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American Journal of Dermatopathology:
doi: 10.1097/DAD.0b013e3181c98249
Letter to the Editor

Porokeratotic Eccrine and Hair Follicle Nevus

Martorell-Calatayud, Antonio MD*; Colmenero, Isabel MD†; Hernández-Martín, Angela MD‡; Requena, Luis MD, PhD§; Torrelo, Antonio MD, PhD¶

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*Department of Dermatology, Hospital del Niño Jesús, Madrid, Spain; †Department of Pathology, Hospital del Niño Jesús, Madrid, Spain; ‡Department of Dermatology, Hospital del Niño Jesús, Madrid, Spain; §Department of Dermatology, Hospital Fundación Jimenez Díaz, Madrid, Spain; and ¶Department of Dermatology, Hospital del Niño Jesús, Madrid, Spain.

The authors have no conflict of interest to disclose.

To the editor:

Porokeratotic eccrine and hair follicle nevus (PEHFN) is an extremely rare disorder of keratinization with eccrine and hair follicle involvement.1-3

A 7-year-old boy without any relevant medical history and with no family history of consanguinity had skin lesions located on his right forearm, right wrist, and upper right trunk. The lesions had their onset at birth as linear white plaques, and over the following 2 years, the lesions became more extensive leading to multiple hyperkeratotic plaques distributed in a linear pattern following the lines of Blaschko (Fig. 1). The patient was otherwise well, and the clinical examination was normal. A skin biopsy revealed a psoriasiform epidermal hyperplasia with cornoid lamellae overlying epidermal invaginations, with dyskeratotic keratinocytes and absent granular layer. These multiple parakeratotic foci occurred characteristically above both acrothrichia and acrosyringia (Fig. 2). There were also sparse dermal lymphocytic perivascular infiltrates with a few eosinophils.

Figure 1
Figure 1
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Figure 2
Figure 2
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PEHFN is a rare nevus of eccrine and hair follicle components, with only 5 cases reported previously (data summarized in the Table 1).1-3 This entity, along with the porokeratotic eccrine ostial and dermal duct nevus (PEODDN), is recently considered a subvariant of the unifying term “porokeratotic adnexal ostial nevus.”3

Table 1
Table 1
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It is a sporadic condition, and lesions are typically present mainly in full-term newborns as multiple, asymptomatic, Blaschko-linear, hyperkeratotic papules and plaques involving bilaterally the distal part of the extremities, although proximal extremities, trunk, face, and scalp can also be affected. There is only 1 isolated report of unilateral lesions located on the forearm, wrist, and palm.1 PEHFN is most likely a mosaic condition due to postzygotic somatic mutations during embryogenesis. This abnormal clone of epidermal cells may be prone to malignant degeneration, as has been documented in PEHFN.3

The differential diagnosis of PEHFN includes keratinocytic nevi, linear porokeratosis, and PEODDN. PEODDN and PEHFN are considered adnexal hamartomas with histological features of porokeratosis, and according to studies on multiple sections, both are probably variants of the same process.4-6

There is no effective treatment for PEHFN. Topical 10% urea and retinoids may be beneficial. Ultrapulsed carbon dioxide laser and simple surgical excision should be reserved for cases with limited body involvement.

Antonio Martorell-Calatayud, MD

Department of Dermatology, Hospital del Niño Jesús, Madrid, Spain

Isabel Colmenero, MD

Department of Pathology, Hospital del Niño Jesús, Madrid, Spain

Angela Hernández-Martín, MD

Department of Dermatology, Hospital del Niño Jesús, Madrid, Spain

Luis Requena, MD

Department of Dermatology, Hospital Fundación Jimenez Díaz, Madrid, Spain

Antonio Torrelo, MD

Department of Dermatology, Hospital del Niño Jesús, Madrid, Spain

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REFERENCES

1. Coskey RJ, Mehregan AH, Hashimoto K. Porokeratotic eccrine duct and hair follicle nevus. J Am Acad Dermatol. 1982;6:940-943.

2. Kroumpouzos G, Stefanato CM, Wilkel CS, et al. Systematized porokeratotic eccrine and hair follicle naevus: report of a case and review of the literature. Br J Dermatol. 1999;141:1092-1096.

3. Goddard DS, Rogers M, Frieden IJ, et al. Widespread porokeratotic adnexal ostial nevus: clinical features and proposal of a new name unifying porokeratotic eccrine ostial and dermal duct nevus and porokeratotic eccrine and hair follicle nevus. J Am Acad Dermatol. 2009;61:e1-e14.

4. Moreno A, Pujol RM, Salvatella N, et al. Porokeratotic eccrine ostial and dermal duct nevus. J Cutan Pathol. 1988;15:43-48.

5. Aloi FG, Pippione M. Porokeratotic eccrine ostial and dermal duct nevus. Arch Dermatol. 1986;122:892-895.

6. Bergman R, Lichtig C, Cohen A, et al. Porokeratotic eccrine ostial and dermal duct nevus. An abnormally keratinizing epidermal invagination or a dilated, porokeratotically plugged acrosyringium and dermal duct? Am J Dermatopathol. 1992;14:319-322.

© 2010 Lippincott Williams & Wilkins, Inc.

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