皮膚病診療 41巻5号 (2019年5月)

  • 文献概要を表示

・メチシリン耐性黄色ブドウ球菌(Methicillin-resistant Staphylococcus aureus:MRSA)によるブドウ球菌性熱傷様皮膚症候群(staphylococcal scalded skin syndrome:SSSS)で入院した姉から母親と弟へ家族内伝播したと考えられる親子3人のMRSA感染症を経験した.

・MRSA感染症は院内獲得型で,日和見感染として認識されてきたが,近年,市中獲得型が増加し問題となっている.

・MRSA感染症ではセフェム系等の抗菌薬で皮膚症状が軽快したのちも,鼻腔内保菌による伝播がおこりうることに注意が必要である.

(「症例のポイント」より)

 

Staphylococcal scalded skin syndrome due to MRSA spread in the family

 

Kubo, Naoko1)Ogawa, Tetsushi2)Ito, Junko2)Hayashi, Nobukazu1) 1)Department of Dermatology, Toranomon Hospital 2)Department of Pediatrics, Toranomon Hospital

 

A two-year-old girl complained of fever, erythema, and ocular mucous and was hospitalized due to staphylococcal scalded skin syndrome caused by methicillin-resistant Staphylococcus aureus (MRSA). Her symptoms disappeared after treatment with cefazolin. One month after discharge, her 4-month-old younger brother and their mother were hospitalized with similar symptoms. MRSA was detected in both their nasal cavities. The mother had been nursing during her son’s hospitalization. Therefore, we supposed that the mother became a carrier of MRSA, which was contracted from her daughter;then, MRSA spread from her nasal cavity to her son’s skin at home. Our case findings indicate that MRSA should be completely treated to prevent familial spread.

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・保育器管理下における超低出生体重児のAspergillus nigerによる原発性皮膚アスペルギルス症を経験した.

・背部に褐色膜状の鱗屑痂皮が脱落してできた潰瘍があり,周囲に黒色壊死物質が付着していた.

・ラノコナゾール軟膏外用とミコナゾール硝酸塩配合液体石鹸による洗浄を行った.

(「症例のポイント」より)

 

Primary cutaneous aspergillosis caused by Aspergillus niger –An extremely low birth weight preterm infant in an incubator–

 

Mukai, Mirei1)Miyazaki, Natsumi2)Iwami, Hiroko3)Fukai, Kazuyoshi4) 1)Department of Dermatology, Fuchu Hospital 2)Department of Nursing, Osaka City General Hospital 3)Department of Neonatology Osaka City General Hospital 4)Department of Dermatology, Osaka City General Hospital

 

The primary cutaneous aspergillosis is rare in premature infant. We experienced the case of 22 weeks and 3 days gestational age, birth weight 325g female infant, who was treated in humidified incubator at NICU. She developed epidermal exfoliation and white necrotic tissue in the surroundings on her back at 7 days of age. A skin ulcer with a black necrotic substance was noted on her back when we were consulted on 18 days. Aspergillus niger was detected on the skin culture test. The presence of a skin ulcer or black necrotic tissue on the back of an extremely low birth weight preterm infant may indicate the primary cutaneous aspergillosis.

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・BCG接種後の乳児に生じた丘疹状結核疹を経験した.

・皮疹部の病理組織像では類上皮細胞性肉芽腫を認め,Ziehl-Neelsen染色では抗酸菌は明らかではなかった.

・ステロイド外用および抗ヒスタミン薬内服にて経過観察し,皮疹は瘢痕を残さずに治癒した.

(「症例のポイント」より)

 

A case of papular tuberculid after Bacille Calmette-Guerin (BCG) vaccination

 

Nakashima, Mai1)Watanabe, Shoichi1) 1)Department of Dermatology, Kainan Hospital

 

We report a 9-month boy presenting with scattered papules across the whole body 7 weeks after receiving a BCG vaccination on his left brachium. A biopsy taken from the scattered papule showed epithelioid cell granuloma, and no mycobacteria in Ziehl-Neelsen staining. We diagnosed him with papular tuberculid. All skin lesions were restored without scarring by the treatment with topical steroid and taking antihistamine.

生後1カ月乳児の疥癬 原 彰吾
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・生後1カ月の乳児の疥癬を経験した.

