Deficiency of Interleukin-36-Receptor Antagonist (DITRA) – aka Generalized Pustular Psoriasis (GPP)

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Acronym:
DITRA/PSORP

SAID group:
Pyogenic Diseases

Gene:
IL36RN

Inheritance:
Autosomal recessive.

Ethnicity:
May affect all races. Patients with Caucasian, Spanish, Asian, African, Algerian or Tunisian ancestry. [58] [75] [77]

Frequency:
Unknown but rare. 1% of Sfax, Tunisians are carriers, with a 0.52% chance of having the disease in this population. [58]

Timing of symptoms:
Flares last days to weeks. Some with chronic symptoms. Most flare from triggers like: infections, stress, medication changes, during pregnancy or menstruation. [58]

Age of onset:
Variable age of onset. Many have symptoms starting in childhood. Some have symptoms beginning in adulthood. [58]

Skin cutaneous:
Recurrent, generalized pustular psoriasis and high fevers after erythematous rash. Some with acral pustules and nail damage, or chronic plaques. [58] [61]

Neurologic:
Sudden-onset high fever >40°C with chills. Some patients have a headache with the onset of the rash and fever, plus asthenia (muscle weakness) and tachycardia. [58] [61]

Auditory:
Not noted.[58] [61] [62]

Ophthalamic:
Not noted. [58] [61] [62]

Cardiopulmonary:
Tachycardia. Electrolyte imbalances can occur during fever and onset of pustular rash. Risk for cardiac arrest and septicemia. [58] [61]

Abdominal:
Nausea during flares. At risk for a loss of appetite. [58] [60] One infant patient noted with failure to thrive and diarrhea. [63]

Lymphatic:
Risk for renal and liver impairment and systemic infection with severe flares. [58] [61]

Joints bones muscles cartilage:
Asthenia (muscle weakness) during fevers and flares. Risk for inflammatory arthritis. [59] [61]

Vasculitis:
Not noted. [59] [60]

Amyloidosis:
Not noted. [59] [60]

Abnormal labs:
High during flares (in most patients): ESR, CRP, neutrophils, lactate levels. Low: plasma albumin, calcium, zinc. Risk for infections with flares. [59] [60]

Search Keywords:
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Photo credit:

Diepgen TL, Yihune G et al. Generalized Pustular Psoriasis. Dermatology Online Atlas. DermIS Dermatology Information System. Published online at: http://www.dermis.net/dermisroot/en/32493/image.htm

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