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whitish&#44; shiny homogeneous structures&#44; with no visible signs of vascularization &#40;<a class="elsevierStyleCrossRef" href="#fig0010">Fig&#46; 2</a>&#41;&#46; Histological examination &#40;hematoxylin&#8211;eosin&#41; of a shave biopsy of one of the lesions confirmed the presence of extensive basophilic deposition in the mid-dermis surrounded by collagen fibers and accompanied by epidermal hyperplasia and parakeratosis &#40;<a class="elsevierStyleCrossRef" href="#fig0015">Fig&#46; 3</a>&#41;&#46; Blood tests&#44; including a metabolic and hormonal profile&#44; revealed no abnormalities&#46;</p><elsevierMultimedia ident="fig0005"></elsevierMultimedia><elsevierMultimedia ident="fig0010"></elsevierMultimedia><elsevierMultimedia ident="fig0015"></elsevierMultimedia><p id="par0015" class="elsevierStylePara elsevierViewall">What Is Your Diagnosis&#63;</p><span id="sec1015" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect1010">Diagnosis</span><p id="par0060" class="elsevierStylePara elsevierViewall">Milia-like idiopathic calcinosis cutis</p></span><span id="sec0015" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0010">Diagnostic Process&#44; Clinical Course&#44; and Treatment</span><p id="par0020" class="elsevierStylePara elsevierViewall">Histology revealed an amorphous crystalline basophilic deposit in the mid dermis&#44; surrounded by collagen fibers&#46; The surrounding tissue was unaffected&#46; Calcium deposition was suspected and von Kossa staining was performed&#44; revealing a blackish tone&#44; which confirmed diagnosis of calcinosis cutis&#46; Calcinosis cutis is defined as the deposition of calcium salts in the skin and subcutaneous tissue&#46; Laboratory tests revealed normal serum levels of calcium&#44; phosphorus&#44; vitamin D&#44; and parathyroid hormone&#46; In the absence of any other underlying cutaneous or systemic disease&#44; a definitive diagnosis of idiopathic calcinosis cutis was established&#46; The patient underwent clinical observation and remains stable&#44; with a similar number of lesions after 1 year of follow-up&#46;</p></span><span id="sec0020" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0015">Comment</span><p id="par0025" class="elsevierStylePara elsevierViewall">Calcinosis cutis is classified into 5 categories according to pathophysiology&#58; dystrophic&#44; metastatic&#44; idiopathic&#44; iatrogenic and mixed&#46;<a class="elsevierStyleCrossRef" href="#bib0035"><span class="elsevierStyleSup">1</span></a> To confirm the idiopathic form&#44; it is necessary to rule out underlying tissue lesions&#44; which distinguish it from dystrophic calcification&#44; as well as alterations in calcium and phosphorus metabolism typical of metastatic calcification&#46;</p><p id="par0030" class="elsevierStylePara elsevierViewall">Milia-like idiopathic calcinosis cutis is a benign and infrequent subtype &#40;with only 32 cases reported in the literature&#41; that was defined in 1978 and is described mainly in pediatric patients of both sexes &#40;mean age&#44; 12 y&#41; with Down syndrome &#40;62&#46;5&#37; of cases&#41;&#46;<a class="elsevierStyleCrossRef" href="#bib0040"><span class="elsevierStyleSup">2</span></a> However&#44; isolated cases have also been described in adults<a class="elsevierStyleCrossRef" href="#bib0045"><span class="elsevierStyleSup">3</span></a> and in patients without Down syndrome&#46;<a class="elsevierStyleCrossRef" href="#bib0050"><span class="elsevierStyleSup">4</span></a></p><p id="par0035" class="elsevierStylePara elsevierViewall">The cause is unknown&#44; although various hypotheses have been proposed&#44; including persistent localized trauma&#44;<a class="elsevierStyleCrossRef" href="#bib0055"><span class="elsevierStyleSup">5</span></a> calcium precipitation in the acrosyringium secondary to increased sweat calcium levels&#44; and calcium deposition in persistently inflamed epidermal cysts&#46;</p><p id="par0040" class="elsevierStylePara elsevierViewall">The clinical presentation is characterized by multiple&#44; small&#44; whitish&#44; round firm papules&#44; which usually appear on the hands and feet but can also affect other areas&#44; and can infrequently develop on the face&#46; Clinically&#44; this condition can be easily confused with other entities&#44; including molluscum contagiosum&#44; viral warts&#44; epidermal inclusion cysts&#44; and syringomas&#46; Although the definitive diagnosis is histological&#44; a petaloid appearance on dermoscopy may be helpful&#44;<a class="elsevierStyleCrossRef" href="#bib0060"><span class="elsevierStyleSup">6</span></a> but this is not pathognomonic&#46;</p><p id="par0045" class="elsevierStylePara elsevierViewall">The condition is characterized by clustered white formations that lack vasculature&#44; 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Vol. 113. Núm. 10.
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Vol. 113. Núm. 10.
Páginas T959-T960 (noviembre - diciembre 2022)
Cases for Diagnosis
Open Access
White Facial Papules in a Boy
Pápulas blancas faciales en un paciente pediátrico
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4477
I. Martínez Pallása,
Autor para correspondencia
imartinezp@salud.aragon.es

Corresponding author.
, A. Martínez Garcíab, S. de la Fuente Meiraa
a Servicio de Dermatología, Hospital Clínico Lozano Blesa. Zaragoza, Spain
b Servicio de Anatomía Patológica, Hospital Clínico Lozano Blesa, Zaragoza, Spain
Contenido relacionado
Actas Dermosifiliogr. 2022;113:959-6010.1016/j.ad.2021.02.027
I. Martínez Pallás, A. Martínez García, S. de la Fuente Meira
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Medical History

An 11-year-old boy of Algerian origin with no pathological history of interest was referred for the presence of asymptomatic facial lesions that had appeared 12 months earlier. He reported an increase in lesion number and size, but had not undergone any treatment. Neither his parents nor his siblings had a history of similar lesions.

