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acral regions&#44; and areas around the joints on all 4 limbs&#44; especially on extensor surfaces &#40;<a class="elsevierStyleCrossRef" href="#fig0005">Fig&#46; 1</a>&#41;&#46; The development of the boy was normal for his age&#46;</p><elsevierMultimedia ident="fig0005"></elsevierMultimedia></span><span id="sec0015" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0015">Histopathology</span><p id="par0015" class="elsevierStylePara elsevierViewall">Histologic examination revealed hyperkeratosis with focal parakeratosis&#44; hypergranulosis&#44; and acanthosis with elongated rete ridges &#40;<a class="elsevierStyleCrossRef" href="#fig0010">Fig&#46; 2</a>&#41;&#46;</p><elsevierMultimedia ident="fig0010"></elsevierMultimedia><p id="par0020" class="elsevierStylePara elsevierViewall"><span class="elsevierStyleBold">What Is Your Diagnosis&#63;</span></p></span><span id="sec0020" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0020">Diagnosis</span><p id="par0025" class="elsevierStylePara elsevierViewall">Progressive symmetric erythrokeratoderma &#40;PSEK&#41;&#46;</p></span><span id="sec0025" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0025">Clinical Course and Treatment</span><p id="par0030" class="elsevierStylePara elsevierViewall">Topical treatment was started with 20&#37; urea cream and systemic treatment was started with oral acitretin at a dose of 0&#46;5<span class="elsevierStyleHsp" style=""></span>mg&#47;kg&#47;d&#46; Patient tolerance was good&#46; Over several months&#44; the lesions clearly involuted until they were limited to the acral regions of the limbs and the flexures&#44; with a minimal effective dose of 0&#46;3<span class="elsevierStyleHsp" style=""></span>mg&#47;kg&#47;d&#46; Periodic blood tests were carried out at follow-up visits&#44; and no abnormalities caused by the treatment were observed&#46; Adherence to treatment has been erratic over several years of follow-up at our dermatology clinic&#59; periods of evident exacerbation have coincided with the voluntary withdrawal of the medication&#46; The patient&#44; now 14 years old&#44; continues to receive treatment with acitretin at a minimum effective dose of 0&#46;3<span class="elsevierStyleHsp" style=""></span>mg&#47;kg&#47;d &#40;<a class="elsevierStyleCrossRef" href="#fig0015">Fig&#46; 3</a>&#41;&#46;</p><elsevierMultimedia ident="fig0015"></elsevierMultimedia></span><span id="sec0030" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0030">Comment</span><p id="par0035" class="elsevierStylePara elsevierViewall">Erythrokeratodermas are a clinically and genetically heterogeneous group of skin diseases characterized by well-defined erythematous-keratotic plaques&#46;<a class="elsevierStyleCrossRefs" href="#bib0035"><span class="elsevierStyleSup">1&#8211;3</span></a> There are various subtypes&#44; the most important of which are PSEK and erythrokeratoderma variabilis&#46;<a class="elsevierStyleCrossRefs" href="#bib0050"><span class="elsevierStyleSup">4&#8211;6</span></a> PSEK is a rare genodermatosis that has been associated with a mutation in the loricrin gene on chromosome 1q21&#44;<a class="elsevierStyleCrossRefs" href="#bib0035"><span class="elsevierStyleSup">1&#44;4&#44;5</span></a> although it is genetically heterogeneous&#46;<a class="elsevierStyleCrossRef" href="#bib0040"><span class="elsevierStyleSup">2</span></a> In