Rapid remission of refractory synovitis, acne, pustulosis, hyperostosis, and osteitis syndrome in response to the Janus kinase inhibitor tofacitinib: A case report

BACKGROUND Synovitis,acne,pustulosis,hyperostosis,and osteitis(SAPHO)syndrome is a rare autoinflammatory disease for which clinical treatment has not been standardized.Janus kinase(JAK)inhibitors represent a novel therapeutic option for rheumatoid arthritis,psoriatic arthritis,and some other autoinflammatory diseases.However,the clinical utility of JAK inhibitors in treating SAPHO syndrome has not been thoroughly investigated.In this study,we describe a patient with SAPHO syndrome who failed to respond to conventional treatment but demonstrated a remarkable and rapid response to the JAK inhibitor tofacitinib.CASE SUMMARY A 62-year-old female patient presented with swelling and pain at the sternoclavicular joints,back pain that limited her activities,arthralgia in the right knee,and cutaneous lesions.Her symptoms were unresponsive to nonsteroidal anti-inflammatory drugs,disease-modifying antirheumatic drugs,Tripterygium wilfordii hook f,and bisphosphonates.SAPHO syndrome was diagnosed in accordance with dermatological and osteoarticular manifestations and abnormal inflammatory factors.Multiple image studies have illustrated bone lesions and pathological fractures of vertebral bodies.Oral treatment with tofacitinib at 5 mg twice daily with methotrexate and bisphosphonates was initiated.The patient reported that her pain symptoms were relieved after 3 d and her cutaneous lesions were reduced after 4 wk of treatment.Vertebral lesions were improved after 6 mo on tofacitinib.No serious adverse effects were noted.CONCLUSION JAK inhibitor therapy may be a promising strategy to treat SAPHO syndrome.

A case of primary biliary cirrhosis complicated by Behcet's disease and palmoplantar pustulosis

A 46-year-old woman was diagnosed with palmoplantar pustulosis （PPP） at the Department of Dermatology, Fukushima Medical University Hospital in 2000, and was treated with ointment. However, because liver dysfunction developed in 2003, she was referred to our department, where primary biliary cirrhosis （PBC） was also diagnosed on the basis of clinical findings. One year later, at the age of 49, she developed manifestations of Behcet＇s disease （BD）, including erythema nodosum in the lower extremities. Because she had a history of uveitis, recurrent oral ulceration was present, and the HLA typing was positive for B51, BD was additionally diagnosed. Liver function normalized within three months of the start of treatment with ursodesoxycholic acid （UDCA）. This is the first case of PBC associated with BD and PPP.

Acute generalized exanthematous pustulosis with airway mucosa involvement:A case report

BACKGROUND Acute generalized exanthematous pustulosis(AGEP)is a severe cutaneous adverse reaction characterized by sterile pustules on erythematous skin associated with fever and leukocytosis.The annual incidence of AGEP is estimated to be 1-5 cases per million.Cases of AGEP with oral mucosa involvement have been reported.However,reports of AGEP involving airway mucosa are limited.CASE SUMMARY We report a 42-year-old woman with serious AGEP involving the airway mucosa.The patient initially developed fever and a small rash on her forehead and face.Over the next 2 d,she developed a diffuse,pustular rash over her trunk and legs.In addition,she complained of a cough with white foam-like sputum,chest tightness and dyspnea.Four days later,due to dyspnea,her mental status started to gradually deteriorate.She became more and more drowsy.Biopsies of the skin and airway mucosa suggested the diagnosis of AGEP.According to the European study of severe cutaneous adverse reactions group’s scoring system,the patient scored+6 indicating a probable diagnosis of AGEP.She received intravenous methylprednisolone 120 mg/12 h for 3 d,and was eventually discharged in good condition.This patient had already experienced respiratory failure and airway mucosa involvement on admission;however,the clinicians had an insufficient understanding of AGEP.Glucocorticoid was administered for more than 10 d following onset of the disease,and her overall prognosis was satisfactory.CONCLUSION This case represents a rare clinical feature of AGEP and an important finding for clinicians.

Acute Generalized Exanthematous Pustulosis during Puerperal Period Induced by Azithromycin: Case Report

Background: The use of antibiotics, especially beta-lactams and macrolides, may be associated with dermatopathies, such as Acute Generalized Exanthematous Pustulosis (AGEP), which is an uncommon cutaneous adverse reaction. Case: We report a case of AGEP, in a 36-year-old multiparous (G5P3C1A1) woman, with 38 weeks of gestation, admitted to the hospital to induce labor. Due to cephalopelvic disproportion, the cesarean section was indicated. In the postoperative period, the patient evolved with cutaneous rash, accompanied by productive cough and dyspnea. Because it was a fever of obscure origin, the treatment with antibiotics, including azithromycin, was initiated. On subsequent days, she presented pustules on the back, abdomen and extremities. Such reaction was attributed to the use of azithromycin. When the drug was discontinued, the lesions regressed significantly. Conclusion: The clinical picture of AGEP may occur with persistent high fever and therefore could be confused with systemic infections, consequently, being treated with wrong medications capable of aggravating the adverse cutaneous reaction, worsening the course of the disease that could be easily treated by stopping the use of the causative drug. This case shows the importance of including AGEP as a differential diagnosis of dermatopathies in the pregnancy-puerperal cycle, especially in women who are using various medications, including antibiotics.