Lupus pernio (LP) is a chronic non-life threatining type of cutaneous sarcoidosis that can be related to chronic fibrotic sarcoidosis, hyperglobulinemia and hypercalcemia. The aim of this case report is to evaluate the clinical and demographic features of cutaneous sarcoidosis mainly presenting with a rare manifestation of LP. In this paper we report a case of systemic sarcoidosis presenting with LP and a review of the available literature. LP is a rare presentation with infiltrated erythematoviolaceous plaques affecting the nose. We address the main management approach, and possible association with an underlying systemic sarcoidosis. LP is a rare but chronic manifestation of systemic sarcoidosis that needs to be treated in order to prevent cosmetic defects and psychological effects. It is important to recognize such a condition early in order to avoid a delay in treatment and worsening of the condition, both physically and psychologically. Further research regarding the diagnostic approach and management is required to understand this condition thoroughly.
PurposeThe aim of this study was to evaluate the progression of a case of a patient with epidermolysis bullosa (EB) since early age who survived to adulthood, presenting with recurrent skin blisters and disfiguring scars and disabling musculoskeletal deformities.BackgroundEB is a rare group of inherited diseases that affect the skin fragility causing it to blister in response to even minor trauma. Established novel treatments are limited in the literature due to its rarity, and more research is needed to set a global management approach. Clinical manifestations range widely from localized to generalized blistering.MethodsA rare case of EB surviving to adulthood despite the complications, which has been evaluated, treated during a relapse, and followed up.ConclusionThe described case is of considerable clinical interest due to its rarity and severity. Optimal management requires a multidisciplinary approach and revolves around the protection of the skin against slightest injury, use of careful wound care dressings, aggressive nutritional support, and early medical or surgical interventions if needed to manage any complications. Prognosis varies considerably depending on each case.
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