Background: Bullous pemphigoid (BP) is the most common autoimmune bullous disease and it primarily affects the elderly population. It typically presents with tense bullae and severe pruritus. Non-bullous pemphigoid is...Background: Bullous pemphigoid (BP) is the most common autoimmune bullous disease and it primarily affects the elderly population. It typically presents with tense bullae and severe pruritus. Non-bullous pemphigoid is a subtype of BP characterized by lacking the typical bullae formation with different presentations including erythematous, eczematous, urticarial, polycyclic, targetoid, nodular, lichenoid, vesicular and erythrodermic. Aim: to document a new case presentation of urticarial BP who was treated for several years as chronic urticarial and chronic eczema. Case Report: A 56-year-old male patient presented with a history of recurrent severely pruritic urticarial wheals for 4 years duration involving the lower abdomen, lower back, upper and lower extremities associated with excoriations, keratosis, and post-inflammatory hyperpigmentations on resolved sites, diagnosed as urticarial BP on histopathology & direct immunofluorescence study (DIF) and was improved on systemic doxycycline therapy. Conclusion: BP can be presented with atypical manifestation. Urticarial BP is a rare variant of non-bullous pemphigoid that should be taken into consideration in the differential diagnosis of an atypical urticarial wheal not responding to conventional therapy.展开更多
Dermatological conditions are intimately related to stress. There was a great interest in this field in the last years. Stress could be involved as a trigger factor for a lot of cutaneous diseases: alopecia areata, ps...Dermatological conditions are intimately related to stress. There was a great interest in this field in the last years. Stress could be involved as a trigger factor for a lot of cutaneous diseases: alopecia areata, psoriasis, vitiligo, lichen planus, acne, atopic dermatitis, urticaria. For other conditions: seborrheic dermatitis, hyperhydrosis, herpes, pemphigus, a.s.o, there are anecdotal notices. On the other hand, the skin disease itself could induce a secondary stress for the patient, influencing his quality of life. The stress per se is less important than the "perceived stress", the patient's perception of the stressful situation. This perception could be influenced by the psychological state of the patient. Anxiety, depression could change the perception of the event. It is important to take care of these aspects during the consultation. A good cooperation with psychiatrist or/and psychologist could improve the results, besides the specific therapy.展开更多
Objective:To analyze the clinical treatment effect of traditional Chinese medicine five-color therapy on chronic urticaria in children.Methods:The income data target of this article is 80 children with chronic urticar...Objective:To analyze the clinical treatment effect of traditional Chinese medicine five-color therapy on chronic urticaria in children.Methods:The income data target of this article is 80 children with chronic urticaria.The grouping method is a randomized method with 40 children in each group.The experimental group was treated with five-color treatment of traditional Chinese medicine,and the control group was treated with western medicine.The incidence,treatment and recurrence of adverse reactions in children with chronic urticaria were compared between the two groups.Results:Showed total effective rate of children with chronic urticaria in the experimental group was compared with the control group,P<0.05,the data showed statistical significance.Conclusion:Stated use of TCM five-color therapy in the treatment of children with chronic urticaria can significantly improve safety.展开更多
Schnitzler syndrome is a rare disease of adult-onset with main features including chronic urticarial rash, recurrent fever, arthralgia or arthritis, monoclonal gammopathy of undetermined significance (MGUS), and marke...Schnitzler syndrome is a rare disease of adult-onset with main features including chronic urticarial rash, recurrent fever, arthralgia or arthritis, monoclonal gammopathy of undetermined significance (MGUS), and marked systemic inflammation. Schnitzler syndrome is often underdiagnosed. Patients with Schnitzler syndrome may present to dermatologists and allergists for urticaria, hematologists for MGUS, or rheumatologists for arthritis. It is important to recognize Schnitzler syndrome for its remarkable response to interleukin (IL)-1 blockade. Besides, many cases of Schnitzler-like syndromes do not meet the diagnostic criteria of classical Schnitzler syndrome but display excellent response to IL-1 inhibitors. The overly produced IL-1 is the result of a somatic mosaic gain of function mutation ofNLRP3 (nucleotide-binding oligomerization domain [NOD]-like receptor [NLR] family pyrin domain containing 3) gene in some patients with Schnitzler-like syndromes. Inflammasome activation is evident in patients with classical Schnitzler syndrome although noNLRP3 gene mutation is identified. Collectively, Schnitzler syndrome and Schnitzler-like syndromes represent a spectrum of IL-1 mediated adult-onset autoinflammatory diseases.展开更多
