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  • The antigenic expression in the basement membrane components of the skin is similar to that of the nail bed. This helps explain the involvement of the nail in bullous disease.

  • Nail dystrophy is more common in pemphigus than pemphigoid disorders and commonly presents with paronychia, onychomadesis, and onycholysis.

  • Nail dystrophy is a frequent component of many subtypes of epidermolysis bullosa, and clinical features can range from pachyonychia to erosion of the nail with subungual granulation tissue.


  • Blistering of the nails can occur before blistering of the skin in epidermolysis bullosa and nail dystrophy may be present in several family members in previous generations.

  • A skin biopsy is the gold standard for diagnosing both chronic and inherited bullous diseases.

  • Nail dystrophy in autoimmune bullous disorders often indicates increased disease severity.


  • In many cases, treating the bullous disease will resolve nail symptoms in addition to skin symptoms.

  • Autoimmune blistering disorders are treated systemically, often with corticosteroids and an adjuvant immunosuppressive like rituximab.

  • Epidermolysis bullosa is managed through wound prevention and care, pain control, and nutrition supplementation.


  • Any patient, especially a pediatric patient, which presents with fragile skin that blisters in severity out of proportion to offending trauma should be considered for a possible diagnosis of epidermolysis bullosa.

  • The nail presentation can vary widely among different types of bullous disease, so it is important to take nail pathology in conjunction with overall physical exam and history to develop a differential.


  • Although nail findings are found often in epidermolysis bullosa, they are not necessarily pathognomonic for the disease. The entire patient presentation should be considered.

  • Nail dystrophy relapse can occur just before the onset of a flair in autoimmune bullous diseases.


  • When acquiring a biopsy sample to evaluate for epidermolysis bullosa, the biopsy should come from a freshly induced blister. However, biopsy to diagnose pemphigus vulgaris should come from skin or mucosa that is intact.

  • When differentiating paronychia caused by pemphigus vulgaris from other possible causes, know that in pemphigus vulgaris, histology will show suprabasal acantholysis that lacks spongiosis or exocytosis.


  • Patients should be counseled on proper nail hygiene. For example, nails should be trimmed regularly straight across.

  • Patients with epidermolysis bullosa need to be selective in material for clothing and shoes to avoid excessive mechanical trauma and prevent blistering.


Bullous diseases are chronic blistering disorders of the skin that can involve the nails. These diseases can be autoimmune in nature, such as pemphigus and pemphigoid conditions, or they can be inherited, such as those classified under epidermolysis bullosa. This chapter will serve to guide in diagnosing and treating these conditions with respect to their accompanying nail abnormalities. In some cases, nail pathology is the first presentation of bullous disease. Dystrophic ...

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