most cases&#44; PSEK is hereditary&#44; with autosomal dominant inheritance&#44; incomplete penetrance&#44; and variable expressivity&#44;<a class="elsevierStyleCrossRefs" href="#bib0035"><span class="elsevierStyleSup">1&#44;2&#44;6</span></a> although sporadic cases<a class="elsevierStyleCrossRefs" href="#bib0050"><span class="elsevierStyleSup">4&#44;6</span></a> and cases with a recessive inheritance pattern<a class="elsevierStyleCrossRef" href="#bib0040"><span class="elsevierStyleSup">2</span></a> have also been reported&#46;</p><p id="par0040" class="elsevierStylePara elsevierViewall">Clinically&#44; PSEK is characterized by the appearance of large&#44; erythematous&#44; orange-colored&#44; well-defined&#44; symmetrical plaques that in some cases can have a keratotic or verrucous appearance&#44; usually located on the buttocks&#44; cheeks&#44; and limbs&#46;<a class="elsevierStyleCrossRefs" href="#bib0040"><span class="elsevierStyleSup">2&#44;6</span></a> Palmoplantar involvement has been reported in up to 50&#37; of cases&#46;<a class="elsevierStyleCrossRefs" href="#bib0050"><span class="elsevierStyleSup">4&#44;6</span></a> The lesions usually appear in the first years of life and spread slowly or remain stable during the following years&#59; spontaneous improvement sometimes occurs&#44; usually during puberty&#46;<a class="elsevierStyleCrossRefs" href="#bib0035"><span class="elsevierStyleSup">1&#44;6</span></a></p><p id="par0045" class="elsevierStylePara elsevierViewall">Histologic findings are nonspecific&#44; with foci of parakeratosis and marked acanthosis&#46;<a class="elsevierStyleCrossRefs" href="#bib0035"><span class="elsevierStyleSup">1&#44;4</span></a> Perinuclear vacuolization can be observed in the granular cell layer&#46;<a class="elsevierStyleCrossRef" href="#bib0050"><span class="elsevierStyleSup">4</span></a></p><p id="par0050" class="elsevierStylePara elsevierViewall">PSEK is treated symptomatically&#46; Topical treatments&#8212;including emollients and keratolytic agents&#8212;are reserved for the mildest cases&#46;<a class="elsevierStyleCrossRefs" href="#bib0035"><span class="elsevierStyleSup">1&#44;4</span></a> For severe cases&#44; systemic treatment&#8212;oral retinoids&#44; such as acitretin at a dose of 0&#46;5<span class="elsevierStyleHsp" style=""></span>mg&#47;kg&#47;d&#8212;is usually added&#46; Psoralen-UV-A phototherapy has also been used&#44; with variable clinical responses&#46;<a class="elsevierStyleCrossRefs" href="#bib0035"><span class="elsevierStyleSup">1&#44;4</span></a></p><p id="par0055" class="elsevierStylePara elsevierViewall">We have presented a new case of PSEK&#44; a rare entity&#46; The case is unusual because it is sporadic&#44; autosomal dominant inheritance being more common in this disease&#46;</p></span><span id="sec0035" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0035">Conflicts of Interest</span><p id="par0060" class="elsevierStylePara elsevierViewall">The authors declare that they have no conflicts of interest&#46;</p></span></span>"
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Vol. 106. Núm. 8.
Páginas 667-668 (octubre 2015)
Visitas
6071
Vol. 106. Núm. 8.
Páginas 667-668 (octubre 2015)
Case for Diagnosis
Acceso a texto completo
Can You Identify the Genodermatosis?
¿Qué genodermatosis es?
Visitas
6071
R. Santesteban Muruzábal
Autor para correspondencia
raquel.santesteban@hotmail.com