文摘Background: Bullous pemphigoid (BP) is the most common autoimmune bullous disease and it primarily affects the elderly population. It typically presents with tense bullae and severe pruritus. Non-bullous pemphigoid is a subtype of BP characterized by lacking the typical bullae formation with different presentations including erythematous, eczematous, urticarial, polycyclic, targetoid, nodular, lichenoid, vesicular and erythrodermic. Aim: to document a new case presentation of urticarial BP who was treated for several years as chronic urticarial and chronic eczema. Case Report: A 56-year-old male patient presented with a history of recurrent severely pruritic urticarial wheals for 4 years duration involving the lower abdomen, lower back, upper and lower extremities associated with excoriations, keratosis, and post-inflammatory hyperpigmentations on resolved sites, diagnosed as urticarial BP on histopathology & direct immunofluorescence study (DIF) and was improved on systemic doxycycline therapy. Conclusion: BP can be presented with atypical manifestation. Urticarial BP is a rare variant of non-bullous pemphigoid that should be taken into consideration in the differential diagnosis of an atypical urticarial wheal not responding to conventional therapy.
文摘Dermatological conditions are intimately related to stress. There was a great interest in this field in the last years. Stress could be involved as a trigger factor for a lot of cutaneous diseases: alopecia areata, psoriasis, vitiligo, lichen planus, acne, atopic dermatitis, urticaria. For other conditions: seborrheic dermatitis, hyperhydrosis, herpes, pemphigus, a.s.o, there are anecdotal notices. On the other hand, the skin disease itself could induce a secondary stress for the patient, influencing his quality of life. The stress per se is less important than the "perceived stress", the patient's perception of the stressful situation. This perception could be influenced by the psychological state of the patient. Anxiety, depression could change the perception of the event. It is important to take care of these aspects during the consultation. A good cooperation with psychiatrist or/and psychologist could improve the results, besides the specific therapy.
文摘Objective:To analyze the clinical treatment effect of traditional Chinese medicine five-color therapy on chronic urticaria in children.Methods:The income data target of this article is 80 children with chronic urticaria.The grouping method is a randomized method with 40 children in each group.The experimental group was treated with five-color treatment of traditional Chinese medicine,and the control group was treated with western medicine.The incidence,treatment and recurrence of adverse reactions in children with chronic urticaria were compared between the two groups.Results:Showed total effective rate of children with chronic urticaria in the experimental group was compared with the control group,P<0.05,the data showed statistical significance.Conclusion:Stated use of TCM five-color therapy in the treatment of children with chronic urticaria can significantly improve safety.
基金Dr. Chu’s work was supported by the Innovative Award from the American College of Rheumatology Research Foundation and the VA Merit Review grant(No. I01BX005195)。
文摘Schnitzler syndrome is a rare disease of adult-onset with main features including chronic urticarial rash, recurrent fever, arthralgia or arthritis, monoclonal gammopathy of undetermined significance (MGUS), and marked systemic inflammation. Schnitzler syndrome is often underdiagnosed. Patients with Schnitzler syndrome may present to dermatologists and allergists for urticaria, hematologists for MGUS, or rheumatologists for arthritis. It is important to recognize Schnitzler syndrome for its remarkable response to interleukin (IL)-1 blockade. Besides, many cases of Schnitzler-like syndromes do not meet the diagnostic criteria of classical Schnitzler syndrome but display excellent response to IL-1 inhibitors. The overly produced IL-1 is the result of a somatic mosaic gain of function mutation ofNLRP3 (nucleotide-binding oligomerization domain [NOD]-like receptor [NLR] family pyrin domain containing 3) gene in some patients with Schnitzler-like syndromes. Inflammasome activation is evident in patients with classical Schnitzler syndrome although noNLRP3 gene mutation is identified. Collectively, Schnitzler syndrome and Schnitzler-like syndromes represent a spectrum of IL-1 mediated adult-onset autoinflammatory diseases.
