Prolonged use of topical corticosteroids causes systemic adverse effects including Cushing's syndrome and hypothalamic-pituitary-adrenal (HPA) axis suppression, which is less common than that of the oral or parenteral route. At least 43 cases with iatrogenic Cushing syndrome from very potent topical steroid usage (Clobetasol) in children and adult have been published over the last 35 years particularly in developing countries. In children group (n = 22), most are infants with diaper dermatitis and two cases who had started topical application at a very early age and died from severe disseminated CMV infection. For the adult group (n = 21), the most common purpose of steroid use was for treatment of Psoriasis. The recovery period of HPA axis suppression was 3.49 ± 2.92 and 3.84 ± 2.51 months in children and adult, respectively. We report on an 8-month-old female infant who developed Cushing's syndrome and adrenal insufficiency after diaper dermatitis treatment through the misuse of Clobetasol without doctor's prescription. Physiologic dose of hydrocortisone was prescribed to prevent an adrenal crisis for 3 months and discontinued when HPA axis recovery was confirmed by normal morning cortisol and ACTH levels.
The high prevalence of acne vulgaris in teenagers has increased comorbidities. Lasers offer alternative options for acne treatment because they have rapid action, low systemic adverse effects, and do not require everyday treatment. To study the efficacy and patients' satisfaction of 595-nm pulse dye laser (PDL) treatment of acne vulgaris and acne erythema in adolescents and early adulthood, we designed a blocked-randomized, split-faced 595-nm PDL (fluence 8 J/cm pulse duration 10 ms, spot size 7 mm, 2 session every 2 weeks) study in patients with mild to moderate acne by comparing the laser-treated and non-treated side. The acne lesion counts, acne erythema grading, and acne severity grading were evaluated at baseline and 2, 4, and 8 weeks. Thirty patients were recruited. The results showed no statistically significant difference except the papule count at week 4 which was -1.828 on the treated side and 0.103 on the non-treated side of the face, P-value 0.0018. There was no statistically significant difference of acne severity grading and acne erythema grading between both sides of the face. The mean scores of patients' satisfaction on the laser-treated side were 75, 81, and 81%, respectively. The PDL treatment in this study reveals no significant improvement in acne therapy; however, the patients were satisfied with this laser treatment.
KMS may be treated in a stepwise approach. High-dose steroid does not result in a high response rate and is not tolerated well. The response to IFNalpha2b is more favorable, but life-threatening adverse events may occur. Chemotherapy with vincristine or vinblastine may be useful as adjunctive therapy in KMS, but experience is still limited.
Long-term follow-up is needed to detect flaring of the disease and the side-effects of immunosuppressive drugs.
The normal value of the absolute CD4-positive T-lymphocyte count is relatively high in normal infants and declines steadily until 6 years of age, whereas the CD4 percentage of the total lymphocyte count is constant. The immunologic categories according to the 1994 revised pediatric human immunodeficiency virus (HIV) classification, based on CD4-positive percentage of the total lymphocyte count, is classified into three categories: no evidence of suppression (> or =25%), moderate suppression (15-24%), and severe suppression (1-14%). Our objective was to determine the prevalence of mucocutaneous findings in pediatric acquired immunodeficiency syndrome (AIDS) related to the degree of immunosuppression. We prospectively examined 120 children less than 13 years of age who were born to HIV-seropositive women and developed definite HIV infection. The prevalence of mucocutaneous findings in those children who had severe, moderate, and no evidence of immunosuppression were 62%, 43%, and 20%, respectively. The mucocutaneous findings in patients in the moderate and severe suppression groups were significantly more common than in patients without evidence of immunosuppression (p < 0.001). In the moderate immunosuppression group, 11% had two mucocutaneous findings while 21% in the severe immunosuppression group had two or more mucocutaneous findings. The most common mucocutaneous finding was oral candidiasis (33%), which had a mean corresponding CD4 percentage of the total lymphocyte count of 11.3%. Herpes zoster was found in 6% of the patients (mean CD4 percentage of the total lymphocyte count = 13.5%). Chronic herpes simplex virus (HSV) stomatitis was found in 3% of the patients (mean CD4 percentage of the total lymphocyte count = 3%). Mucocutaneous manifestations are common in pediatric AIDS. The majority of these findings have an infectious etiology. The prevalence increases as the CD4-positive percentage of the total lymphocyte count decreases. More than one mucocutaneous finding can be found at the same time in patients with moderate or severe immunosuppression.
Focal dermal hypoplasia (FDH) is an X-linked dominant disorder characterized by patchy dermal hypoplasia with digital, ocular and dental abnormalities. Very recently, mutations in the PORCN gene were demonstrated to cause FDH. Here, we described three unrelated Thai girls who were sporadic cases of FDH. One of them had unilateral athelia, which has never been described in FDH. Mutation analysis by polymerase chain reaction sequencing the entire coding regions of PORCN successfully revealed three potentially pathogenic mutations, c.373+1G>A, c.737_738insA and c.1094G>A (p.R365Q). One was found in each of three patients. In addition, another sequence variant c.682C>T (p.R228C) with an inconclusive role was found in one patient and her unaffected mother. The two missense mutations were not detected in at least 100 ethnic-matched control chromosomes, and all four mutations had never been previously described. X chromosome inactivation studies showed random patterns in all of them. This study demonstrates that PORCN is the gene responsible for FDH across different populations and extends the total number of confirmed mutations to 26.
Half of AD had completely disappeared at preschool age. Good prognosis was mostly determined by early onset AD and mild severity. Late onset, family history of atopy and increased serum IgE level are associated with respiratory allergy.
We examined 91 children under the age of 13 years with definite HIV infection born to HIV-seropositive women. The clinical spectrum of HIV infection in children younger than 13 years who are born to HIV-infected mothers was revised in 1994 into four clinical categories: category N (not symptomatic), category A (mildly symptomatic), category B (moderately symptomatic), and category C (severely symptomatic). Mucocutaneous manifestations were found in 47 (51.6%) of these children. The prevalence of mucocutaneous manifestations in categories A, B, and C were 4%, 62%, and 75%, respectively. The mucocutaneous manifestations in patients in categories B and C were significantly more common than in those category A (p < 0.001). The most common finding was oral candidiasis (36.3%). Drug rash, pruritic papular eruption, herpes zoster, cutaneous candidiasis, Penicillium marneffei infection, and herpes simplex virus stomatitis were found in 6.6%, 5.5%, 4. 4%, 3.4%, and 2. 2% of patients, respectively. All three patients who had disseminated P. marneffei infection were in category C.
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