Corresponding author.
, M. Hervella Garcés, C. Ros Martín
Servicio de Dermatología, Hospital de Navarra, Complejo Hospitalario de Navarra, Pamplona, Navarre, Spain
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Patient History

A 5-year-old boy from Colombia with no known family history of skin disease presented with asymptomatic lesions on the limbs, predominantly in acral regions and areas around the joints. His family reported slow, peripheral growth of the lesions over the past few months but were unable to specify exactly when the clinical signs first appeared. No other associated symptoms were present at any level.

Physical Examination

Physical examination revealed keratotic, lichenoid plaques with mild peripheral erythema affecting the hips, acral regions, and areas around the joints on all 4 limbs, especially on extensor surfaces (Fig. 1). The development of the boy was normal for his age.

Figure 1
(0.1MB).
Histopathology

Histologic examination revealed hyperkeratosis with focal parakeratosis, hypergranulosis, and acanthosis with elongated rete ridges (Fig. 2).

Figure 2.

A, Hematoxylin-eosin, original magnification ×100. B, Hematoxylin-eosin, original magnification ×200.

(0.22MB).

What Is Your Diagnosis?

Diagnosis

Progressive symmetric erythrokeratoderma (PSEK).

Clinical Course and Treatment

Topical treatment was started with 20% urea cream and systemic treatment was started with oral acitretin at a dose of 0.5mg/kg/d. Patient tolerance was good. Over several months, the lesions clearly involuted until they were limited to the acral regions of the limbs and the flexures, with a minimal effective dose of 0.3mg/kg/d. Periodic blood tests were carried out at follow-up visits, and no abnormalities caused by the treatment were observed. Adherence to treatment has been erratic over several years of follow-up at our dermatology clinic; periods of evident exacerbation have coincided with the voluntary withdrawal of the medication. The patient, now 14 years old, continues to receive treatment with acitretin at a minimum effective dose of 0.3mg/kg/d (Fig. 3).

Figure 3
(0.08MB).
Comment

Erythrokeratodermas are a clinically and genetically heterogeneous group of skin diseases characterized by well-defined erythematous-keratotic plaques.1–3 There are various subtypes, the most important of which are PSEK and erythrokeratoderma variabilis.4–6 PSEK is a rare genodermatosis that has been associated with a mutation in the loricrin gene on chromosome 1q21,1,4,5 although it is genetically heterogeneous.2 In most cases, PSEK is hereditary, with autosomal dominant inheritance, incomplete penetrance, and variable expressivity,1,2,6 although sporadic cases4,6 and cases with a recessive inheritance pattern2 have also been reported.

Clinically, PSEK is characterized by the appearance of large, erythematous, orange-colored, well-defined, symmetrical plaques that in some cases can have a keratotic or verrucous appearance, usually located on the buttocks, cheeks, and limbs.2,6 Palmoplantar involvement has been reported in up to 50% of cases.4,6 The lesions usually appear in the first years of life and spread slowly or remain stable during the following years; spontaneous improvement sometimes occurs, usually during puberty.1,6

Histologic findings are nonspecific, with foci of parakeratosis and marked acanthosis.1,4 Perinuclear vacuolization can be observed in the granular cell layer.4

PSEK is treated symptomatically. Topical treatments—including emollients and keratolytic agents—are reserved for the mildest cases.1,4 For severe cases, systemic treatment—oral retinoids, such as acitretin at a dose of 0.5mg/kg/d—is usually added. Psoralen-UV-A phototherapy has also been used, with variable clinical responses.1,4

We have presented a new case of PSEK, a rare entity. The case is unusual because it is sporadic, autosomal dominant inheritance being more common in this disease.

Conflicts of Interest

The authors declare that they have no conflicts of interest.

References
[1]
I. Bilgin, K.E. Bozdag, S. Uysal, M. Ermete.
Progressive symmetrical erythrokeratoderma - response to topical calcipotriol.
J Dermatol Case Rep, 5 (2011), pp. 50-52
[2]
A. Akman, M. Masse, E. Mihci, G. Richard, A.M. Christano, B.J. Balle, et al.
Progressive symmetrical erythrokeratoderma: Report of a Turkish family and evaluation for loricrin and connexin gene mutations.
Clin Exp Dermatol, 33 (2008), pp. 582-584
[3]
B.R. Guo, L.D. Sun, Y. Cui, S. Yang, X.J. Zhang.
Progressive symmetrical erythrokeratoderma: Report of two Chinese families and evaluation for mutations in the loricrin, connexin 30.3 and connexin 31 genes.
Clin Exp Dermatol, 38 (2013), pp. 925-927
[4]
A. Álvarez-Vieitez, A. Torrelo, I.G. Medeiro, A. Zambrano.
Symmetric progressive erythrokeratoderma.
Actas Dermosifiliogr, 90 (1999), pp. 633-635
[5]
A. Ishida-Yamamoto, J.A. McGrath, H. Lam, H. Iizuka, R.A. Friedman, A.M. Christiano.
The molecular pathology of progressive symmetric erythrokeratoderma: A frameshift mutation in the loricrin gene and perturbations in the cornified cell envelope.
Am J Hum Genet, 61 (1997), pp. 581-589
[6]
J. Rodero, A. Torrelo, I.G. Medeiro, A. Zambrano.
Generalized progressive symmetric erythrokeratoderma.
Actas Dermosifiliogr, 92 (2001), pp. 207-209

Please cite this article as: Santesteban Muruzábal R, Hervella Garcés M, Ros Martín C. ¿Qué genodermatosis es?. Actas Dermosifiliogr. 2014;106:667–668.

Copyright © 2014. Elsevier España, S.L.U. and AEDV
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