Physical examination revealed small (3–4mm in diameter), whitish, markedly indurated papules without central umbilication located on the chin and periocular area (Fig. 1). Polarized light dermoscopy showed rounded, whitish, shiny homogeneous structures, with no visible signs of vascularization (Fig. 2). Histological examination (hematoxylin–eosin) of a shave biopsy of one of the lesions confirmed the presence of extensive basophilic deposition in the mid-dermis surrounded by collagen fibers and accompanied by epidermal hyperplasia and parakeratosis (Fig. 3). Blood tests, including a metabolic and hormonal profile, revealed no abnormalities.

Figure 1.

Indurated whitish papules on the chin.

(0.05MB).
Figure 2.

Polarized light dermoscopy reveals bright white structures.

(0.04MB).
Figure 3.

Hematoxylin–eosin, original magnification ×4.

(0.1MB).

What Is Your Diagnosis?

Diagnosis

Milia-like idiopathic calcinosis cutis

Diagnostic Process, Clinical Course, and Treatment

Histology revealed an amorphous crystalline basophilic deposit in the mid dermis, surrounded by collagen fibers. The surrounding tissue was unaffected. Calcium deposition was suspected and von Kossa staining was performed, revealing a blackish tone, which confirmed diagnosis of calcinosis cutis. Calcinosis cutis is defined as the deposition of calcium salts in the skin and subcutaneous tissue. Laboratory tests revealed normal serum levels of calcium, phosphorus, vitamin D, and parathyroid hormone. In the absence of any other underlying cutaneous or systemic disease, a definitive diagnosis of idiopathic calcinosis cutis was established. The patient underwent clinical observation and remains stable, with a similar number of lesions after 1 year of follow-up.

Comment

Calcinosis cutis is classified into 5 categories according to pathophysiology: dystrophic, metastatic, idiopathic, iatrogenic and mixed.1 To confirm the idiopathic form, it is necessary to rule out underlying tissue lesions, which distinguish it from dystrophic calcification, as well as alterations in calcium and phosphorus metabolism typical of metastatic calcification.

Milia-like idiopathic calcinosis cutis is a benign and infrequent subtype (with only 32 cases reported in the literature) that was defined in 1978 and is described mainly in pediatric patients of both sexes (mean age, 12 y) with Down syndrome (62.5% of cases).2 However, isolated cases have also been described in adults3 and in patients without Down syndrome.4

The cause is unknown, although various hypotheses have been proposed, including persistent localized trauma,5 calcium precipitation in the acrosyringium secondary to increased sweat calcium levels, and calcium deposition in persistently inflamed epidermal cysts.

The clinical presentation is characterized by multiple, small, whitish, round firm papules, which usually appear on the hands and feet but can also affect other areas, and can infrequently develop on the face. Clinically, this condition can be easily confused with other entities, including molluscum contagiosum, viral warts, epidermal inclusion cysts, and syringomas. Although the definitive diagnosis is histological, a petaloid appearance on dermoscopy may be helpful,6 but this is not pathognomonic.

The condition is characterized by clustered white formations that lack vasculature, in contrast to molluscum contagiosum and viral warts.

These lesions tend to disappear spontaneously in adulthood without leaving scars, and therefore the most appropriate approach appears to be follow-up and observation. Other treatment alternatives include curettage and topical retinoids. Recognition and differentiation from other clinically similar entities is essential to avoid traumatic treatments that can exacerbate the lesions and promote their recurrence.

Conflicts of Interest

The authors declare that they have no conflicts of interest.

References
[1]
D. Jiménez-Gallo, L. Ossorio-García, M. Linares-Barrios.
Calcinosis cutis and calciphylaxis.
Actas Dermosifiliogr, 106 (2015), pp. 785-794
[2]
B. Solak, R.O. Kara, E. Vargol.
Milia-like calcinosis cutis in a girl with Down syndrome.
An Bras Dermatol, 91 (2016), pp. 655-657
[3]
M. Houtappel, R. Leguit, V. Sigurdsson.
Milia-like idiopathic calcinosis cutis in an adult without Down's syndrome.
J Dermatol Case Rep, 1 (2007), pp. 16-19
[4]
E. Cho, H.J. Jun, S.H. Cho, J.D. Lee.
Subcorneal milia-like idiopathic calcinosis cutis: a rare presentation.
Ann Dermatol, 25 (2013), pp. 249-250
[5]
D.H. Kim, H. Kang, S.H. Cho, Y.M. Park.
Solitary milia-like idiopathic calcinosis cutis unassociated with Down's syndrome: two case reports.
Acta Derm Venereol, 80 (2000), pp. 151-152
[6]
G.N. Fox, D.A. Mehregan, M.N. Jablonowski.
Acral milia-like idiopathic calcinosis cutis in a child with down syndrome: report of a case, review of the literature, and description of dermoscopic findings.
Pediatr Dermatol, 30 (2013), pp. 263-264
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