CASE IV

1. What is your diagnosis? And why?

2. How would you establish a diagnosis in such a patient?

1. What is your diagnosis? And why? The patient has multiple vesiculobullous lesions, many of the bullae are on normal skin. Depending on whether the disease is acute or chronic and the age of the patient the differential diagnosis of such a lesion could include: Acute: bullous impetigo especially if the patient is a child Chronic: pemphigus vulgaris

Bullous Impetigo
Acute
Usually an infant Bulla on bland skin, heal in centre and spread peripherally Oral lesions usually absent Face Acantholytic cells negative; rarely positive Histopathology shows polymorphs subcorneal bulla with

Pemphigus Vulgaris
Chronic
Usually adult Bulla on bland skin. Rupture to form non healing erosions Oral lesions universal and consist of Flexures, scalp Positive on Tzanck smear Suprabasal bulla with acantholytic cells

2. How would you establish a diagnosis in such a patient? 3. What are the variants of the disease?

4.
5.

What are the complications of pemphigus?

What are the modalities of treatment available?

2. How would you establish a diagnosis in such a patient? Tzanck smear Biopsy: - Histopathology - Direct immunofluroscence Indirect immunofluroscence 3. What are the variants of the disease? Pemphigus vulgaris Pemphigus foliaceus Pemphigus vegetans Pemphigus erythematosus 4. What are the complications of pemphigus? Water and electrolyte imbalance. Dehydration and prerenal renal failure Infection of lesions and septicemia

5. What are the modalities of treatment available?

The treatment depends on the severity:
Extensive lesions: Supportive treatment: - Fluid-electrolyte balance - Wound care - Control of secondary infections Specific treatment - Corticosteroids: which in patients with extensive lesions needs to be given daily initially and when the lesions have healed they are continued either in a daily schedule or as monthly pulses. - Immunosuppressives: these are used as steroid sparing agents to reduce the side effects of the steroids. The 3 immunosuppressives used include cyclophosphamide, methotrexate and azathioprine. Localized lesions: - Corticosteroids: which are the 1st line of therapy. They can be given daily, or as monthly/ weekly pulses. - Immunosuppressives: these are used as steroid sparing agents to reduce the side effects of the steroids. The 3 immunosuppressives used include cyclophosphamide, methotrexate and azathioprine