ABSTRACT
Introduction@#Bullous pemphigoid (BP) is an acquired autoimmune subepidermal bullous disease characterized by linear depo- sition of IgG and C3 along the basement membrane. It rarely occurs in childhood, especially in adolescence, with only 14 cases identified in literature. Treatment of choice is systemic corticosteroids but other treatment options such as anti-inflammatory antibacterials and methotrexate are available.@*Case report@#A 16-year-old Filipino girl presented with a three-month history of generalized vesicles and bullae. Nikolsky and Asboe-Hansen signs were negative. Histopathology and direct immunofluorescence were consistent with BP. ELISA to BP180 au- toantibody levels was elevated at 135 IU (normal <9 IU). Complete blood count showed leukocytosis with increase in neutrophils. Chest x-ray revealed pulmonary tuberculosis. The patient was given quadruple anti-Koch’s medication (pyrazinamide, rifampi- cin, ethambutol, isoniazid), prednisone, oral erythromycin and topical clobetasol propionate. Complete remission was attained at 10 months and is sustained at the time of writing.@*Conclusion@#To establish a definitive diagnosis and appropriate management, BP requires clinical, histopathologic, and immuno- logical correlation. Childhood BP has good prognosis and rapid treatment response, with rare relapses.
Subject(s)
Pemphigoid, BullousABSTRACT
Background: Psoriasis vulgaris is an infl ammatory skin condition characterized by dramatic biochemical and immunological changes. Aims: The aim of the study was to evaluate antimicrobial response, tissue degeneration reactions and distribution of infl ammatory cytokines in untreated psoriatic skin as well as the correlations between these factors and infl uence on the course of the disease. Methods: We evaluated skin samples obtained from routine punch biopsies in 40 patients with psoriasis vulgaris. All tissue specimens were examined by hematoxylin and eosin staining and immunohistochemistry for human beta defensin 2 (HBD-2), matrix metalloproteinase 2 (MMP-2), tumor necrosis factor-alpha (TNF-alpha), interleukin 6 (IL-6) and IL-8. The staining intensity was semi-quantitatively graded. Results: Numerous keratinocytes, fibroblasts and macrophages expressed HBD-2 while the number of MMP-2-positive macrophages, fi broblasts and epitheliocytes varied. TNF-alpha-positive cells varied from a few to numerous in each microscopic fi eld. IL-6-positive cells varied from a few to abundant and IL-8-positive cells from numerous to abundant in each fi eld. Limitations: This study had a rather small patient number. Conclusions: Psoriatic skin shows a strong correlative increase in skin antimicrobial proteins and enzymes mediating tissue degeneration suggesting that the skin maintains compensatory mechanisms during persistent remodeling. While individual notable decrease in antimicrobial proteins was observed in some tissue samples, generally the increased human beta defensin associated with psoriasis is likely to be due to an altered immune status. TNF-alpha, IL-6 and IL-8 are common cytokines expressed in psoriatic skin plaques to maintain the infl ammatory cycle. HBD-2, MMP-2 and TNF-alpha positively correlate with the severity of psoriasis. Meanwhile, the expression of IL-8 signifi cantly decreases with clinically more severe psoriasis, perhaps making these factors candidate prognostic factors for psoriatic inflammation.