Table of Content

Archive

Autumn 2012, Vol. 20 No. 3

Hong Kong J. Dermatol. Venereol. (2012) 20, 134-135


Dermato-venereological Quiz

Dermato-venereological Quiz

JC Chan 陳俊彥, CK Yeung 楊志強, NJ Trendell-Smith

A 50-year-old woman was admitted to the hospital for widespread blisters and painful oral ulcers. She had a known history of follicular dendritic cell sarcoma refractory to chemotherapy and was on palliative care. She had been given a course of Ticarcillin disodium/Potassium clavulanate for pneumonia two weeks previously. Physical examination showed generalized blisters and extensive erosions (Figures 1 & 2). There was severe mucositis affecting the lips, buccal mucosa and tongue (Figure 3). Minute erosions were also found over the vulva. An incisional skin biopsy was performed over the back and the histological section and immunofluorescence study is shown (Figures 4 & 5).

 
Figure 1 Widespread erosions noted over the trunk and limbs.   Figure 2 Tense blisters over both soles.

Figure 3 Severe mucositis over the lips and oral cavity.

Figure 4 Photomicrograph (H&E X100) showing lichenoid interface dermatitis including cytoid bodies (thin line) with basal vacuolation, suprabasal acantholysis and tombstoning (thick line).

Figure 5 Direct immunofluorescence showing strong intercellular staining pattern with IgG (X100).

 

Questions

1) What are the clinical differential diagnoses?

2) What are the histopathological features shown in the figure?

3) What is the diagnosis?

4) How would you manage the patient?


Answers to Dermato-venereological Quiz

  1. The clinical differential diagnoses include Stevens-Johnson syndrome, severe fixed drug eruption, pemphigus vulgaris, bullous pemphigoid and paraneoplastic pemphigus.
  2. Histopathological section showed patchy lichenoid inflammation (interface dermatitis) with exocytosis of lymphocytes, apoptotic keratinocytes and basal vacuolar degeneration. There was additional suprabasal acantholysis and clefting with tombstoning. Direct immunofluorescence study showed intercellular and basement membrane deposits of C3, IgG, IgM and C1q.
  3. The diagnosis is paraneoplastic pemphigus (PNP). Indirect immunofluorescence study was positive for anti-skin antibodies against intercellular substance. Oral swabs were negative for herpes simplex virus and fungus.
  4. PNP is in general refractory to most treatments and the overall prognosis is poor. The condition is typically associated with lymphoproliferative disorders, including Castleman's disease, non-Hodgkin lymphoma, chronic lymphocytic leukaemia and thymoma. The hallmark feature of PNP is a painful, intractable stomatitis. Patients may present with polymorphic skin lesions including tense blisters, erosions, erythema multiforme- or lichen planus-like lesions. Patients with PNP are at risk of developing bronchiolitis obliterans (BO), which is frequently the cause of lethality. For patients with underlying benign neoplasms such as thymoma, complete resolution of the skin lesions may be achieved after surgical removal of the tumour. No consensus exists for the treatment of PNP associated with malignant tumours. Potent immunosuppressants are often required to control the blistering process, but they are generally ineffective. Mucosal lesions are in particular difficult to treat. High-dose corticosteroid is the first-line therapy for PNP. Other therapies with reported success include azathioprine, cyclosporine, mycophenolate mofetil, plasmapheresis, intravenous gammaglobulin and anti-CD 20 monoclonal antibody (rituximab). Patients should also be cautiously monitored for respiratory symptoms for the development of BO.