ABSTRACT
Bullous pemphigoid (BP) and psoriasis are both immune-related skin diseases. Still, the comorbidities between the two are rare, and there is no consensus on the optimal treatment strategy for BP combined with psoriasis. JAK inhibitors are emerging, molecularly targeted therapeutic agents that target the molecule of Janus kinase, a signal transducer and activator of transcription (JAK/STAT). JAK inhibitors block intracellular signaling pathways by blocking the gene transcription of key pro-inflammatory cytokines that play a central role in the pathogenesis of many inflammatory and autoimmune diseases. Tofacitinib is a first-generation JAK inhibitor. The purpose of this article is to describe the first report of the use of tofacitinib in treating BP combined with psoriasis vulgaris with significant results. According to our findings, tofacitinib may be a safe and effective treatment option for patients suffering from BP and psoriasis together. The implications of this are substantial for the guidance of treatment strategies for both comorbid conditions.
Subject(s)
Janus Kinase Inhibitors , Pemphigoid, Bullous , Psoriasis , Humans , Janus Kinase Inhibitors/therapeutic use , Pemphigoid, Bullous/drug therapy , Protein Kinase Inhibitors/therapeutic use , Protein Kinase Inhibitors/pharmacology , Psoriasis/drug therapy , Janus Kinases/metabolism , STAT Transcription Factors/metabolismSubject(s)
Exanthema , Psoriasis , Humans , East Asian People , Psoriasis/drug therapy , Antibodies, Monoclonal, HumanizedABSTRACT
Common autoimmune bullous diseases (AIBDs) include pemphigus and bullous pemphigoid (BP), which are primarily caused by IgG autoantibodies against the structural proteins of desmosomes at the cell-cell junction and hemidesmosomes at the epidermal-dermal junction. Few studies have assessed nail changes in patients with pemphigus or BP. In the present study, we collected the clinical data of 191 patients with AIBDs (108 patients with pemphigus and 83 patients with BP) and 200 control subjects. Nail changes were observed in 77.0% (147/191), 77.8% (84/108), and 75.9% (63/83) of patients with AIBDs, pemphigus, and BP, respectively, and 14.5% (29/200) of control subjects. Beau's lines and paronychia were the most common nail involvement, observed in 22.5% (43/191) and 22.5% (43/191) of patients with AIBDs, 25.0% (27/108) and 25.9% (28/108) of patients with pemphigus, 19.3% (16/83) and 18.1% (15/83) of patients with BP, respectively. The autoimmune bullous skin disorder intensity score (ABSIS) and the onset time of patients with pemphigus or BP with nail changes were different. Onychomycosis accounted for 21.5% (41/191) of all patients with AIBDs. The ABSIS was correlated with nail involvement in patients with BP (r = 0.46, p < 0.001), and weakly correlated with nail involvement in patients with AIBDs (r = 0.37, p < 0.001), pemphigus (r = 0.29, p = 0.009), and pemphigus vulgaris (PV; r = 0.35, p = 0.008). No correlation was observed between nail involvement and disease antibody titers. In conclusion, nail changes are frequently observed in patients with pemphigus and BP. The type and onset time of nail changes may indicate the severity of pemphigus and BP, which warrants the attention of dermatologists.
ABSTRACT
BACKGROUND: Generalized pustular psoriasis (GPP) is a rare type of psoriasis with potentially life-threatening implications. Mutations in IL36RN gene have been suggested to be causative or predisposing factors for GPP. OBJECTIVE: To evaluate the genetic heterogeneity of GPP, PV and GPP alone, GPP with PV. METHODS: We performed a sanger sequencing identify IL36RN mutations in 62 Chinese Han patients with sporadic GPP, including 17 GPP without psoriasis vulgaris (PV) (GPP alone) cases vs. 45 GPP with preceding, later or accompanied by PV (GPP with PV) cases; 16 patients with pediatric-onset GPP (PGPP) vs. 46 adult-onset GPP (AGPP). We included 96 healthy controls and 174 sporadic patients with PV. RESUTS: We found 2 new variants and 4 known IL36RN variants in 29 GPP patients, 18 individuals carried recessive (homozygous/compound heterozygous) alleles and 11 cases harbored a single heterozygous change. Twelve PV patients and six controls harbored a single heterozygous for three out of the six variants. Significant differences were observed between GPP and PV groups, GPP alone and GPP with PV groups when compared frequencies of IL36RN variants, but we did not found association between PGPP and AGPP groups. CONCLUSION: Our study provided more evidence that GPP and PV are distinct subtypes of psoriasis caused by different pathogenesis, and GPP alone could be regarded as an especial entities of GPP which is different from GPP with PV on the etiology.
Subject(s)
Interleukins/genetics , Mutation , Psoriasis/genetics , Adolescent , Adult , Animals , Asian People/genetics , Cats , Child , Child, Preschool , DNA Mutational Analysis , Female , Humans , Male , Middle AgedABSTRACT
Epidermolysis bullosa acquisita (EBA) is a rare, acquired, subepidermal blistering disease characterized by autoantibodies directed against type VII collagen, the major component of anchoring fibrils. We report a 5-year-old Chinese boy who presented with extensive lesions consisting of disseminated pruritic vesicles and tense blisters. The diagnosis of EBA was confirmed by histopathology, immunofluorescence, and immunoblotting analysis. The disease was controlled with a combination of prednisone and dapsone.
Subject(s)
Epidermolysis Bullosa Acquisita/diagnosis , Asian People , Autoantibodies/blood , Autoantibodies/immunology , Basement Membrane/immunology , Child, Preschool , Collagen Type VII/immunology , Dapsone/therapeutic use , Dermatologic Agents/therapeutic use , Drug Therapy, Combination , Epidermolysis Bullosa Acquisita/drug therapy , Epidermolysis Bullosa Acquisita/pathology , Fluorescent Antibody Technique , Humans , Immunoblotting , Male , Prednisone/therapeutic use , Treatment OutcomeABSTRACT
BACKGROUND: Disaster Medicine training is not included in medical education curriculum in China, even though the country has suffered various disasters annually. We intended to assess the need for continual education regarding disaster management for health professionals in China. METHODS: A survey was conducted among 324 health professionals who participated in the response to the Wenchuan earthquake medical relief and public health assessment in October, 2008. RESULTS: The most of participants (67.3%) received informal disaster medicine training, and only a few (12.7%) participated in disaster drills. Most of the participants wanted to get continual education about disaster medicine training (89.8%), but prefer on-line training course for the flexibility of time scheduling and travel through China. CONCLUSION: The need for continual disaster medicine training is high; health professionals should be equipped with knowledge and skills for disaster management.