・産科退院後,母親の実家で里帰り中に,3カ月前にイオウ含有入浴剤で疥癬の自己治療歴がある祖父から感染した,と推測した.

・治療はフェノトリンが著効した.顔面,頭部を含む全身に塗布し,塗布後は手にベビーミトンを着用した.今後の小児への適用拡大が期待される.

・小児の疥癬の場合,保護者への適切な外用方法の指導,および実践できているか否かの確認が重要なことを再認識した.

(「症例のポイント」より)

 

A case of scabies in a one-month-old infant

 

Hara, Shogo1) 1)Hara Dermatology Clinic

 

A 1-month-old male was brought to our hospital because he had been treated with a topical steroid preparation for a skin eruption for 2 weeks in a pediatric department before his initial examination, but it had become worse. Scattered brown papules and small nodules were observed, mainly over his right lower limb and right foot. A direct microscopy examination was positive for scabies mites. After discharge from the obstetrics department the patient resided in his mother’s parents’ home, where his grandfather lived with them. His grandfather had a history of treatment for scabies at another hospital 3 months before, and a strong possibility of transmission from his grandfather was inferred. The patient responded to treatment with phenothrin. Phenothrin is recommended for infants under 2 months of age by the Guideline for the diagnosis and treatment of scabies in Japan (third edition)of the Japanese Dermatological Association.

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・ステロイド外用薬で難治の皮疹は疥癬を鑑別する必要がある.

・疥癬と診断した際は家族や濃厚接触者にも同症状がないか必ず確認をする.

・ダーモスコピーが疥癬診断に有用であった.

・乳児の疥癬治療にフェノトリンローションを用いた.

(「症例のポイント」より)

 

Scabies infestation in a family including an infant: A case report

 

Iwanaga, Atsuko1)Inamo, Akiko1)Fukai, Tatsuo1) 1)Department of Dermatology and Allergy, Juntendo University Nerima Hospital

 

A 3-month-old male infant presented with a skin rash affecting his limbs since the age of 1 month. He was treated with topical steroids by his pediatrician; however, he did not improve. He visited our hospital with his mother because his father was diagnosed with scabies at our hospital. Examination showed red papules scattered on his face, trunk, and limbs. Scales and crusts were observed predominantly on his palmoplantar regions and the medial surface of the thighs. Several scabies burrows were identified at these aforementioned sites, and he was diagnosed with scabies Notably, his mother was also diagnosed with scabies. We treated him and his mother with 5% phenothrin lotion and both showed symptomatic improvement. Dermoscopy was useful to diagnose these patients.

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・川崎病治療後に発症した乳児好酸球性膿疱性毛包炎(Eosinophilic pustular folliculitis:EPF)を経験した.

・持続する好酸球増多を認め,毛包周囲に好酸球の著明な浸潤を認めた.

・プレドニゾロン(PSL)とスプラタストトシル酸塩内服,ステロイド外用により,好酸球増多,皮疹ともに改善した.

(「症例のポイント」より)

 

A case of infantile eosinophilic pustular folliculitis with marked eosinophilia which accompanied treatment of Kawasaki disease

 

Sato, Atsuko1)Komine, Mayumi1)Murata, Satoru1)Ohtsuki, Mamitaro1) 1)Department of Dermatology, School of Medicine, Jichi Medical University

 

A 3-month-old boy was consulted due to erythema, papules, pustules spread on the abdomen, the face and the head along with peripheral blood eosinophilia. He was diagnosed as Kawasaki disease when he was 2-month-old. Although it was refractory to IVIG, which had been tried five times in total, fever and inflammatory reaction subsided with concomitant administration of cyclosporine in one month after the onset of the disease. Three days after the termination of cyclosporine treatment, however, erythema developed on the trunk and spread to whole body. Histopathologically, there were significant infiltrations of eosinophils in the dermis and hair follicles. Both skin rash and peripheral blood eosinophilia were improved with oral prednisolone 0.2 mg/kg/day.

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・当科で経験した新生児期発症の皮膚肥満細胞症の2例を報告する.

・自験例では,肥満細胞の増殖活性や悪性転換にかかわっていると推測されている,4つのホットスポット領域を含むc-kit遺伝子変異を同定できなかった.

