Mucopolysaccharide polysulfate cream for reducing the occurrence of eczema and skin atrophy in patients with moderate- or low-risk infantile hemangioma after treatment: a multicenter clinical study / 中华皮肤科杂志

Objective@#To investigate whether topical mucopolysaccharide polysulfate (MPS) cream can reduce the incidence of eczema and skin atrophy in patients with moderate- or low-risk infantile hemangioma after the treatment with topical beta-blockers or 595-nm pulsed dye laser (PDL) , and to analyze factors influencing the occurrence of eczema and skin atrophy.@*Methods@#A total of 722 patients aged 0- 1 years with moderate- or low-risk infantile hemangioma were enrolled from 5 Children′s Hospitals in China. According to the disease condition and therapy acceptability, these patients were divided into 6 groups to be treated with topical beta-blockers and MPS cream (blocker+ MPS group) , topical beta-blockers (blocker group) , 595-nm PDL and topical MPS cream (PDL+ MPS group) , 595-nm PDL (PDL group) , 595-nm PDL combined with topical beta-blockers and MPS cream (PDL+ blocker+ MPS group) , and 595-nm PDL and topical beta-blockers (PDL+ blocker group) , respectively. All the externally applied agents were applied twice a day, and PDL was performed once every 4 weeks. Efficacy and adverse reactions were evaluated after 3-month treatment. Univariate and multivariate Logistic regression analyses were carried out to analyze factors influencing the incidence of eczema and skin atrophy in patients with infantile hemangioma after treatment, and chi-square test was carried out to compare efficacy among the groups.@*Results@#After 3-month treatment, multivariate Logistic regression analysis for comparing the blocker+ MPS group with blocker group showed that the risk factor for eczema on the surface of hemangiomas was no topical treatment with MPS cream (P= 0.007, OR= 3.887, 95% CI: 1.439-10.493) , while no correlations were observed between the occurrence of skin atrophy on the surface of hemangiomas and analyzed factors. Multivariate Logistic regression analysis for comparing the PDL+MPS group with PDL group showed that no topical treatment with MPS cream (P < 0.001, OR= 7.402, 95% CI: 2.604-21.042) and northern areas (P < 0.001, OR= 67.048, 95% CI: 7.977-563.518) were risk factors for eczema on the surface of hemangiomas, and risk factors for skin atrophy on the surface of hemangiomas included no topical treatment with MPS cream (P = 0.001, OR = 9.371, 95 CI: 2.590 - 33.900) and abundant blood supply of hemangiomas (P = 0.036, OR = 2.971, 95% CI: 1.075-8.208) . Multivariate Logistic regression analysis for comparing the PDL + blocker+ MPS group with PDL+ blocker group showed that risk factors for eczema on the surface of hemangiomas were no topical treatment with MPS cream (P= 0.005, OR= 3.426, 95% CI: 1.446-8.119) and northern areas (P < 0.001, OR= 31.704, 95% CI: 6.924-145.158) , and risk factors for skin atrophy on the surface of hemangiomas included no topical treatment with MPS cream (P < 0.001, OR= 6.011, 95% CI: 2.558-14.126) and southern areas (P= 0.022, OR= 3.021, 95% CI: 1.177-7.753) . After 3-month treatment, the response rate was significantly higher in the PDL group than in the PDL+ MPS group (χ2= 4.531, P= 0.033) , and significantly higher in the blocker group than in the blocker+ MPS group (χ2= 4.344, P= 0.037) . There were no significant differences in the response rate or cure rate among the other groups (all P > 0.05) .@*Conclusion@#During the treatment of moderate- or low-risk infantile hemangioma with topical beta-blockers or 595-nm PDL, the combination with topical MPS cream can reduce the occurrence of eczema and skin atrophy without affecting the therapeutic effect.

Mucopolysaccharide polysulfate cream for reducing the occurrence of eczema and skin atrophy in patients with moderate- or low-risk infantile hemangioma after treatment: a multicenter clinical study / 中华皮肤科杂志

