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Pemphigoid nodularis

Author: Dr Jenny Chung, House Officer, Department of Dermatology, Auckland City Hospital, Auckland, New Zealand. DermNet NZ Editor In Chief: A/Prof Amanda Oakley, Dermatologist, Hamilton, New Zealand. Copy edited by Gus Mitchell/Maria McGivern. August 2018.

Pemphigoid nodularis — codes and concepts
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What is pemphigoid nodularis?

Pemphigoid nodularis is a rare nodular variant of bullous pemphigoid. There is limited literature on pemphigoid nodularis to date, with only case reports describing the condition. It was first described by Provost et al in 1979 and Yung et al in 1981.

Pemphigoid nodularis
Pemphigoid nodularis

Pemphigoid nodularis

Credit: Das D, Bandyopadhyay D. Juvenile pemphigoid nodularis: Report of a rare case. Indian Dermatol Online J 2014; 5: 189–92.

Who gets pemphigoid nodularis?

Pemphigoid nodularis is reported to be more common in women and elderly people.

What causes pemphigoid nodularis?

The causes of this nodular type of bullous pemphigoid are unknown. One theory is that the nodules are induced by rubbing and scratching in individuals with subclinical bullous pemphigoid.

Patients with prurigo nodularis (nodular prurigo) may induce pemphigoid antibodies through physical trauma to the skin and basement membrane.

What are the clinical features of pemphigoid nodularis?

Pemphigoid nodularis presents with hyperkeratotic nodules and blisters, which are usually located on the extremities and trunk.

The nodules are similar to those seen in nodular prurigo. Pruritic papules or confluent plaques have also been reported.

How is pemphigoid nodularis diagnosed?

Pemphigoid nodularis requires clinical or histological evidence of both pruritic nodules and blisters.

Histopathological features of pemphigoid nodularis include:

  • Hyperkeratosis
  • Spongiosis
  • Subepidermal blisters
  • Lymphocytes in the upper dermis.
  • Positive direct immunofluorescence, with linear deposition of immunoglobulin (Ig)G and complement C3 along the basement membrane.

Blood tests may reveal positive indirect immunofluorescence and autoantibody titres to the 180-kD and 230- kD bullous pemphigoid antigens (BP180 and BP230).

What is the differential diagnosis for pemphigoid nodularis?

Pemphigoid nodularis can appear similar to nodular prurigo and other forms of prurigo. However, nodular prurigo lacks blisters and has no serum or immunofluorescence evidence of IgG to bullous pemphigoid antigens. Histology can be used to differentiate between the two conditions.

What is the treatment for pemphigoid nodularis?

The treatment for pemphigoid nodularis can be challenging. Topical steroid therapy alone is inadequate.

Pemphigoid nodularis is treated with the following:

What is the outcome for pemphigoid nodularis?

The prognosis of pemphigoid nodularis is uncertain. Older age and/or neurological comorbidities are associated with higher mortality rates.

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References

  • Provost TT, Maize JC, Ahmed AR, Strauss JS, Dobson RL. Unusual subepidermal diseases with immunologic features of bullous pemphigoid. Arch Dermatol 1979; 115: 156–60. PubMed
  • Yung CW, Soltani K, Lorinez AL. Pemphigoid nodularis. J Am Acad Dermatol 1981; 5: 54–60. PubMed
  • Al-Salhi W, Alharithy R. Pemphigoid nodularis. J Cutan Med Surg 2015; 19: 153–5. DOI: 10.2310/7750.2014.14018. PubMed
  • Dangel B, Kofler L, Metzler G. Nodular subtype of bullous pemphigoid. J Cutan Med Surg 2016; 20: 570–2. DOI: 10.1177/1203475416648322.PubMed
  • Kwong HL, Lim SPR. Pemphigoid nodularis mimicking nodular prurigo in an immune-suppressed patient with rheumatoid arthritis. Acta Derm Venereol 2015; 95: 237–8. Journal
  • Ray J, Camilleri M. Pemphigoid nodularis: Two new cases demonstrating distinguishing clinical clues from prurigo nodularis. J Am Acad Dermatol 2009; 60 (3 Suppl): AB103–4. DOI: 10.1016/j.jaad.2008.11.461. Journal
  • Schachter M, Brieva JC, Jones JCR, Zillikens D, Skrobek C, Chan LS. Pemphigoid nodularis associated with autoantibodies to the NC16A domain of BP180 and a hyperproliferative integrin profile. J Am Acad Dermatol 2001; 45: 747–54. DOI: 10.1067/mjd.2001.116229. PubMed
  • Zhang W, Liu Y, Li C. Generalised nodules in pemphigoid nodularis. Lancet 2017; 398: 1930. DOI: 10.1016/s0140-6736(17)30064-8. PubMed
  • Das D, Bandyopadhyay D. Juvenile pemphigoid nodularis: Report of a rare case. Indian Dermatol Online J 2014; 5: 189–92. PubMed

On DermNet NZ

Other websites

  • Pem Friends — a UK support group for pemphigoid and pemphigus

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