・c-kit遺伝子変異を有する肥満細胞は,発症時期や罹患期間によってclonalityのレベルが異なり,変異の同定率に関与している可能性が推測される.

(「症例のポイント」より)

 

Two newborn cases of cutaneous mastocytosis who undergone c-kit gene mutation analysis

 

Uesugi, Saori1)Chino, Takenao1)Utsunomiya, Natsuko1)Utsunomiya, Akira1)Oyama, Noritaka1)Hasegawa, Minoru1) 1)Department of Dermatology, Faculty of Medical Sciences, University of Fukui

 

Two 5-month-old Japanese infants presented a birth-history of brownish maculo-papules spreading over their entire body. The lesional skin biopsy revealed intense accumulation of mast cells in the upper dermis, all of these were positive for tyrosine kinase receptor KIT on immunohistochemistry, suggestive of urticaria pigmentosa. We performed mutation analysis for c-kit gene by direct sequencing using genomic DNA from paraffin-embedded skin samples, to check the four distinct hot spot missense mutations, codons 816, 560, 820, and 839. No mutations were detectable in both cases. We discussed the perspective association between c-kit gene mutation and disease phenotypes, as well as clinical courses and outcome, in cutaneous mastocytosis.

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・乳幼児期に発症した難治性の凍瘡の症例を経験した.

・凍瘡の治療は一般的には対症療法であり寒冷刺激を避け,保湿剤などで対症療法を行う.

・難治性の場合,凍瘡様皮疹をきたすさまざまな疾患を鑑別することが重要である.

(「症例のポイント」より)

 

Differential diagnosis of refractory chilblain in infancy

 

Ebato, Takasuke1)Bando, Yuki2) 1)Department of Pediatrics, Kitasato University School of medicine 2)Department of Pediatrics, Kitasato University Medical Center

 

A 1-year old boy developed many small erythema and ulcers on his paw and toes in winter. Histological examination revealed inflammatory cells mainly lymphocytes infiltrated around the vascular of the dermis, and the papillary dermis changed edematous. Those findings were consistent with pathogenesis of chilblain. The many evaluations were performed because the symptom was persistent with treatment and he had family history of chilblain. So far he was not diagnosed with auto-inflammatory diseases, however it should be necessary to careful observation.

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・新生児エリテマトーデス(NLE:neonatal lupus erythematosus)の兄弟例を経験した.母親は無症状であったが,第1子の環状紅斑を契機に精査し,無症候性Sjögren症候群と診断した.2年後に出産した第2子の男児にも生後3週より耳介の紅斑が出現し,NLEと診断した.

・NLEでは皮膚症状以外に,不可逆性の先天性心ブロックを生じることがあり,次子以降の妊娠出産時に慎重な対応が必要となる.

(「症例のポイント」より)

 

A case of siblings with neonatal lupus erythematosus

 

Nakamura, Yuka1)Takahata, Hiroaki1)Terachi, shinichi2) 1)Department of Dermatology, Yamaguchi Red Cross Hospital 2)Department of Pediatrics, Yamaguchi Red Cross Hospital

 

A 6-week-old male infant, who was the first-born infant of a 25-year-old woman, presented with erythematous annular lesions on the face and abdomen. He had positive for anti-SS-A and anti-SS-B antibodies, and was diagnosed with neonatal lupus erythematosus (NLE). His mother did not complain of any symptoms, but she was examined and diagnosed with subclinical Sjögren’s syndrome. Two years later, the patient’s younger brother, who was the second-born infant of the mother, presented with skin eruptions on the ears at 3 weeks after birth. He was also diagnosed with NLE due to positivity for anti-SS-A and anti-SS-B antibodies. Because NLE may induce irreversible congenital heart block in addition to skin symptoms, their mother needs to be carefully followed at the time of her next pregnancy and delivery.

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・ロリクリン角皮症は山本ら1)によって提唱された疾患概念で,角層細胞の周辺帯を形成するロリクリンという表皮のタンパク構造をコードするLOR(ロリクリン)遺伝子の変異によって生じる,非常にまれな常染色体優性遺伝性の角化異常症である.本症は,これまでに,世界でも18家系しか報告されていない2〜6).