Objective To investigate whether topical mucopolysaccharide polysulfate (MPS)cream can reduce the incidence of eczema and skin atrophy in patients with moderate-or low-risk infantile hemangioma after the treatment with topical beta-blockers or 595-nm pulsed dye laser(PDL), and to analyze factors influencing the occurrence of eczema and skin atrophy. Methods A total of 722 patients aged 0-1 years with moderate-or low-risk infantile hemangioma were enrolled from 5 Children' s Hospitals in China. According to the disease condition and therapy acceptability, these patients were divided into 6 groups to be treated with topical beta-blockers and MPS cream(blocker+MPS group), topical beta-blockers(blocker group), 595-nm PDL and topical MPS cream(PDL+MPS group), 595-nm PDL(PDL group), 595-nm PDL combined with topical beta-blockers and MPS cream(PDL+blocker+MPS group), and 595-nm PDL and topical beta-blockers(PDL+blocker group), respectively. All the externally applied agents were applied twice a day, and PDL was performed once every 4 weeks. Efficacy and adverse reactions were evaluated after 3-month treatment. Univariate and multivariate Logistic regression analyses were carried out to analyze factors influencing the incidence of eczema and skin atrophy in patients with infantile hemangioma after treatment, and chi-square test was carried out to compare efficacy among the groups. Results After 3-month treatment, multivariate Logistic regression analysis for comparing the blocker + MPS group with blocker group showed that the risk factor for eczema on the surface of hemangiomas was no topical treatment with MPS cream(P = 0.007, OR = 3.887, 95％ CI: 1.439- 10.493), while no correlations were observed between the occurrence of skin atrophy on the surface of hemangiomas and analyzed factors. Multivariate Logistic regression analysis for comparing the PDL + MPS group with PDL group showed that no topical treatment with MPS cream(P < 0.001, OR = 7.402, 95％ CI: 2.604- 21.042)and northern areas(P <0.001, OR=67.048, 95％CI:7.977-563.518)were risk factors for eczema on the surface of hemangiomas, and risk factors for skin atrophy on the surface of hemangiomas included no topical treatment with MPS cream(P=0.001, OR=9.371, 95 CI:2.590-33.900)and abundant blood supply of hemangiomas(P=0.036, OR=2.971, 95％CI:1.075-8.208). Multivariate Logistic regression analysis for comparing the PDL+ blocker + MPS group with PDL + blocker group showed that risk factors for eczema on the surface of hemangiomas were no topical treatment with MPS cream(P=0.005, OR=3.426, 95％CI:1.446-8.119) and northern areas(P<0.001, OR=31.704, 95％CI:6.924-145.158), and risk factors for skin atrophy on the surface of hemangiomas included no topical treatment with MPS cream(P<0.001, OR=6.011, 95％CI:2.558- 14.126) and southern areas (P = 0.022, OR = 3.021, 95％ CI: 1.177- 7.753). After 3-month treatment, the response rate was significantly higher in the PDL group than in the PDL+MPS group(χ2=4.531, P = 0.033), and significantly higher in the blocker group than in the blocker + MPS group (χ2 =4.344, P=0.037). There were no significant differences in the response rate or cure rate among the other groups(all P>0.05). Conclusion During the treatment of moderate-or low-risk infantile hemangioma with topical beta-blockers or 595-nm PDL, the combination with topical MPS cream can reduce the occurrence of eczema and skin atrophy without affecting the therapeutic effect.

Hipoplasia dérmica focal (síndrome de Goltz) / Focal dermal hypoplasia (Goltz syndrome)

Resumen: Introducción: La hipoplasia dérmica focal o síndrome de Goltz es una rara genodermatosis de herencia dominante ligada al X, que afecta al tejido proveniente de las placas del ectodermo y del mesodermo. El cuadro clínico se caracteriza por alteraciones cutáneas, oftálmicas, neurológicas, dentales, ungueales, bucales, de tejidos blandos y esqueléticas. El diagnóstico se realiza por los hallazgos clínicos en un individuo con alteraciones ectodérmicas y malformaciones características en las extremidades. El manejo es multidisciplinario y, al igual que el pronóstico, depende de las alteraciones específicas que presente cada paciente. Caso clínico: Se presenta el caso de un recién nacido de sexo femenino, de 15 días de vida, con zonas de alopecia en piel cabelluda, herniación de tejido celular subcutáneo en varias áreas de todos los segmentos corporales, escotadura en ala nasal, hendidura en encía superior, defecto grave de extremidad superior izquierda con rizomelia (acortamiento de segmento proximal) y aplasia de radio, así como ectrodactilia de miembro pélvico derecho. Todos los hallazgos son compatibles con hipoplasia dérmica focal de acuerdo con los criterios diagnósticos. Conclusiones: Se presenta el caso de una paciente recién nacida con síndrome de Goltz.

