Severe eczema and pruritus in a two year old boy with diabetes mellitus.
Thomas L. Ray and Arthur Huntley (editors)
Dermatology Online Journal Volume2 Number 1: 9

The family of this two year old Italian infant have requested help from the readers of this Journal. In the first issue of the Journal, an unknown case was presented for comment by the readers, and the comments were appended to the article as they arrived. Although there is information missing from this case, and there was a problem translating from Italian to English, we believe that this material might serve as another focal point for discussion. If you would like to comment on the diagnosis and treatment of this child, you may mail your comments to the editor:
achuntley@ucdavis.edu

Report of a Case

Lorenzo Marchese is a 2 year old baby suffering from IDDM and extensive eczema with intense pruritus. He was born on January 4, 1994 at the 40th week of pregnancy, with a normal delivery. His weight was 4,950 grams and Apgar scores were 9 and 10 at 1 and 5 minutes respectively. The mother had 3 previous pregnancies:
  1. 1983 : vacuum delivery of a female newborn, weight 3,800 grams
  2. 1992 : spontaneous abortion at 26 weeks, normal karyotype, male.
  3. 1993 : spontaneous abortion at 7 weeks
During this last pregnancy, the mother developed an abnormal OGTT and was treated only with dietary restriction. Family history was negative for diabetes mellitus.

Lorenzo received breast feeding plus Mellin 1.

He was admitted to Latisana Hospital at one month of age with a purulent and exudative erythroderma desquamativum with:
  1. pruritus on the face, trunk and limbs, especially in the retroauricular sulcus
  2. mucous diarrhea with blood
  3. abdominal colic, pain
  4. continuous crying
His weight was 5,600 grams.
  • WBC: 24,300/ mm3; eosinophils: 17.4%, neutrophils: 20%, lymphocytes: 53%, monocytes: 6%
  • RBC: 4,400,000/mm3, Hgb: 14.3, MCV: 96
  • IgE: 293 KU/L
  • Rare fecal eosinophils
  • RAST for milk, fish, grain, soya: negative
He was discharged from the hospital with diet therapy (hydrolysate proteins).

On the 15th of February, 1994 he was admitted to Trieste Hospital (specialized in pediatrics ) and his exams showed:
  • Hypoproteinemia ( 3.6 grams % )
  • Hypogammaglobulinemia with IgG: 218 mg % (IgM: 29%, IgA: 37%)
  • Leukocytosis with eosinophilia (WBC: 40,000/mm3, PMN: 40%, Eos: 25-30%)
  • Bone marrow: hyperplasia of white cells (myelocytes/erythroblasts = 9/1)
  • Lymphocytes T4/T8 = 5/1 (helper 70%, suppressor 15%)
  • Increasing IgE (361 U/L, 595 U/L, 1,849 U/L)
  • Multi-RAST test: negative
He was treated with immunoglobulins I.V. (20 grams in all), prednisone, antihistamine, azithromycin (50 mg/d x 3 days), fluconazole (15 mg), diet (Nutrinaut 15%).

He was admitted again to Trieste hospital on the 14th of March, 1994 with persistent symptomatology (exudative erythroderma desquamativum). Weight was 5,320 grams.
  • Lymphocytes and their subtypes: normal (normal mature T and B lymphocytes)
  • Normal response of T lymphocytes to mitogens (rule-out OMMEN syndrome)
  • Remarkable lymphocyte activation (CD25: 34%, DR: 23%)
  • Low number of naive lymphocytes (CD8: 8%, CD4: 17%)
  • IgE: 1800 U/L
  • WBC: 22,000/ mm3, eosinophils 3,000-4,000/mm3
Lorenzo showed worsening of his symptoms (exudation) with I.V. gamma globulins and albumin. Hee was then treated with immunosuppressors (cyclosporine plus prednisone 1 mg/kg), antibiotics (erythromycin, cotrimoxazole, fluconazole) and H1 and H2 blocker antihistamines.

On April 5th, 1994 he was again admitted to Trieste Hospital.
  • Eosinophils: 3,000/ mm3 (30%)
  • Prick test for cow's milk - positive
The dosage of cyclosporine was increased to 35 B.I.D. and there was improvement of cutaneous symptoms (especially desquamation); Eosinophils: 980/mm3.

