A 10-year-old female patient referred to the outpatient dermatology clinic of the National Children's Hospital Benjamin Bloom in San Salvador, for presenting thickening and color changes in the right nail.
On physical examination of the scalp, three plaques are observed parietal apex, with healing in the periphery and few serosanguinolent crusts in the center, two of these plaques are located in the right skull.
In the oral cavity and lips, whitish plaques are observed in the center and edges of the tongue, as well as both lip commissures. When removing these, erythema and fissure are observed.
The nail of the right thumb is thickened, thickened, thickened, and erythematous in the periungual folds of the nail of the middle left hand has a thickened lateral fold.
No clinical signs of hypoparathyroidism or Addison's disease were found.
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From 2 months of age he has required hospital care for diarrhea, acute gastroenteritis, pneumonia and moderate malnutrition, with multiple hospitalizations; suffers from oral candidiasis from 5 months of age.
The laboratory tests archived in their clinical records report chronic anemia and intestinal parasitism; three ELISA tests for human immunodeficiency virus, taken during the second year of life, were negative, so this infection was ruled out.
The patient is the second of three sisters and has two older siblings with whom she only shares father; parents and siblings are healthy.
No evidence of recurrent skin infections or endocrinopathies was found in the patient's grandparents and uncles.
A possible primary immunodeficiency, specifically a CMC, is indicated immunologic studies.
Flow cytometry reports low serum levels of T lymphocytes and NK cells, which affect the total number of lymphocytes; serum concentrations of B lymphocytes are normal.
The deficit in cell-mediated immunity is also verified through a candidine test, which denotes the absence of induration both at 48 h and one week after application of the tuberculin test; the same results were observed with the test.
Serum levels of immunoglobulins A, E and M are normal, and IgG levels are high.
Mycological culture of the nail of the right thumb showed a growth of Trichomon menta thick; growth of Candida albicans was detected in scalp lesions.
Discrete serum concentrations of heteroethics: sodium, potassium, chlorine, calcium, phosphorus, as well as tests of renal function and glutamic-pyruvic transaminase are normal; a glutaxamic elevation was found.
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Clinical studies confirm the diagnostic suspicion of CMC, specifically by a deficit of T lymphocytes and NK cells.
Itraconazole 100 mg orally, once a day for two months, and nistatin 1,000 000 U/ml orally, four times a day, was indicated, with resolution of cutaneous and oral lesions, but not onychomycosis.
Approximately one month after treatment with itraconazole, oral candidiasis reappeared.
