Impetigo herpetiformis

Last Updated: 2023-10-12

Author(s): Bianchi R.

ICD11: -

Kaposi, 1887 and Hebra, 1872

  • Generalized pustular psoriasis of pregnancy
  • Generalized pustular psoriasis of pregnancy
  • Impetigo herpetiformis Hebra-Kaposi
  • Psoriasis pustulosa hypocalcaemica
  • Psoriasis pustulosa generalisata

Mostly pustulosis of unclear etiology occurring during pregnancy with a range of additional clinical symptoms and laboratory changes, which is associated with increased mortality and morbidity in both mother and child and therefore requires systemic treatment.

  • Occurrence in pregnant patients between 20-40 years.
  • Manifestation usually in the second half of pregnancy.
  • Occurrence is also possible outside pregnancies and recurrence is possible with each new gravidity.
  • Rarely after parathyroidectomies

  • The exact genesis of the disease is not known, but the clinical picture is currently interpreted as a clinical manifestation of latent or newly developed generalised pustular psoriasis (GPP). I.e. pregnancy is a trigger for GPP.
  • Occasionally, impetigo herpetiformis presents after parathyroidectomies. In this context, one can also speak of hypocalcaemic pustulosis. Therefore, a pathogenesis in the context of parathyroid insufficiency with hypocalcaemia has also been discussed, which could be due to pregnancy or surgical injury to the epithelial corpuscles.
  • The spontaneous improvement after birth, suggests a pregnancy-related genesis.
  • There are reports of mutations in the IL36RN gene in affected individuals.

  • Typically acute onset with deterioration of the general condition accompanied by severe malaise, nausea/emesis, fever, chills and occasionally diarrhoea
  • Patients may additionally present with corresponding symptoms in the context of the hypocalcaemia that usually accompanies the skin symptoms, with tetany being particularly prominent.
  • Exanthema emphasising the intertriginous skin areas, erythematous macules form which extend to the proximal extremities and trunk. At the same time, pustules form. These are disseminated, but also sometimes grouped and closely spaced, yellowish-white with a size of 1-3mm. In the course, there is confluence up to large pus accumulations.
  • A circular appearance of the skin with collerette-like scaly seams directed towards the centre is also possible, especially after the pustules have dried out. This process starts at the earliest one day after the onset of effluorescence
  • In extreme cases, erythroderma develops.
  • Rarely are peripheral neuropathy or renal failure.

  • Clinic
  • Biopsy
  • Pregnancy test if necessary
  • Laboratory to detect hypocalcaemia (not a diagnostic criterion), dysproteinemia, neutrophilia, increased erythrocyte sedimentation rate, iron deficiency with anaemia, Vit D3 deficiency and increased infection parameters
  • Urine status for the diagnosis of proteinuria

  • Eemphasis on intertriginous skin areas
  • Torso
  • Upper and lower extremities
  • Mucosal involvement observable only enorally and in the upper respiratory tract

Subcorneal pustule formation, dermal lymphohistiocytic infiltrate.

  • There is a higher risk of preterm birth, stillbirth, miscarriage and fetal malformations
  • Heart failure in the setting of hypocalcaemia
  • Kidney failure with possible lethal outcome
  • Peripheral neuropathy

None known.

  • This pregnancy dermatosis is associated with increased mortality for the expectant mother.
  • Hypocalcaemia can cause heart failure in the pregnant woman.
  • There is a higher risk of preterm birth, stillbirth and fetal malformations.
  • Recurrence of effluorescence is possible with each new pregnancy.

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