1/4. Ophthalmic manifestations of neonatal onset multisystem inflammatory disease.PURPOSE: To report the ophthalmic manifestations of neonatal onset multisystem inflammatory disease, a recently recognized, rare systemic disorder characterized by the triad of arthropathy, rash, and abnormal central nervous system development. METHOD: Case report. RESULTS: A 2-year-old female with neonatal onset multisystem inflammatory disease presented with visual acuity of fix and follow with each eye, bilateral optic nerve head pallor and gliosis, as well as marked sheathing of the peripapillary vessels. No vitreous inflammation or macular edema was found. visual acuity was stable from the neonatal period through the 3 months of follow-up after the changes involving the optic nerve heads and peripapillary vessels were observed (a total of 33 months). CONCLUSIONS: This report provides a description of the ocular manifestations of neonatal onset multisystem inflammatory disease and supports earlier suggestions that swelling of the optic nerve heads can occur. In this case, optic nerve head pallor may have been a sequela of such swelling. The pathogenesis of neonatal onset multisystem inflammatory disease is unknown.- - - - - - - - - - ranking = 1keywords = onset multisystem inflammatory disease, neonatal onset multisystem inflammatory disease, multisystem inflammatory disease, onset multisystem, neonatal onset multisystem, neonatal onset, inflammatory disease, multisystem (Clic here for more details about this article) |
2/4. Perineal hidradenitis suppurativa: presentation of two unusual complications and a review.Two patients with advanced perineal hidradenitis suppurativa, complicated by fecal incontinence and squamous cell carcinoma, are presented. The first patient was a 58-year-old man who had a 30-year history of chronic recurring perianal abscesses and perineal sinuses. At the time of presentation, he had extensive perineal suppurative disease, and scarring and fixation of the anal sphincters with resultant fecal incontinence. He was treated with wide excision and skin graft closure. The second patient was a 27-year-old man with an 11-year history of recurrent gluteal abscesses and perineal sinuses. At the time of presentation, his inflammatory disease was only mildly active, but he had a nonhealing gluteal lesion. The nonhealing lesion was diagnosed as a squamous cell carcinoma and was managed with wide excision and primary closure. The inflammatory disease was excised and grafted. Complications of advanced hidradenitis suppurativa can be debilitating and life threatening. We review the etiology, pathophysiology, complications, and treatment options of hidradenitis suppurativa, including a literature review of the association with malignancy. We propose that the incidence of disabilities and complications may be reduced by early diagnosis and treatment, by emphasis on prevention of recurrence, and by more aggressive surgical intervention for recurrent and extensive disease.- - - - - - - - - - ranking = 0.00010474866123069keywords = inflammatory disease (Clic here for more details about this article) |
3/4. Neonatal onset multisystem inflammatory disease.Neonatal onset multisystem inflammatory disease (NOMID) is a rare disorder involving a triad of arthropathy, rash, and central nervous system (CNS) involvement. We describe a girl with NOMID who presented with typical neonatal rash, arthropathy, fever, and failure to thrive, but has not developed evidence of ocular or CNS involvement. This case illustrates the spectrum of involvement seen in NOMID. Histopathology of the skin demonstrated neutrophilic eccrine hidradenitis, a unique finding, which may serve as a diagnostic clue in patients with this rare disorder.- - - - - - - - - - ranking = 0.4693816381568keywords = onset multisystem inflammatory disease, multisystem inflammatory disease, onset multisystem, inflammatory disease, multisystem (Clic here for more details about this article) |
4/4. Neutrophilic eccrine hidradenitis simulating orbital cellulitis.Orbital swelling in patients with cancer can reflect neoplastic or infectious processes. Accurate diagnosis can be especially difficult in the face of associated fever and neutropenia. We treated a 30-year-old man undergoing induction chemotherapy for acute myelogenous leukemia, who had fever of unknown origin and periorbital swelling suggestive of orbital cellulitis. However, the periorbital findings were more compatible with passive swelling and hemorrhage. A skin biopsy specimen demonstrated isolated neutrophilic inflammation and necrosis of the eccrine glands. Cultures of the tissue for bacteria and fungi were negative. Pertinent literature regarding eccrine-gland inflammatory disease was reviewed. This unusual entity, termed neutrophilic eccrine hidradenitis, is most common in patients undergoing induction chemotherapy. Cases with infectious causes and cases in neutropenic patients have also been reported. No other patients, to our knowledge, with periocular involvement by neutrophilic eccrine hidradenitis have been described. Neutrophilic eccrine hidradenitis should be added to the differential diagnosis of cases of periocular hemorrhage and swelling in patients with cancer who receive chemotherapy.- - - - - - - - - - ranking = 5.2374330615346E-5keywords = inflammatory disease (Clic here for more details about this article) |