History
A 29-year-old lawyer attends the accident and emergency department with a sudden and severe skin eruption. She has a 3-day history of a rapidly progressing, painful, ero-sive eruption affecting her upper trunk and intertriginous (groin and axillae) areas. She is unable to sleep and finds dressing and undressing difficult as her skin ‘sticks’ to her clothes and then tears off. The eruption is occurring on the background of a 4-month history of painful ‘ulcers’ in her mouth and on closer questioning also her genital skin, which she had attributed to stress. She has been avoiding hot, spicy or hard food for a couple of months. She is otherwise well and is on no medication. Her mother has type 2 diabetes and hyper-tension. Otherwise there is no family history of significant skin or medical disorders.
Examination
She has a widespread skin eruption over her trunk, within her axillae and groin, and involving the inner lips of her vulva. There are multiple discrete and coalescing lesions. The individual lesions are erythematous and tender with overlying erosions, flaccid bullae and friable scale (Fig. 29.1). There are similar erosions involving the mouth, particularly the lips and buccal mucosae. Nikolsky’s sign is positive. The remainder of her physical examination is normal.
Questions
• What is Nikolsky’s sign?
• What is the likely diagnosis?
• How would you confirm the diagnosis?
• What is the management?
Medicine
Nikolsky’s sign occurs in patients with active blistering diseases – firm sliding pressure with a finger separates normal-looking epidermis, producing an erosion.
The presence of flaccid blisters on an inflammatory base affecting the skin and mucosa in this age group is most suggestive of a diagnosis of pemphigus vulgaris. Differential diagnoses to consider include other immunobullous diseases such as bullous pemphigoid, linear immunoglobulin A (IgA) disease or dermatitis herpetiformis, erythema multiforme or Stevens–Johnson syndrome. If the lesions were confined to the skin only, pemphigus foliaceous or staphylococcal scalded skin syndrome could be considered.
The diagnosis of pemphigus vulgaris, an intra-epidermal antibody-mediated autoimmune blistering disease, is made on skin biopsy. Histology of a blister shows separation at the suprabasal layer of the epidermis with acantholysis or individual separation of single keratinocytes. Immunofluorescence of peri-lesional skin will demonstrate intercellular IgG throughout the epidermis (Fig. 29.2). Circulating antibodies against desmosomal adhesion molecules (desmogleins 1 and 3) can also be detected, their titre often reflecting the severity of disease.
Pemphigus is a chronic skin disease associated with significant morbidity and formerly, before the synthetic corticosteroid era, a significant mortality due to infection and elec-trolyte imbalance secondary to fluid loss. Although pemphigus is frequently responsive to systemic steroids, nowadays the side effects of long-term steroid treatment mean that patients are frequently treated with steroid-sparing agents such as azathioprine or myco-phenolate mofetil. The use of plasma cell targeting with rituximab, an anti-CD20 monoclonal antibody, has been shown to dramatically reduce circu-lating autoantibodies and can pro-vide disease control. Extensive skin involvement such as this case may require in-patient management for supportive treatment, skin care, sepsis prevention and pain relief. Specialized dressings which do not adhere to fragile skin may be used, but fre-quently patients are nursed in such a way as to minimize friction and trauma to the skin.
KEY POINTS
• Pemphigus vulgaris is a chronic, antibody-mediated autoimmune blistering disease affecting skin and mucosa.
• Diagnosis is confirmed by the demonstration of bound intercellular antibodies in vivo within the epidermis by direct immunofluorescence and circulating antibodies targeting desmogleins 1 and 3 by indirect immunofluorescence.
• Immunosuppressive treatment particularly aimed at antibody suppression is the cornerstone of therapy. Pain relief and prevention of secondary infection are crucial.
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