Infantile Systemic Hyalinosis.

Infantile systemic hyalinosis is a rare disorder characterized by widespread deposition of hyaline. They usually present with skin lesions, joint contractures, and intractable diarrhea. We report a 2 year 4 month old boy with growth retardation, typical facial appearance, gingival enlargement, generalized stiff skin, joint contractures, and intermittent diarrhea. Skin biopsy revealed deposition of hyaline.

Prognostic factors for skin-involved inflammatory and non-inflammatory breast cancer / 中华放射肿瘤学杂志

Objective To analyze the prognostic factors and the role of postmastectomy radiotherapy (PMRT) in skin-involved breast cancer.Methods Fifty-three skin-involved breast cancer patients treated with mastectomy and axillary dissection were retrospectively analyzed.Ten patients had inflammatory breast cancer (IBC).Of the 43 non-inflammatory breast cancer (NIBC) patients, 19(36%) had clinical signs of skin involvement and 24(45%) had pathological skin infiltration without clinical signs.Thirty-three patients (62%) received PMRT with a median dose of 50 Gy, 45 received chemotherapy and 27 received hormone therapy.Results The median follow up time for alive patients was 42 (7 -83) months.The overall 5-year locoregional recurrence (LRR),disease-free survival (DFS) and overall survival (OS) rates were 18%,45% and 64%.Patients with NIBC had a significantly better 5-year DFS (49% vs.30% ,χ2=4.29,P=0.038) and OS (71% vs.37% ,χ2=5.92,P=0.015) than those with IBC.In patients with NIBC, those with primary tumor ≤5 cm had a lower 5-year LRR (11% vs.33% ,χ2= 3.75 ,P =0.053) and a higher 5-year OS (90% vs.38% ,χ2=4.44,P=0.035) as compared to those >5 cm.No significant difference in terms of LRR, DFS or OS was observed between patients with clinical signs of skin involvement and those without.Patients with 0 - 3 positive nodes had an improved 5-year DFS (80% vs.29%, χ2= 6.71, P =0.010) and OS (93% vs.52% ,χ2=6.00,P=0.014) than those with ≥4 positive nodes.Patients with Rec + / Her2 - had a lower5 - year LRR (7 % vs.3 4 % , χ2= 5.70, P= 0.017) and a higher DFS (54% vs.32% ,χ2=8.82 ,P =0.003) than those with triple-negative or Her2 +.There was no significant difference in 5-year LRR (12% vs.30%, χ2= 2.45, P = 0.118) between patients with PMRT and without PMRT.However, the 5-year chest wall recurrence rate was 0% and 50% (χ2= 9.15 ,P =0.002) for patients with chest wall bolus dose > 20 Gy and 20 Gy.Conclusions Skin-involved breast cancer is a heterogeneous group of diseases.Patients with NIBC, small tumor (≤5 cm), less positive nodes (0 -3) or rec +/Her2-have favorable prognosis.Patients with pathologically proven skin involvement without clinical signs have similar prognosis to those with clinical signs.PMRT with chest wall bolus dose >20 Gy is recommended.

Tacrolimus tópico nas lesões cutâneas refratárias da dermatomiosite juvenil / Topical tacrolimus in refractory skin lesions of juvenile dermatomiosytis

A dermatomiosite juvenil (DMJ) é uma doença inflamatória crônica idiopática que afeta principalmente músculos e pele. As lesões cutâneas podem persistir apesar do controle bem-sucedido da miosite. O tacrolimus tópico é um novo agente imunossupressor que tem sido usado no tratamento da dermatite atópica, com poucos relatos de seu uso em miopatias inflamatórias na faixa etária pediátrica. Foram descritos três pacientes com DMJ (dois meninos) com idade atual de 5,7 a 10,6 anos. A terapia inicial administrada para esses pacientes foi: corticosteróide (sistêmico em três e tópico em um), antimalárico em três e metotrexato em dois. Todos apresentavam lesões cutâneas refratárias (eritema malar, eritema difuso e/ou vasculite cutânea) após melhora significativa da fraqueza muscular. Tacrolimus tópico 0,03 por cento foi usado duas vezes ao dia após falha do tratamento prévio. As características das lesões foram avaliadas de acordo com a extensão e a gravidade no início do uso do fármaco e após oito e 16 semanas. Na segunda avaliação (oito semanas), notavelmente um paciente apresentou melhora completa do eritema malar e do eritema difuso em membros e tronco, e dois tiveram melhora parcial do eritema malar e da vasculite cutânea. Na terceira avaliação (16 semanas), dois pacientes tiveram resolução completa das lesões e um apresentava eritema malar persistente e eritema moderado em glúteos e região lombar. No último paciente, a melhora das lesões cutâneas foi alcan-çada apenas após o uso de ciclosporina por 16 semanas. Nenhum deles teve efeitos adversos. Tacrolimus tópico pode ser considerado em pacientes com DMJ e manifestações cutâneas refratárias. Estudos posteriores controlados e randomizados com esse fármaco devem ser realizados nessa doença inflamatória.

