In 2010, a 5-year-old child attended a Public Hospital in Buenos Aires for the first time, with a history of relapsing atopic dermatitis and urticaria. The patient had been breastfed until the age of five. His family was of lower middle socioeconomic status, with no remarkable clinical history. He was of Amerindian descent from his father's side. On physical examination, he was mildly overweight with +1 <z <= +2, and presented an exudative eczema in axillae, neck, groins, inframammary folds, and periumbilical area. Retroauricular regions and auricles were also compromised, and the patient complained of ear recurrent discharge. Eczema was prominent on the scalp, showing thick scaling and chronic exudation. There were scabs on nostrils and he suffered from nasal discharge. There were also inflammatory papules on the neck, abdomen and back (Figure 1). Pruritus was mild. Overall, the patient looked healthy.
Seborrheic dermatitis and tinea capitis were suspected and ruled out. Normal values were observed in blood count, kidney and liver function, electrolytes, and urine analysis. Laboratory tests showed high IgE levels (>1,000 IU) and dyslipidemia. Parasitological analysis was negative. The patient was treated with topical corticosteroids and topical calcineurin inhibitors for skin lesions; topical salicylic acid, coal tar, and betamethasone lotions for the scalp. He also received antihistamines and antibiotics, such as cephalosporins, T/S. On antibiotic courses he experienced a good response, but relapsed after discontinuation.
At the age of seven, he was hospitalized due to exacerbation of his symptoms, skin infection and blepharoconjunctivitis which improved with empiric antibiotics. Blood analysis was within normal values and both blood and urine cultures were negative for fungi and bacteria. Inverted psoriasis was suspected. Skin biopsy revealed psoriasiform dermatitis with epidermal hyperplasia, parakeratosis with few polymorphonuclear cells, acanthosis and hypergranulosis, elongations of vascular connective axes, edematous dermis with mononuclear inflammatory infiltrates and isolated melanophages. At the age of eight, due to the persistence of his psoriasiform-like dermatitis, narrow-band ultraviolet B (NB-UVB) therapy was initiated, and subsequently methotrexate. There was no favorable response to any of them so treatment was discontinued. Skin affection impacted not only on the child's school attendance but also on social interactions. The child's embarrassment and some discrimination episodes prevented him from participating in physical activities and social meetings, and he didn't like to be photographed.
After these events, at the age of 11 in 2016, he was re-evaluated at the dermatology unit. Infective dermatitis was suspected. A new skin biopsy revealed once again spongiosis and acanthosis, but with a perivascular lymphocytic infiltrate. Phenotypical analysis was performed by flow cytometry, which showed a decreased ratio of naive (49.6%) vs. memory effector (20.6%) CD8+ lymphocyte population, compatible with viral infection. Thus, HTLV-1 infection was suspected and confirmed at INBIRS Institute in Buenos Aires. Specific antibodies anti-HTLV-1 were detected by enzyme-linked immunoassay (ELISA) (ELISA HTLV I&II Ab, ULTRA version, Diapro), and infection was confirmed by nested polymerase chain reaction (n-PCR, in-house) after DNA extraction (ADN PuriPrep-S kit, Highway, Inbio). PVL was determined to be 11.5 copies/100 peripheral blood mononuclear cells (PBMC) by quantitative PCR (qPCR) (SybrGreen, ThermoFisher, MA, USA). His mother was also found positive for HTLV-1, but none of his siblings. Human immunodeficiency virus (HIV) and other immunodeficiencies were excluded. IDH was confirmed based on La Grenade's modified criteria. Blood tests were normal, ruling out T-cell leukemia/lymphoma. Stool parasitological and Strongyloides stercoralis tests were negative. Neurological evaluation and brain magnetic resonance imaging (MRI) were normal. Nonetheless, IgE levels increased (3,314 IU). Cytokines in plasma were also analyzed. An increase in interleukin (IL)-6 and IL-9 concentrations was determined while no increase in plasma levels of Th1 (IFNg, IL-2), Th2 (IL-2, -4, -5, -13) or Th17 (IL-17A, -17F, -22) cytokine profiles was observed by LEGENDplex immunoassay (BioLegend). Specific allergen radio allergo sorbent test (RAST) was requested, with a positive result for Dermatophagoides. A specific immunotherapy was indicated with slight improvement.
Once diagnosed, appropriate chronic low dose treatment (alternating antibiotics against gram positive cocci) was indicated to avoid bacterial superinfection of the skin. T/S, minocycline and clindamycin were used according to local resistance patterns. On chronic antibiotic therapy, no further hospital admissions were required. Prophylaxis with T/S was started and the patient experienced partial remission although relapses of folliculitis and erythematous scaly crusty lesions were present. For acute skin infections, 10-day courses of these drugs or clindamycin were alternated. Staphylococcus aureus decontamination was periodically indicated with mupirocin 2% ointment and chlorhexidine. The clinical status evolved with recurrences but favorably. The patient continued to present lower intensity exudative and crusty eczema in folds, scalp, and retroauricular regions; generalized papular eruption involving the face and trunk.
At the age of 14, in 2019, a complete urine test showed glomerular hematuria (>50% dysmorphic red blood cells) and significant proteinuria. Blood pressure was always within normal limits. He was then followed up in the nephrology department. A significant increase in the PVL values (22 copies/100 PBMC) was observed when compared to the first measurement in 2016.
At the age of 15 (in 2020), prophylaxis with minocycline was prescribed, during which period less soft tissue infections were observed. However, the patient still suffered from relapsing chronic dermatitis, presenting recurrences when discontinuing or lowering the antibiotics dose, especially in hot and humid weather. Minocycline was interrupted 6 months later because of a photosensitivity reaction.
Currently, the severity of outbreaks is lower (with scattered skin lesions, mild eczema and papules) and the patient leads a normal social life. He started practicing physical activities and playing soccer. It should be noted that the patient's dyslipidemia improved with changes in lifestyle and diet. At present, the patient remains under multidisciplinary monitoring. New blood and urine controls, PVL determination, and a renal biopsy have been scheduled. A brief outline of the chronology of this case is provided in Figure 2.