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Pemphigoid nodularis | DermNet New Zealand

Pemphigoid nodularis

Author(s): Dr Jenny Chung, House Officer, Department of Dermatology, Auckland City Hospital, New Zealand. DermNet NZ. Editor-in-chief: A/Prof Amanda Oakley,  Dermatologist, Hamilton, New Zealand. Copy Editor: Gus Mitchell, August 2017.


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 (1979) and Yung et al (1981) [1,2].

Pemphigoid nodularis*

* Reproduced under Creative Commons Licensing, from Indian Dermatol Online J. 2014 Apr-Jun; 5(2): 189–192. doi: 10.4103/2229-5178.131101. PMC.

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 IgG and C3 along the basement membrane.

Blood tests may reveal positive indirect immunofluorescence and autoantibody titres to 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.

 

Related Information

References

  1. Provost TT, Maize JC, Ahmed AR, Strauss JS, Dobson RL. Unusual subepidermal diseases with immunologic features of bullous pemphigoid. Arch Dermatol. 1979;115(2)156-60. Pubmed
  2. Yung CW, Soltani K, Lorinez AL. Pemphigoid nodularis. J Am Acad Dermatol. 1981;5(1):54-60. Pubmed
  3. Al-Salhi W, Alharithy R. Pemphigoid nodularis. J Cutan Med Surg. 2015;19(2):153-5. Pubmed
  4. Dangel B, Kofler L, Metzler G. Nodular subtype of bullous pemphigoid. J Cuta Med Surg. 2016;20(6):570-2. Pubmed
  5. 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
  6. Ray J, Camilleri M. Pemphigoid nodularis: Two new cases demonstrating distinguishing clinical clues from prurigo nodularis, Journal of the American Academy of Dermatology , Volume 60 , Issue 3 , AB103 - AB104. Journal
  7. 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. Pubmed
  8. Zhang W, Liu Y, Li C. Generalised nodules in pemphigoid nodularis. Lancet. 2017;398:1930. Pubmed

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