・自験例は先天性魚鱗癬様紅皮症との鑑別を要し,全エクソームシークエンスによってLOR遺伝子変異を同定したことでロリクリン角皮症と確定診断した.今回の変異はc.684dupGで,ロリクリン角皮症の原因遺伝子変異として8家系において報告されている2).

(「症例のポイント」より)

 

A case of loricrin keratoderma

 

Ito, Yasutoshi1)Takeichi, Takuya1)Akiyama, Masashi1) 1)Department of Dermatology, Nagoya University

 

The patient is an 8-month-old Japanese girl, the first child born to non-related parents. The birth weight was 2898g. She was noticed to have collodion membranes at birth and presented with symptoms of ichthyosis after birth. In addition, she had palmoplantar keratoderma. There was no blistering. Initial Sanger sequencing of TGM1, ALOXE3 and ALOX12B implicated in congenital ichthyosiform erythroderma did not reveal any mutations. Whole-exome sequencing revealed a previously reported heterozygous frameshift mutation in LOR, c.684dupG. From these findings, we diagnosed her as loricrin keratoderma.

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・乏汗症,歯牙欠損,乏毛を呈したX連鎖性低汗性外肺葉形成不全症(hypo- or anhidrotic ectodermal dysplasia:HED)の1家系を経験した.

・EDA遺伝子解析にて確定診断に至った.

・自験例で同定されたsplice site変異c.924+7A>Gは新規変異であった.

(「症例のポイント」より)

 

A novel EDA mutation identified in a patient with X-linked hypohidrotic ectodermal dysplasia

 

Mizawa, Megumi1)Makino, Teruhiko1)Shimomura, Yutaka2)Adachi, Yuichi3)Shimizu, Tadamichi1) 1)Department of Dermatology, Graduate School of Medicine and Pharmaceutical Sciences, University of Toyama 2)Department of Dermatology, Yamaguchi University Graduate School of Medicine 3)Department of Pediatrics, Graduate School of Medicine and Pharmaceutical Sciences, University of Toyama

 

A six-month-old Japanese boy presented with recurrent episodes of a high body temperature. He also presented with hypohidrosis, sparse scalp hair and eyebrows, a prominent forehead and a saddle nose. X-ray showed hypodontia. Minor’s starch test showed only slight sweating on his palms. We performed a sequence analysis of the EDA gene using peripheral blood samples after obtaining written informed consent from the patient’s parents. A novel hemizygous splice site mutation c.924+7A>G was subsequently detected in intron 7 of the EDA gene. His mother was heterozygous for the mutation. These findings were diagnostic of X-linked recessive hypohidrotic ectodermal dysplasia.

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・特徴的な顔貌を有する歌舞伎症候群の典型例を経験した.

・原因としてKMT2D遺伝子が同定された.

・皮膚症状として指尖隆起を認めた.

(「症例のポイント」より)

 

A case of Kabuki syndrome

 

Okuma, Mika1)Sasaki, Keiko1)Toi, Yoichiro1)Kimura, Naritaka2)Nagaoka, Yoshiharu3)Miyake, Noriko4) 1)Department of Dermatology, Hiroshima City Hiroshima Citizens Hospital 2)Department of Plastic Surgery, Hiroshima City Hiroshima Citizens Hospital 3)Department of Pediatrics, Hiroshima City Hiroshima Citizens Hospital 4)Department of Genetics, Yokohama City University of Medicine

 

An 18 months girl presented with cleft lip and cleft palate. Her eyelids were extended and lower eyelids were fluttered. After genetic test KMT2S gene has been detected, we diagnosis her with Kabuki syndrome. Kabuki syndrome is a rare, multisystem disorder characterized by multiple abnormalities including distinctive facial features, growth delays, varying degrees of intellectual disability, skeletal abnormalities, and short stature. She had also deafness and breast enlargement.

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・Langerhans cell histiocytosis(LCH)は病変部位と病変数による分類が近年頻用されており,自験例では症例1と症例2で異なる分類,治療方針となった.

・症例2は皮疹からLCHの診断に至った症例であり,まれな疾患ではあるものの,小児の難治性皮疹をみた場合,鑑別としてあげるべきと考えた.

