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Hematopoietic Stem Cell Transplantation in Patients with Autoimmune Bullous Skin Disorders
Published in Richard K. Burt, Alberto M. Marmont, Stem Cell Therapy for Autoimmune Disease, 2019
Cicatricial pemphigoid has recurring blisters on mucous membranes or on skin near orifices (mouth, oropharynx, nasopharynx, esophagus, genitals, and conjunctiva).1 The loss of function due to scarring and adhesions often necessitates surgical interventions. Genital areas, conjunctivae, larynx, pharynx and esophagus involvement causes significant morbidity. Brunsting and Perry described a variant of CP characterized by deep skin bullous lesions involving the head and neck that heal with scars.13
Sulfonamides
Published in M. Lindsay Grayson, Sara E. Cosgrove, Suzanne M. Crowe, M. Lindsay Grayson, William Hope, James S. McCarthy, John Mills, Johan W. Mouton, David L. Paterson, Kucers’ The Use of Antibiotics, 2017
Natasha E. Holmes, M. Lindsay Grayson
Ocular cicatricial pemphigoid is a severe, sight-threatening systemic disease that sometimes requires systemic immunosuppression. Sulfapyridine, 50 mg twice daily, has been used successfully to reduce moderate to marked inflammation without significant adverse effects (Elder et al., 1996). Saw et al. (2008) demonstrated that sulfonamides alone were not as efficacious as immunosuppressive therapy for ocular pemphigoid. However, outcomes were improved if used in combination with immunosuppressants such as cyclophosphamide or mycophenolate. Oral sulfasalazine with pentoxifylline has been shown to be an effective treatment in young patients with pemphigus (Dogra et al., 2015) and in psoriasis (el-Mofty et al., 2011), and topical sulfasalazine has been used for steroid-unresponsive oral lichen planus (Jeong et al., 2015; Omidian et al., 2010).
Dermatology
Published in Fazal-I-Akbar Danish, Essential Lists of Differential Diagnoses for MRCP with diagnostic hints, 2017
Alopecia – scarring (often patchy):1 DLE.2 Lichen planus (LP).3 Cicatricial pemphigoid (CP).4 Sarcoidosis.5 Infections (including fungal kerion)6 Tumours (e.g. basal cell carcinoma).7 Radiation/burns.8 Traction (late stages).
Systemic Immunosuppression in Cornea and Ocular Surface Disorders: A Ready Reckoner for Ophthalmologists
Published in Seminars in Ophthalmology, 2022
The presence of active disease with significant ocular inflammation at presentation requires intravenous methyl prednisolone either in isolation or with intravenous cyclophosphamide (Figure 5). This is followed by a short course of oral steroids in tapering doses over 6–8 weeks with oral azathioprine or mycophenolate mofetil. The latter medications can be added in a step-wise manner, starting with MTX or azathioprine and then adding the second immunosuppressive medication if no response is noted after 1–2 months. Oral cyclophosphamide though effective is not preferred in view of the side effect profile that occurs with prolonged usage.105,108,109 The use of intravenous immunoglobulins and biologics is reserved as the last step in this management protocol. Freemont et al. reported a 100% response rate with both mycophenolate and intravenous immunoglobulin in their retrospective review of 34 eyes with cicatricial pemphigoid.110 The use of newer biological agents such as rituximab and infliximab have also been reported to have good results however the expense involved in the long term use of these medications restricts their widespread adoption.111,112
Reliability of Conjunctival Biopsy for Diagnosis of Ocular Mucous Membrane Pemphigoid: Redetermination of the Standard for Diagnosis and Outcomes of Previously Biopsy-Negative Patients
Published in Ocular Immunology and Inflammation, 2021
Stephen D. Anesi, Laura Eggenschwiler, Mariantonia Ferrara, Pichaporn Artornsombudh, Marisa Walsh, C. Stephen Foster
Ocular cicatricial pemphigoid, more recently termed ocular mucous membrane pemphigoid (oMMP), is a subset of mucous membrane pemphigoid (MMP), a group of systemic autoimmune diseases characterized by similar phenotype with subepithelial mucosal blistering, erosions, and scarring, potentially involving nasal, oral, and esophageal mucosa, epiglottis, larynx, urethra, vagina, anus, and/or skin.1 The most commonly involved mucosa in patients with MMP is the oral mucosa, followed by the conjunctiva.2 oMMP is considered a rare disease with incidence ranged from 1:12000 to 1:60000 with a mean age of 65 years old at diagnosis.1,3,4 Clinically oMMP presents as a bilateral chronic cicatrizing conjunctivitis, often with advanced stage, that can lead progressively to blindness due to severe ocular surface keratinization, corneal scarring, and neovascularization.3,5 Initial symptoms are usually nonspecific, but can include burning, irritation, foreign body sensation, discomfort, and tearing; and this issue can lead to a significant delay in the diagnosis. The progressive course of the disease is characterized by subepithelial fibrosis, forniceal foreshortening, symblepharon formation, distichiasis, trichiasis, cicatricial entropion, loss of goblet cells, impairment of limbal stem cells, meibomian duct obstruction, possible involvement of lacrimal duct with consequent reduced ocular surface lubrication, and eventual blindness stemming from irreversible corneal scarring and keratinization.5 Early diagnosis and appropriate treatment are essential to avoid the vision-threatening complications of this disease.6
A conjunctival-sparing surgical technique for lower eyelid cicatricial entropion repair in ocular cicatricial pemphigoid
Published in Orbit, 2020
Hokyung Choung, Edith R. Reshef, Thidarat Tanking, Suzanne K. Freitag
Ocular cicatricial pemphigoid (OCP) is an autoimmune disease affecting the palpebral conjunctiva and ocular surface. Clinical findings include sub-conjunctival and tarsal fibrosis, fornix foreshortening and symblepharon formation. In more severe cases, cicatricial entropion with trichiasis may develop secondary to conjunctival and tarsal contraction with posterior lamellar shortening, leading to corneal epitheliopathy, vascularization, scarring, ulceration, and epidermalization.1,2 It is important in these patients to correct the cicatricial entropion in order to decrease local conjunctival trauma, break the inflammatory cycle and contribute to halting disease progression. Surgical intervention involving the ocular surface in patients with OCP should only be considered when inflammation is well controlled with systemic immunomodulatory medications in order to maximize surgical outcome and prevent local disease exacerbation.2,3 Various surgical techniques for cicatricial entropion repair in OCP have been described including inferior eyelid retractor plication4,5, mucous membrane grafting6,7, eyelash resection8, and anterior lamellar repositioning.6,9 Despite systemic immune modulation with pre-operative disease remission, the incidence of surgical failure and post-operative disease reactivation remains high in this patient population, particularly in procedures that involve conjunctival manipulation.7,10 There are limited reports of moderately successful conjunctival-sparing surgical techniques to repair lower eyelid cicatricial entropion in patients with OCP in an effort to avoid disease exacerbation.4,5