Male sex, born after controlled pregnancy and vaginal delivery, at 38 weeks of gestation.
He weighed 3.100 grams and measured 49 cm. He received breastfeeding and artificial feeding from the first days of life.
Both parents were apparently healthy, with no history of consanguinity.
At one month of life papuletin papules appeared in the age extended erythema in the retroauricular area and gradually appeared in the rest of the face, neck, axillae and consultation of the groin.
A first skin biopsy suggested seborrheic dermatitis.
During the weeks without diffuse scalp disease, the lesions spread to the whole body surface, including palms and soles, with intense exudation and special predilection for the cervical, retroauricular mucosa.
Given the severity and extent of skin lesions, she was hospitalized with the diagnosis of exfoliative erythrodermia under study, and probable immunodeficiency was proposed.
1.
The following tests were performed: hematocrit: 24%, hemoglobin: 7.5 g/dL, CRP: 2 mg/L, ESR: 6 mm/h, normal liver function tests, albumin x 2.4 g/dL bilateral interstitial images and RBC.
VDRL (-), HIV ELISA (-).
The immunological study showed elevated serum IgE (131 IU/ml) and increased subpopulations of CD3, CD4 and CD8 T lymphocytes.
feverish rash, with purulent exudate in skin lesions, and both hemocultives and skin and nasal secretion cultures were positive to multi-sensitive Staphylococcus aureus, which improved with intravenous
Due to its acute protein malnutrition, attributed to extensive skin exfoliation, 4.8 g/kg weight/day proteins were added.
Due to its poor evolution, a new evaluation was requested, this time in the Pathological Service of Roberto del Rio Hospital, where it was suggested to perform a maternal plasma transfusion (planting a Leiner disease).
During the plasma processing in the blood bank of the Felix Bulnes Hospital, the presence of HTLV-I antibodies was detected through the ELISA technique, both in the high mother and in the patient, confirmed in public health tests.
Subsequently, the mother went to controls sporadically.
In the following months, the patient presented with a significant delay in height, with a conserved weight/ height ratio, which corrected anemia and hypoalbuminemia.
She had frequent exacerbations of her eczematous condition, which were managed with topical or oral antimicrobials and low potency corticosteroids, applied in the most affected areas.
At two years of age, the skin lesions became more nodular and generalized and a second biopsy was performed which showed epidermal spongiosis and in the dermis, a marked lymphoid exocytic infiltrate of lymphocytes.
Immunohistochemical study showed CD8 (+) in approximately 60% of lymphoid cells, CD4 weakly (+) in 3% and CD7 marker was negative.
The CD25 marker was intensely positive with a meiotic pattern in the dermal-epidermal infiltrate.
These findings were considered compatible with infectious dermatitis (ID), although the possibility of HTLV-I associated cutaneous T-cell lymphoma (CTCL) was initially thought.
Myelogram performed at that time was normal.
Both biopsies (2 and 24 months) underwent a dissection under the magnifying glass of the lymphoid infiltrate, DNA was extracted and the presence of the virus was sought by positive polymerase chain reaction (PCR).
Two primers for the tax gene of the virus were used, as published by Vandamme4, which is routinely used for detection in serum of affected patients.
1.
At the age of three, he came to control in good general conditions, with disseminated papular lesions throughout the skin, distributed especially on the face, upper and lower extremities, without signs of overinfection.
He had acute suppurative colitis and polyadenopathies of the lateral chains of the neck and inguinal area; the rest of the physical examination was normal.
A language delay was also investigated.
Blood count, chest X-ray and abdominal ultrasound were normal.
