Mucous Membrane Pemphigoid-Associated Paronychia With Onychomadesis

BMC Dermatology
05 Aug, 2019 ,

This is a case of a 58 years old man with severe mucous membrane pemphigoid (MMP) who presented with onychomadesis. He presented with 2 years history of recurrent painful mouth sores and cutaneous blisters on his extremities and genital area. The patient was initially treated with 1 mg/kg of prednisone which resulted in rapid control of his symptoms but when the dose was tapered to 0.5 mg/kg the patient showed signs of disease recurrence. 2 g/kg/cycle of intravenous immunoglobulin therapy IVIG was added. The patient received three cycles on a monthly interval and showed remarkable improvement.

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A 58 years old man presented to the dermatology clinic with 2 years history of recurrent painful mouth sores and cutaneous blisters on his extremities and genital area. A review of symptoms was notable for eye irritation, redness and foreign body sensation in both eyes. The patient was not known to have any medical illnesses and was not taking any medications. Physical examination found confluent erosions on the hard and soft palates, buccal mucosa, and on the lateral sides of his tongue. Skin examination revealed atrophic and hyperpigmented scars on the anterior side of both thighs. We also noticed a small atrophic scar on the penile shaft. His left middle finger showed periungal erythema and swelling that was tender to palpation. The ophthalmologic evaluation revealed chronic conjunctivitis on both eyes with fornix shortening in the right eye. Nasal scope examination showed few erosions. Laryngoscopy showed erythematous mucosa over the arytenoids. The gastrointestinal evaluation was normal. Histopathological examination of an oral mucosal biopsy showed sub-epithelial blister with underlying chronic inflammation. Immunofluorescence studies were negative. On the basis of the clinical assessment and histopathological results, we retained the diagnosis of MMP. The patient was initially treated with 1 mg/kg of prednisone which resulted in rapid control of his symptoms but when the dose was tapered to 0.5 mg/kg the patient showed signs of disease recurrence. 2 g/kg/cycle of intravenous immunoglobulin therapy IVIG was added. The patient received three cycles on a monthly interval and showed remarkable improvement. Prednisone dose was tapered to 0.25 mg/kg with no signs of disease activity. The left middle finger periungual inflammation had subsided but onychomadesis was noted on the same nail.