On May 17th, he was admitted to Brescia Hospital. Weight was 5,830 gr. (10th-20th percentile); height was 61 cm. (10th-20th percentile). He was noted to have diffuse desquamative erythroderma, hepatomegaly and inguinal adenopathy.
Lorenzo had an episode of bronchospasm treated with aminophyllin. He was discharged with diagnosis of food allergy. All the previous drugs were stopped and he was given only topical steroids; diet with Nutrinaut. At that time he had only erythroderma.

  • RBC: 3,040,000/mm3, Hgb: 8.3, MCV: 81, ESR: 10
  • Eosinophils: 17%
  • C-reactive protein: 5
  • Reaction to tetanus toxoid: negative
  • RAST for whole milk: 73.6 KU/L (5th class)
  • Skin and lymph node biopsy: negative.
  • HLA:
    • - MARCHESE, LORENZO: A1, A2, B62, B57, CW3, DR2, DR7, DQ1, DQ3
    • - MARCHESE, ENZO(father): A2, B7, B62, CW3, CW7, DR2, DQ1
    • - CAMPANELLA, MARIA (mother): A1, A24, B57, B35, CW4, DR11, DR7, DR52, DQ3;
    • - MARCHESE, ELENA (sister): A2, A24, B62, B35, CW3, CW4, DR2, DR11, DR52, DQ1, DQ3.
On June 27th, he was admitted to Trieste Hospital for evaluation of growth retardation. This was determined to be due to an inadequate diet and he was treated with a supplement (Nutrinaut 600-700 cc per day).
  • RAST for milk proteins, rye, wheat, oats: 4th-5th class
  • Eosinophils: 1,750/ mm3
  • Blood proteins: 5.5
On October 13th, he was admitted to Trieste Hospital for acute worsening of his exudative erythroderma, especially on the face and on the head.
  • IgE: 19 U/ml
  • Eosinophils: 1,800/ mm3
  • PLT: 540,000/ mm3
  • RAST for milk, oats, casein: 2nd class.
The patient was treated with topical steroids, and oral zinc sulfate.

On October 18th 1995 he was admitted to Brescia Hospital. Weight was 8,500 grams (less than 3rd percentile); height was 74 cm (25th-50th percentile).

  • Eosinophils: 0 (zero)
  • RAST for milk: negative
  • Total IgE: normal
  • Cellular and humoral immune response: normal.
On December 27th, he was admitted to Latisana Hospital for herpetic stomatitis with dehydration, bronchitis and fungal otitis.
  • Hgb: 11.1, Hct: 35.5, MCV: 75.4, RBC: 4,710,000/ mm3
  • WBC: 7,700/ mm3
  • PLT: 265,000/ mm3
On February 25th, 1996 he was admitted to Latisana Hospital for polyuria and polydipsia. His blood glucose was 728 mg/100 ml, and his weight 9,300 grams He was treated with insulin.

The patient subsequently developed viral gastroenteritis (rotavirus), thought to be hospital acquired. He also had a respiratory infection with bronchospasm.

  • Hgb: 10, MCV: 75
  • WBC: 10,600/ mm3, PLT: 549,000/ mm3,
  • Lymphocytes and their subtypes: normal
  • IgG: 1,360 mg/dl, IgA: 102, IgM: 55, IgE: 41 kU/l
  • Ab anti-thyroglobulin - thyroid peroxidase: negative
  • Ab anti-endomysium: negative
  • Ab anti-gliadin IgA: 22.47 U/A (normal values: > 7 U/A) and
    Ab anti-gliadin IgG: 80.3 U/A (normal values: > 15 U/A) by a GLIA-TEST EUROSPITAL, Italy
  • C-reactive protein: normal
  • HbA1c: 9.7% (normal values 3.4-6.1)
  • C-peptide: 0.21 ng/ml (normal values: 1-3 ng/ml)
  • IgA: 22.47%, IgG: 80.3%;
  • ANA (Hep-2): negative
  • Ab anti-reticulin: negative
  • RAST for lactalbumin, lactoglobulin, casein, egg, fish, soya, tomato: 0 (zero) class.