Abstract: Background: Focal dermal hypoplasia or Goltz syndrome is a rare X-linked dominant inherited genodermatosis, affecting both the ectodermal and mesodermal tissue. Clinical manifestations include skin abnormalities, defects in eyes, teeth, nails, mouth, soft tissues and skeleton. The diagnosis is based on clinical findings and is suspected in individuals with ectodermal abnormalities and characteristic malformations in the extremities. The management is multidisciplinary and, like the prognosis, depends on the specific alterations that each patient presents. Case report: We report the case of a 15-day-old female newborn with alopecic areas on the scalp, herniation of subcutaneous cellular tissue at the lumbar level, nasal wing notch, severe left superior limb defect with rhizomelia (proximal segment shortening) and radio aplasia, as well as right leg ectrodactyly, areas of atrophy compatible with focal dermal hypoplasia according to diagnostic criteria. Conclusions: We present a case of female newborn patient with Goltz syndrome.

A Rare Case of Suspected Dystrophic Epidermolysis Bullosa Pruriginosa

Inherited epidermolysis bullosa (EB) encompasses over 30 phenotypes or genotypes. A characteristic feature of all types of EB is the presence of recurrent blistering or erosions, the result of even minor traction to this tissues. There are four major types of inherited EB: EB simplex (EBS), junctional EB (JEB), dystrophic EB (DEB), and Kindler syndrome. These differ not only phenotypically and genotypically but more importantly by the site of ultrastructural disruption or cleavage. Dystrophic epidermolysis bullosa (DEB) is a rare mechanobullous genodermatosis inherited either with autosomal dominant or recessive pattern and characterized by fragility, blistering and scarring of the skin and mucous membranes. Blistering is due to abnormalities in anchoring fibrils (AF), microstructures mainly composed of type VII collagen (COLLVII), which contributes to the maintaining of dermal-epidermal adhesion. Most cases are sporadic, but a few show autosomal dominant or autosomal recessive pattern of inheritance. Microscopic studies of EB pruriginosa show typical findings of dystrophic EB, and it has been postulated that itching lesions of EB pruriginosa could represent an abnormal dermal reactivity of some subjects to their inherited bullous disorder. The study of the molecular basis of dominant dystrophic EB (classical) and EB pruriginosa shows that both diseases are caused by a missense glycine substitution mutation by different amino acids in the same codon of COL 7A (G2028R and G2028A)

Neonatal lupus erythematosus presenting as multiple photosensitive annular plaques with skin atrophy

Neonatal lupus erythematosus (NLE) is an autoimmune disease affecting the fetus as a result of transplacental transfer of anti-Ro autoantibodies. Typically, it presents in the first few months of life with an annular form of subacute cutaneous lupus erythematosus. We report an infant of NLE presenting at birth with multiple annular erythematous plaques with skin atrophy involving the face, head, and upper trunk. Histopathology of skin biopsy was consistent with subacute cutaneous lupus. The mother was clinically free of disease and had no family history of autoimmune disease. Serology (extranuclear antigens) was positive in both the baby and the mother. This is a rare presentation of a rare disease.

Cutis marmorata telangiectatica congenita

Cutis marmorata telangiectatica congenita (CMTC) is a rare congenital disorder with persistent cutis marmorata, telengiectasia, and phlebectesia, which may be associated with cutaneaus atrophy and ulceration of the involved skin. We herewith report a three month old male baby with CMTC at birth involving left side of the face, upper limbs, both flanks, and left gluteal and left leg with ulceration over the extensor aspects of the left knee joint. The baby had a reticulated bluish purple skin changes all over the body including the face and limb. Although it resembled physiological cutis marmorata, it was strikingly pronounced and defi n i t e ly was unvarying and permanent. A variety of vascular malformation has been described along with this disorder. Etiology is not very clear. Prognoses in uncomplicated cases are good.

Hipoplasia dérmica focal (Síndrome de Goltz): amplia variabilidad fenotípica / Focal Dermal Hypoplasia (Goltz's Syndrome): Wide Phenotypic Variability

La hipoplasia dérmica focal (MIM# 305600), también llamada hipoplasia mesoectodérmica, es una rara entidad genética con un mecanismo de herencia dominante ligado al cromosoma X. Principalmente compromete piel, sistema esquelético, ojos y cara, con diferentes grados de severidad. Se describe dos casos clásicos e ilustrativos de hipoplasia dérmica focal, observando la amplia heterogeneidad fenotípica que estos pacientes pueden presentar. Hasta el momento es el segundo reporte en la literatura indexada de Colombia. Se realiza una amplia y actualizada revisión de la literatura.

The focal dermal hypoplasia (MIM # 305600), also called mesoectodermica hypoplasia is a rare disease. It is thought to be an X-linked dominant disorder. Mainly undertakes skin, skeletal system, eyes and face, with varying degrees of severity. We describe two cases illustrative of classical and focal dermal hypoplasia, noting the extensive phenotypic heterogeneity that these patients may present. So far is the second report in the literature indexed in Colombia, is a comprehensive and updated review of the literature.