Juvenile dermatomyositis (JDM) is a rare idiopathic chronic inflammatory disease that affects mainly muscle and skin. Cutaneous lesions may persist despite successful treatment of myositis. Of note, topical tacrolimus is a new immunosuppressive agent that has been used to treat atopic dermatitis with few reports in pediatric inflammatory myopathies. Three JDM patients (two males) were described, current age from 5.7 to 10.6 years. The initial therapy administered for these patients were: corticosteroid (oral in three and topical in one), antimalarial in three and methotrexate in two. All of them had refractory skin lesions (malar rash, extensive rash and/or cutaneous vasculitis) after significant improvement of muscle weakness. Topical tacrolimus 0.03 percent was used twice daily after failure of previous treatment. The features of lesions were evaluated according to extension and severity at start of drug and after 8 and 16 weeks. At the second evaluation (8 weeks), remarkably one patient had complete improvement of malar rash and extensive rash in limbs and trunk, and two had partial improvement of malar rash and cutaneous vasculitis. At the third evaluation (16 weeks), two patients had complete resolution of lesions and one had persistent malar rash and moderate lumbar and gluteus rash. In the last patient, the improvement of skin lesions was reached only after 16 weeks of cyclosporine use. None of them had adverse effects. Topical tacrolimus could be considered in JDM patients with refractory cutaneous manifestations. Further randomized controlled trials with this agent should be performed in this inflammatory disease.

Invasions and Metastases of Facial and Neck Skin by Head and Neck Cancer / 대한이비인후과학회지

BACKGROUND AND OBJECTIVES: This study was designed to review the incidence of skin involvement in the head and neck cancer. We also reviewed its pattern, treatment results, and prognostic significance of skin invasion and metastases. MATERIALS AND METHODS: We carried out an retrospective evaluation of 41 patients who required resection of the facial or neck skin during surgery for their head and neck cancer in the last ten years. Patients with head and neck skin cancer were excluded from this study. Patients who developed skin involvement were evaluated with regard to the primary origin of tumor, the tumor stage, and the pattern of skin involvement, treatment and outcome. The cases were classified into four groups: firstly, a group with the presence of skin scar made by previous biopsy; secondly, a group with tumor fixed to skin; thirdly, a group with skin resected with a sufficient resection marginal, and lastly a group with gross involvement of skin by tumor. RESULTS: Among 41 patients who required resection of the skin, patients with skin invasion were noted in 24 cases. Only one out of four cases of the biopsy group had skin involvement whereas a half of the fixed group had skin invasion. For the marginal group, only two cases had invasion of skin. The treatment result of patients with skin involvement had poor prognosis compared to patients without skin involvement. In particular, patients with skin metastasis had extremely poor survival. CONCLUSIONS: Skin involvement in the head and neck cancer indicates a poor prognosis, and should not be considered for surgery.

Two Cases of Systemic Candidiasis in Very Low Birth Weight Infants / 대한의진균학회지

Reports of systemic candidiasis in neonates have been noted with major advances in neonatal care permitting the frequent survival of low birth weight infants. We experienced 2 cases of systemic candidiasis associated with skin involvement in very low birth weight infants. One case presented with abdominal distension and an erythematous maculopapular eruption on trunk, buttock, and lower extremities. Culture of peritoneal fluid grew Candida of albicans. In another case, a diffuse erythematous patch was noted on the back and buttock. Culture of blood sample grew Candida of albicans. Despite the treatment with intravenous fluconazole, clinical status of the patients continued to remain unstable, and they died.

A Case of Systemic Candidiasis with Skin Manifestations / 대한의진균학회지

Candidiasis is the most common fungal infection complicating the course of the patients with hematologic malignancies. Although widespread organ involvement is characteristic of systemic candidiasis, report of skin involvement is rare. A 22-year-old man with acute lymphocytic leukemia showed fever and painful erythematous maculopapular eruptions on the whole body after combination chemotherapy of mitoxanthrone, cytosine arabinoside and etoposide. Histopathologic sections from the skin lesions showed sparse perivascular mononuclear cell infiltration in the dermis, and many blastospores and pseudohyphae within the dermis and subcutis. The patient died in spite of treatment with intravenous amphotericin B and oral flucytosine.

Evaluation of risks of skin sparing breast conserving surgery for early stage breast cancer / 解放军医学杂志

Objective To evaluate the oncologic risks of skin sparing breast conserving surgery for carcinoma of breast at early stage.Methods Serial sections of skin overlying the tumor were done for pathological examination in 50 cases of early stage breast cancer after skin sparing breast conserving operation.Results In 50 cases of breast conserving surgery,the extent of skin removal was 0.5-1.0cm in 21 cases,1.0-1.5cm in 25 cases,and 1.5-2.0cm in 4cases.Skin involvement was found in one patient with stage Ⅱ breast cancer,and the rate of skin involvement was 2%.Skin involvement was closely related with the changes in skin overlying the tumor,but not with the tumor size,pathologic types or axillary lymph node metastasis.Conclusion The rate of skin involvement in early stage breast cancer is rare,therefore if there are no abnormal clinical manifestations in the skin,skin sparing breast conserving surgery is safe.