The aims of this study were to translate the QoLEB into Brazilian Portuguese, to culturally adapt it, and to verify its reliability and validity. METHODS: The study followed the steps proposed by the World Health Organization, which include: translation; evaluation by a panel of experts and patients; back translation; and linguistic and cultural adaptation. All subjects were examined and assessed using the QoLEB and the Dermatology Life Quality Index (DLQI) or the Children's Dermatology Life Quality Index (CDLQI). Translation and cultural and linguistic adaptation were conducted by 10 patients, three translators, and the bilingual authors. RESULTS: The population was composed of 40 children (mean age: 8,15 years) and 17 adults (mean age: 33 years). Among the children, eight had epidermolysis bullosa (EB) simplex (EBS), one had junctional EB (JEB), and 31 had dystrophic EB (DEB). In the adult group, four of the subjects had EBS, one had JEB, and 12 had DEB. Mean ± standard deviation (SD) scores on the QoLEB in children were 10.60 ± 7.13 in EBS subjects, 9.71 ± 7.87 in children with dominant DEB (DDEB), and 14.25 ± 9.67 in children with recessive DEB (RDEB). Mean ± SD scores in adults were 12.50 ± 9.95 in EBS subjects, 12.00 ± 5.83 in DDEB subjects, and 20.20 ± 9.21 in RDEB subjects. The QoLEB-BP (Brazilian Portuguese) showed high internal consistency (Cronbach's α = 0.88) and high test-retest reliability (intraclass correlation coefficient: 0.70), confirming the internal consistency and reproducibility of this Portuguese version. There were significant correlations between QoLEB scores and both CDLQI (Pearson's r = 0.688, P < 0.002) and DLQI (Pearson's r = 0.807, P < 0.001) scores. CONCLUSIONS: Epidermolysis bullosa has marked impacts on the lives of EB patients and their families, which are strongly correlated with disease severity. The Brazilian Portuguese version of the QoLEB is validated and can be recommended for use in subsequent studies.

Clinical, dermoscopic and histopathological features of melanocytic nevi in dystrophic epidermolysis bullosa.

BACKGROUND: Epidermolysis bullosa (EB) nevi are acquired pigmented melanocytic lesions which may have clinical and dermoscopic features quite similar to those found in melanoma. More detailed information on this phenomenon is still lacking. OBJECTIVES: To evaluate clinical, dermoscopic, and histopathological features of melanocytic lesions in 13 patients with dystrophic EB (DEB). PATIENTS AND METHODS: Patients underwent clinical and dermoscopic evaluation. Suspicious lesions were excised and examined microscopically. RESULTS: There were 12 cases of recessive DEB and one of dominant DEB. Five patients were men; 8 were women; the ages ranged from 2 to 27 years old. All patients had at least 2 atypical melanocytic lesions. Two of the 5 biopsied patients showed an atypical nevus or lentigo on histopathological examination. CONCLUSIONS: We observed a high incidence of large and atypical melanocytic lesions in DEB patients. Although the exact explanation for this is still unclear, it seems that re-epithelization and the chronic inflammatory process may stimulate the proliferation of melanocytes, as well as the emergence of lesions with atypical clinical and dermoscopic features. As an unequivocal discrimination from malignant melanoma in vivo is not always possible, regular clinical follow-up and histopathological evaluation of suspicious lesions in EB patients are mandatory.