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Milia-like idiopathic calcinosis cutis in a patient with translocation Down syndrome

2001; Elsevier BV; Volume: 45; Issue: 1 Linguagem: Inglês

10.1067/mjd.2001.113456

1097-6787

Tomoe KOTSUJI, Sumihis Imakado, Nobuaki Iwasaki, Hiroshi Fujisawa, Fujio Otsuka,

Cancer and Skin Lesions

To the Editor:A 7-year-old girl with translocation Down syndrome (karyotype 46,XX,der[14;21][q10;q10],+21) was referred to our department for evaluation of multiple asymptomatic papules on her hands, which had been present for 2 years. On examination, small discrete whitish papules, 1 to 3 mm in diameter, were scattered on her fingers, palms, and the dorsal aspect of her hands. Some papules were flesh-colored and scaly. Many small flesh-colored papules were also present on her lower eyelids. A skin biopsy specimen of a papule from her thumb showed amorphous basophilic deposition in the papillary dermis and epidermis. This basophilic deposition was black with von Kossa staining. There were no histologic changes suggestive of syringoma. On the basis of these pathologic findings and clinical features, the case was diagnosed as milia-like idiopathic calcinosis cutis (MICC) in Down syndrome. Serum calcium, phosphate, and parathyroid hormone levels were within the normal range. An antinuclear antibody test was negative. A computed tomographic brain scan and echocardiogram showed no calcification.Table ISummary of reported MICCsAuthorsAge (y)/SexKaryotypeLocation of MICCPerforationSyringoma of palpebralSyringoma adjacent to MICCSano et al3Sano T Tachi S Ishikawa C [Down syndrome with syringomas, milia and subepidermal calcified nodules].Jpn J Dermatol. 1978; 88 (Japanese): 740Google Scholar19/MNRDorsal aspect of handsNRPresent (histologic)NRShibuya et al4Shibuya M Iizuka H Oogawara A Kishiyama K [A case of calcinosis cutis in Down syndrome].Hifurinsho. 1985; 27 (Japanese): 1218-1219Google Scholar12/MNRPalms, left soleYesNRNRSmith & Golitz2Smith ML Golitz LE Milia-like idiopathic calcinosis cutis in Down's syndrome.Arch Dermatol. 1989; 125: 1586Crossref PubMed Scopus (29) Google Scholar6/MNRHands, wrists, elbows, knees, faceNoNRNoMaroon et al5Maroon M Tyler W Marks VJ Calcinosis cutis associated with syringomas: a transepidermal elimination disorder in a patient with Down syndrome.J Am Acad Dermatol. 1990; 23: 372-375Abstract Full Text PDF PubMed Scopus (61) Google Scholar12/MNRHands, forearms, thighsYesPresent (clinical)PresentKanzaki & Nakajima6Kanzaki T Nakajima M Milia-like idiopathic calcinosis cutis and syringoma in Down's syndrome.J Dermatol. 1991; 18: 616-618Crossref PubMed Scopus (31) Google Scholar6/FNRHands, wrists, feetYesPresent (clinical)PresentSchepis et al7Schepis C Siragusa M Palazzo R Batolo D Romano C Perforating milia-like idiopathic calcinosis cutis and periorbital syringomas in a girl with Down syndrome.Pediatr Dermatol. 1994; 11: 258-260Crossref PubMed Scopus (42) Google Scholar11/F47,XX,+21Hands, feetYesPresent (clinical)NoSais et al8Sais G Jucgla A Moreno A Peyri J Milia-like idiopathic calcinosis cutis and multiple connective tissue nevi in a patient with Down syndrome.J Am Acad Dermatol. 1995; 32: 129-130Abstract Full Text PDF PubMed Scopus (33) Google Scholar7/FNRPalmsNoNRNRSchepis et al9Schepis C Siragusa M Palazzo R Batolo D Romano C Milia-like idiopathic calcinosis cutis: an unusual dermatosis associated with Down syndrome.Br J Dermatol. 1996; 134: 143-146Crossref PubMed Scopus (36) Google Scholar10/M47,XY,+21Palms, fingers, soleNoNoPresent11/M47,XY,+21Hands, toesYesNoPresentPatrizi et al10Patrizi A Neri I Raone B Passarini B Calcinose miliaire idiopathique perforante des extremites chez une malade trisomique 21.Ann Dermatol Venereol. 1997; 124: 162-164PubMed Google Scholar6/F47,XX,+21PalmsYesNRNRDelaporte et al11Delaporte E Gosselin P Catteau B Nuyts JP Piette F Bergoend H Calcinose miliaire idiopathique perforante des extremites chez une malade trisomique 21.Ann Dermatol Venerol. 