(「症例のポイント」より)

 

Two cases of infantile Langerhans cell histiocytosis

 

Nakagawa, Tomoe1)Hori, Masako2)Kanno, Kyoko2)Yamamoto, Akemi2) 1)Department of Dermatology, Nayoro City General Hospital 2)Department of Dermatology, Asahikawa Medical University

 

We report two cases of Langerhans cell histiocytosis (LCH). Case 1: A seven-month-old girl was admitted to our hospital with a pre-auricular tumor and purpura on chest. Histopathologic examination revealed a proliferation of Langerhans cells. A diagnosis of LCH was made. These symptoms remitted with a combination therapy. Case 2:A six-month-old boy presented with papules, purpura, and depigmentation on the face and trunk. Histopathological examination revealed an increased number of histiocytic cells in the epidermis and the papillary dermis. The patient was diagnosed with LCH and the lesions resolved spontaneously.

editorial

令和元年によせて 浅井 俊弥

  • 文献概要を表示

新生児~乳児期にみられる皮膚疾患には,新生児特有の生理的なものから,先天性の遺伝性皮膚疾患や腫瘍までさまざまあり,それらを即時に鑑別することが求められる.母体の羊水の中で感染や乾燥から完全に守られていた胎児の皮膚は,出産と同時に乾燥,感染,摩擦のような外力などの危険にさらされる.この大きな環境の変化に適応していく激動の時期に,さまざまな生理的皮膚変化が生じるが,それらは一過性で治療の必要がなく,自然消褪を示すものも多い.一方,出生時ないし生後まもなくから現れる遺伝性皮膚疾患,炎症性疾患,感染症,腫瘍などもあり,それらを正確に,即座に診断し適切な治療を開始する必要がある.(冒頭より)

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伝染性膿痂疹は小児に好発する表在性細菌感染症である.夏季に多く虫刺されや外傷をきっかけに紅斑,水疱,びらんを生じる.水疱が飛び火するため,別名「とびひ」と呼ばれる.原因菌に黄色ブドウ球菌とA群β溶血性レンサ球菌があるが,近年メチシリン耐性黄色ブドウ球菌(MRSA)によるものが増加している.臨床像から水疱性膿痂疹と痂皮性膿痂疹に分類され,痂皮性膿痂疹は全身症状(発熱,所属リンパ節腫脹,咽頭発赤,咽頭痛など)を伴うことがある.従来,水疱性膿痂疹の場合は黄色ブドウ球菌,痂皮性膿痂疹の場合はA群β溶血性レンサ球菌がそれぞれ原因菌とされてきた.しかし,痂皮性膿痂疹では黄色ブドウ球菌とA群β溶血性レンサ球菌の両方が分離されることがあり,臨床像と原因菌は必ずしも一致しないとの報告もある1).また,原因菌がA群β溶血性レンサ球菌の場合は後日,糸球体腎炎を併発することがあるため尿検査が必要である.原因菌を特定することは治療や合併症を考えるうえで重要である.  伝染性膿痂疹はMRSAが原因となることがあり,治療に難渋することがある.起因菌がMRSAの際の抗菌薬選択には感受性試験結果が重要となるが,治療対象は小児である.小児には使用しづらい薬剤も多い.本稿では伝染性膿痂疹の治療として統計学的結果をもとに検討した.(「はじめに」より)

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巨大色素性母斑は2~5万出生に1人の発生率1, 2)で,女児にやや多く,成長とともに皮膚の獣皮様変化をきたすため,整容性が問題となることに加え,若年期における悪性化の危険性から早期切除が推奨されている.しかし,母斑細胞は皮下脂肪織にまで及ぶとされており,全切除は困難な症例も少なくない.  われわれは,このような巨大色素性母斑に対して新生児期から治療を開始しているので,その方法と結果について報告する.(「はじめに」より)

皮心伝心

いま皮膚科が面白い 山中 恵一

診察室の四季

新緑 斉藤 隆三

皮膚科のトリビア

第167回 浅井 俊弥

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目次

編集後記・次号予告

基本情報

pd-41-5_cover.jpg
皮膚病診療
41巻5号 (2019年5月)
電子版ISSN:2434-0340 印刷版ISSN:0387-7531 協和企画

文献閲覧数ランキング(
9月14日~9月20日
)