Generalised Blistering Eruption in an Elderly Man

presented by

Hussain Mahdi MD

Manana, Bahrain

on May 26, 2006

Senior Resident, Dermatology Unit, SMC, Manama, Kingdom of Bahrain

Abstract An 80-year-old man with a generalised blistering eruption was brought to the A&E Department. Biopsy of the skin showed a subcorneal bullae with PMNs and acantholytic cells consistent with diagnosis of pemphigus foliaceus. He had dramatic response to IVIgG.
H.A. 80-year-old male
Few weeks
trunk, thighs and forearms

Hilal is an 8o-Year-old Bahraini male. He was brought to the A/E department by one of his relatives. His clothes were bloody and dirty since few weeks patient refused to go to the hospital. The patient is a poor historian and was not cooperative. He was in good health till few weeks of his admission when his relatives noticed blood stains on his clothes and they offered him to go to hospital but he refused. After a few days the patient was unable to go to his work and he was tired all the time. His wife asked one of their relatives to take him to hospital even by force. His past medical history was insignificant. He was not on any medications Living with his wife in old house without electricity

Physical Examination

Multiple erosions, ruptured bullae, erythmatous patches and one intact bulla over the right thigh. There were crusted ulcers over the trunk, lower limbs and forearms No lesions were noted on the face or scalp. No oral or genital lesions No eye involvement No palpalple lymph nodes.


Laboratory Data

Complete blood count: witin normal limit Liver function test: within normal limit Urea/Electrolyte/Creatinine: within normal limit Skin Swab culture: Sterile CXR: no abnormality detected. CT scan chest and abdomen : no abnormality detected


Intact bulla 3mm punch biopsy was sent for routine histology H & E stain Perilesional 3mm punch biopsy was sent for DIF Serum was sent abroad for IIF for Guinea pig esophagus Rat bladder epithelium substrates but the results are not ready yet

Histopathology report: Subcorneal bullae with PMNs and acantholytic cells with perivascular eosinophilc infiltration .

DIF: IgG deposits in intracellular spaces of upper epidermal cell layer. C3 deposits along DEJ


Diagnosis Pemphigus foliaceus
Reasons Presented

Could we consider Paraneoplastic pemphigus as a differential diagnosis on the DIF results C3 deposition in DEJ not on clinical basis?


He was treated with diluted potassium permanganate wash, topical fucidin cream and betnovate cream, IV hydrocortisone and IV ranitidine. The patient was fit for discharge on oral prednisolone after 12 days. He did not come back for follow up, however. After 10 weeks he was readmitted again with multiple erosions, crusted ulcers and ruptured bullae. He was started on IVIg course 2g/kg/cycle given over four days. He showed excellent response.

References A. Razzaque Ahmed, MD, and Naveed Sami, MD Intravenous immunoglobulin therapy for patients with pemphigus foliaceus unresponsive to conventional therapy J Am Acad Dermatol 2002;46:42-9
Keywords pemphigus foliaceus, therapy, intravenous immunoglobulin G
Comments from Faculty and Members

Rick Sontheimer MD, Professor and Vice-Chairman, Dept. of Dermatology University of Oklahoma Health Sciences Center, Oklahama City,OK, USA on May 26, 2006

Might consider a trial of antimalarials (hydroxychloroquine 6.5 mg/kg/day lean body mass and if that fails add quinacrine 100 mg/day) as a systemic immunosuppressive sparing agent

David Elpern MD, Williamstown, MA, USA on March 27, 2006

This patient would probably do very well if he were compliant. There are strong cultural factors which prevent this from the sounds of it. Does he want to live? At age 80, is he ready to die? If he came in for regular visits, he might be maintained with low doses of prednisone and doxycycline or dapsone. I have a similar case who has done well with 100 mg of dapsone a day for the past ten years after a month or so of prednisone. Dapsone, in an 80 yo non-compliant patient could be hazardous, however. IVIg is a very expensive proposition and one wonders if it is necessary for most uncomplicated cases of pemphigus. This case highlights the difficulties of treating such a disease process in patients who do not buy into our therapeutic paradigm.

Khalifa Shaquie MD, PhD, Professor of Dermatology, College of Medicine, University of Baghdad, Baghdad, Iraq on March 29, 2006

There are no features of paraneoplastic pemphigus and the picture is in favour of pemphigus vulgaris but there is no oral involvement. The management is by oral prednisolone plus azathioprine. Dapsone could be added.

Henry Foong FRCP Ipoh, Malaysia on March 29, 2006

Paraneoplastic pemphigus (PNP) is characterised by a polymorphic eruption with severe mucosal involvement which is not present in this patient. Histology typically shows suprabasal clefting, prominent vacuolar changes of the basal cells and a lichenoid infiltration. Direct immunofluorescence shows the typical intercellular deposition of IgG and complement.

The first-line therapy for treatment of PF is a systemic corticosteroid. Immunosuppressive drugs such as azathioprine and dapsone may be used as adjuvants to corticosteroids but hydroxychloroquine, cyclophosphamide, mycophenolate mofetil, and methotrexate or even immunomodulators such as tetracyclines and niacinamide are possible alternatives.

Due to exorbitant costs, I would reserve IVIg for severe widespread PF not responding to oral corticosteroids and adjuvant therapy.

Thamir Alkubaisi MD, Baghdad, Iraq, on June 2, 2006

Thank you Dr. Hussain Mahdi, with much thanks for VGRD for this interesting case. Prednisolone and azathioprine (as steroid sparing drug) can be used until improvement in clinical picture and reduction in serum Antibodies depending on IIF.

Haitham Alqari MD, Bahrain on June 2, 2006

Nice case. Clinically the pictures are strongly suggestive of pemphigus vulgaris. No feature of pemphigus foliaceus at all. Paraneoplastic pemphigus presents typically with prominent mucosal involvement especially lips. I will consider repeating skin biopsy for histo and immunofluorescence. With regard to treatment I want consider IVIG at first because of the cost + pemphigus vulgaris responds very well to systemic steroid and other modalities.

Abbas Alshammari MD, Doha, Qatar on June 5, 2006

Clinically,the case is compatabe with PF in the absence of oral involvment. So is IF. The histopathology seems to me a subcorneal blister of secondary infection full of PMNs plus secondary acantholysis. The idea behind presenting this case is to demonstrate the role of IVIG in resistant cases of pemphigus.
1.The case showed good respons to topicals used and oral prednisolone and the author has many choices before considering it as a resistant case.
2.The goal of treating pemphigus is not how to induce a rapid remission but to maintain a good control with a safest medicine considering costing.
3.The author didn't show what is next after a 4 days of IVIG therapy or follow up period.

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