1997; 124: 159-161PubMed Google Scholar15/F47,XX,+21Hands, feetYesNRNRPresent case7/FTranslocationHandsYesNoNoNR, Not reported. Open table in a new tab To the Editor:A 7-year-old girl with translocation Down syndrome (karyotype 46,XX,der[14;21][q10;q10],+21) was referred to our department for evaluation of multiple asymptomatic papules on her hands, which had been present for 2 years. On examination, small discrete whitish papules, 1 to 3 mm in diameter, were scattered on her fingers, palms, and the dorsal aspect of her hands. Some papules were flesh-colored and scaly. Many small flesh-colored papules were also present on her lower eyelids. A skin biopsy specimen of a papule from her thumb showed amorphous basophilic deposition in the papillary dermis and epidermis. This basophilic deposition was black with von Kossa staining. There were no histologic changes suggestive of syringoma. On the basis of these pathologic findings and clinical features, the case was diagnosed as milia-like idiopathic calcinosis cutis (MICC) in Down syndrome. Serum calcium, phosphate, and parathyroid hormone levels were within the normal range. An antinuclear antibody test was negative. A computed tomographic brain scan and echocardiogram showed no calcification.Table ISummary of reported MICCsAuthorsAge (y)/SexKaryotypeLocation of MICCPerforationSyringoma of palpebralSyringoma adjacent to MICCSano et al3Sano T Tachi S Ishikawa C [Down syndrome with syringomas, milia and subepidermal calcified nodules].Jpn J Dermatol. 1978; 88 (Japanese): 740Google Scholar19/MNRDorsal aspect of handsNRPresent (histologic)NRShibuya et al4Shibuya M Iizuka H Oogawara A Kishiyama K [A case of calcinosis cutis in Down syndrome].Hifurinsho. 1985; 27 (Japanese): 1218-1219Google Scholar12/MNRPalms, left soleYesNRNRSmith & Golitz2Smith ML Golitz LE Milia-like idiopathic calcinosis cutis in Down's syndrome.Arch Dermatol. 1989; 125: 1586Crossref PubMed Scopus (29) Google Scholar6/MNRHands, wrists, elbows, knees, faceNoNRNoMaroon et al5Maroon M Tyler W Marks VJ Calcinosis cutis associated with syringomas: a transepidermal elimination disorder in a patient with Down syndrome.J Am Acad Dermatol. 1990; 23: 372-375Abstract Full Text PDF PubMed Scopus (61) Google Scholar12/MNRHands, forearms, thighsYesPresent (clinical)PresentKanzaki & Nakajima6Kanzaki T Nakajima M Milia-like idiopathic calcinosis cutis and syringoma in Down's syndrome.J Dermatol. 1991; 18: 616-618Crossref PubMed Scopus (31) Google Scholar6/FNRHands, wrists, feetYesPresent (clinical)PresentSchepis et al7Schepis C Siragusa M Palazzo R Batolo D Romano C Perforating milia-like idiopathic calcinosis cutis and periorbital syringomas in a girl with Down syndrome.Pediatr Dermatol. 1994; 11: 258-260Crossref PubMed Scopus (42) Google Scholar11/F47,XX,+21Hands, feetYesPresent (clinical)NoSais et al8Sais G Jucgla A Moreno A Peyri J Milia-like idiopathic calcinosis cutis and multiple connective tissue nevi in a patient with Down syndrome.J Am Acad Dermatol. 1995; 32: 129-130Abstract Full Text PDF PubMed Scopus (33) Google Scholar7/FNRPalmsNoNRNRSchepis et al9Schepis C Siragusa M Palazzo R Batolo D Romano C Milia-like idiopathic calcinosis cutis: an unusual dermatosis associated with Down syndrome.Br J Dermatol. 1996; 134: 143-146Crossref PubMed Scopus (36) Google Scholar10/M47,XY,+21Palms, fingers, soleNoNoPresent11/M47,XY,+21Hands, toesYesNoPresentPatrizi et al10Patrizi A Neri I Raone B Passarini B Calcinose miliaire idiopathique perforante des extremites chez une malade trisomique 21.Ann Dermatol Venereol. 1997; 124: 162-164PubMed Google Scholar6/F47,XX,+21PalmsYesNRNRDelaporte et al11Delaporte E Gosselin P Catteau B Nuyts JP Piette F Bergoend H Calcinose miliaire idiopathique perforante des extremites chez une malade trisomique 21.Ann Dermatol Venerol. 1997; 124: 159-161PubMed Google Scholar15/F47,XX,+21Hands, feetYesNRNRPresent case7/FTranslocationHandsYesNoNoNR, Not reported. Open table in a new tab A 7-year-old girl with translocation Down syndrome (karyotype 46,XX,der[14;21][q10;q10],+21) was referred to our department for evaluation of multiple asymptomatic papules on her hands, which had been present for 2 years. On examination, small discrete whitish papules, 1 to 3 mm in diameter, were scattered on her fingers, palms, and the dorsal aspect of her hands. Some papules were flesh-colored and scaly. Many small flesh-colored papules were also present on her lower eyelids. A skin biopsy specimen of a papule from her thumb showed amorphous basophilic deposition in the papillary dermis and epidermis. This basophilic deposition was black with von Kossa staining. There were no histologic changes suggestive of syringoma. On the basis of these pathologic findings and clinical features, the case was diagnosed as milia-like idiopathic calcinosis cutis (MICC) in Down syndrome. Serum calcium, phosphate, and parathyroid hormone levels were within the normal range. An antinuclear antibody test was negative. A computed tomographic brain scan and echocardiogram showed no calcification. NR, Not reported.