CME INDIA Case Presentation by Dr Prabhat Kumar, Agra.

CME INDIA Case Study

CASE: Age 60, No mucosa involved, Initially on upper limbs, then thorax and abdomen and in last involved lower limbs. Initially tense, Itching after bullae rupture.

CME INDIA Discussion:

Dr Amit Kumar, Dermatologist, Ranchi:

  • The diagnosis is pemphigus vulgaris. Clinically also the lesions are showing as pemphigus lesions. However, in pemphigus vulgaris the bullae are not very tense as they are superficial.
  • The differential diagnosis is bullous pemphigoid which is more common in older age group, manifests with tense bullae over erythematous urticarial plaques associated with itching.
  • The best approach would be to go for an excision biopsy of a new lesion. For management, best drug is steroids if can be given but with due control of his diabetes. However, if steroid cannot be given then can plan for cyclophosphamide or azathioprine.

CME INDIA Leaning Points:

Bullous Pemphigoid

  • Intermittent eruptions of large, tense blisters and generalized pruritus
  • The disease most often presents in patients older than 60 years, but can affect those of any age.
  • In two-thirds of patients, bullous pemphigoid presents with prodromal symptoms such as generalized pruritus, dermatitis, or urticaria. Prodromal symptoms may persist for weeks to years before blisters first appear.
  • Bullous pemphigoid lesions, varying in size from vesicles to bullae, may form on erythematous or normal skin. Lesions can occur anywhere on the skin surface in a localized or diffuse manner, but most frequently are found on the flexural areas of the arms, legs, axilla, groin, and lower abdomen.
  • Mucosal lesions are seen in 10-40% of patients, but these generally heal quickly and without complication.
  • Most common autoimmune blistering disease.

Pemphigus vulgaris

  • The most common pemphigus subtype
  • Presents in patients 40-60 years of age.
  • Patients typically present with fragile-appearing flaccid bullae that rupture, which results in denuded areas of skin
  • Two-thirds of patients have painful lesions of the oral mucosa as their initial manifestation.
  • The skin lesions most commonly occur on the scalp, chest, back, and intertriginous areas.
  • Pemphigus vulgaris is a chronic and progressive disease with a 5-10% mortality rate due to sepsis or complications of steroid therapy
  • Skin biopsies for H&E staining and DIF confirm the diagnosis. Histologic findings of a blister show a subepidermal bullae with an inflammatory infiltrate of predominantly eosinophils with neutrophils and histiocytes in the upper dermis. Perilesional, uninvolved skin is ideal for DIF.
  • DIF demonstrates fine, linear IgG and C3 at the basement membrane zone. Biopsies for DIF should be placed in Michel’s transport media for processing rather than saline to avoid false-negative results.
  • Serologic studies for indirect immunofluorescence (IIF) can help to confirm the diagnosis. Circulating IgG antibodies to the basement membrane zone can be detected in approximately 80% of patients with BP. Additionally, a peripheral blood eosinophilia may be present, occurring in half of BP patients.
  • Systemic and topical corticosteroids provide the most rapid and effective control of disease. Prednisone at a dose of 0.5 to 1.0 mg/kg per day, based on ideal body weight, often halts formation of new blisters. Prolonged treatment of PV can result in high cumulative doses of corticosteroids, which are the main causes of treatment-related side effects.
  • Drugs as methotrexate, mycophenolate mofetil, or azathioprine are used together with prednisone at the onset of treatment.
  • The long-term goal is to taper the dose of prednisone slowly over a period of several months to a maintenance dose between 5 and 10 mg/day. IVIg, administered in monthly cycles following a defined protocol, and rituximab have shown promise in treating patients with refractory disease.

What you need to know?

  • Bullous pemphigoid and pemphigus vulgaris are diseases of an ageing population
  • Both are associated with high morbidity and mortality, with the commonest cause of death being opportunistic infections from prolonged immunosuppression
  • Topical and systemic corticosteroids are first line treatment for most patients
  • Steroid-sparing regimens are used where steroid treatment fails or if there are adverse effects
  • Non-specialists have important roles in both early detection and ongoing management of these diseases because of their chronic nature and the systemic effects of treatment.

[doi: (BMJ 2017;357:j2169]

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