|Year : 2016 | Volume
| Issue : 3 | Page : 218-220
Linear porokeratosis over the face: An unusual presentation
Dibyendu Basu1, Indrashis Podder1, Anupam Das2
1 Department of Dermatology, Medical College and Hospital, Kolkata, West Bengal, India
2 Department of Dermatology, KPC Medical College and Hospital, Kolkata, West Bengal, India
|Date of Web Publication||5-Jul-2016|
“Prerana” 19, Phoolbagan, Kolkata - 700 086, West Bengal
Source of Support: None, Conflict of Interest: None
Porokeratosis is a heterogeneous disorder of keratinization usually inherited in an autosomal dominant pattern. It usually affects the trunk and extremities. Lesions exclusively present over face are rare and not well-documented. We present a case of linear porokeratosis along the blaschkoid lines over the face in a 9-year-old girl.
Keywords: Child, face, linear porokeratosis
|How to cite this article:|
Basu D, Podder I, Das A. Linear porokeratosis over the face: An unusual presentation. Indian J Paediatr Dermatol 2016;17:218-20
|How to cite this URL:|
Basu D, Podder I, Das A. Linear porokeratosis over the face: An unusual presentation. Indian J Paediatr Dermatol [serial online] 2016 [cited 2020 Jul 5];17:218-20. Available from: http://www.ijpd.in/text.asp?2016/17/3/218/179492
| Introduction|| |
In 1893, Mibelli coined the term porokeratosis assuming that the columns of parakeratosis evolve from the ostia of eccrine ducts. Though the disorder is mostly inherited in autosomal dominant fashion but most of the cases are sporadic. Subtypes include classic porokeratosis of Mibelli, disseminated superficial porokeratosis, disseminated superficial actinic porokeratosis, punctate porokeratosis, porokeratosis palmaris et plantaris disseminate, and linear porokeratosis. Apart from different clinical subtypes, other varieties include pustular, pruritic papular, verrucous/porokeratosis ptychotropica, giant, follicular, bullous, hypopigmented, and erosive. Further classification of linear porokeratosis includes localized, zosteriform, systematized, or generalized. Though the lesions of porokeratosis may involve any part of the body, lesions on the genitalia  and face  are regarded as uncommon. We present a case of a girl child having linear porokeratosis distributed in a blaschkoid pattern over the face without any lesion over other parts of the body. Only a few cases of linear porokeratosis have been reported from India.
| Case Report|| |
A 9-year-old girl presented with a few elevated lesions over the left side of her cheek, present for the preceding 5 years. Initially, a solitary circular lesion had developed over the left cheek followed by the appearance of three more lesions in a linear arrangement within 5–6 months. These asymptomatic lesions were increasing in size, proportionately with her age. There was no history of itching, pain, irritation, or worsening of the lesions on sun exposure. No involvement of the trunk, extremities or mucous membranes was observed. On examination, three annular plaques (appro × 5–7 mm in diameter) were noted in a linear distribution along the mandibular margin of the left side of face with one lesion over the left cheek [Figure 1]. These lesions were characterized by a well-demarcated raised margin with atrophic center. Our differential diagnoses were porokeratosis, linear verrucous epidermal nevus, linear lichen planus, and verrucous variety of incontinentia pigmenti. Biopsy was taken from the peripheral raised hyperkeratotic ridge which showed a keratin-filled invagination of the epidermis. In the center of this keratin-filled invagination, there was a parakeratotic column, the cornoid lamella [Figure 2]. Biopsy confirmed the diagnosis of porokeratosis. She has been prescribed topical retinoids in the form of retinoic acid 0.025% cream and advised for regular follow-up.
|Figure 2: Photomicrograph showing a column of parakeratotic cells overlying a zone of hypogranulosis (H and E, ×100)|
Click here to view
| Discussion|| |
Porokeratosis, a disorder of keratinization, has a wide variety of clinical manifestations with a characteristic histological finding of cornoid lamella. Although patients with disseminated, superficial, actinic porokeratosis have facial lesions in 15% cases, exclusive facial involvement is extremely rare. In 1979 Nabai et al. reported two patients with facial porokeratosis. There are only a few reports of porokeratosis present exclusively over the face.
Linear porokeratosis is listed as a rare disease by Office of Rare Diseases of the National Institute of Health., There are two forms of linear porokeratosis. In the localized form, which is more common, lesions are unilateral and confined to one extremity, affecting the distal portion more than the proximal areas. In the rare generalized form, lesions are multiple, affect several extremities and involve the trunk. Porokeratosis also involves chest and proximal portions of upper extremities. Uncommon variants of porokeratosis include facial porokeratosis, giant porokeratosis, punched out porokeratosis, hypertrophic verrucous porokeratosis, and reticulate porokeratosis. In 2005 Zhang et al. reported the first case of congenital linear porokeratosis confined to the face in English literature.
Porokeratosis is a premalignant condition. There is an increased risk of malignant transformation to squamous cell carcinoma, especially from disseminated superficial actinic porokeratosis, giant porokeratosis, and linear porokeratosis. Linear porokeratosis has the highest risk of developing a cutaneous malignancy.
This case is reported due to the rarity of reports of linear porokeratosis confined to face. As because there is increased risk of malignant transformation, especially squamous cell carcinoma, long-term follow-up is needed.
Financial Support and Sponsorship
Conflicts of Interest
There are no conflicts of interest.
| References|| |
Rifaioglu EN, Ozyalvaçli G. Follicular porokeratosis at alae nasi; a case report and short review of literature. Indian J Dermatol 2014;59:398-400.
Miranda SM, de Miranda JN, de Souza Filho JB. Facial porokeratosis characterized by destructive lesions. Int J Dermatol 2004;43:913-4.
Vaishnani JB, Bosamiya SS, Sapariya BJ, Udhreja PR. Linear porokeratosis with follicular involvement. Indian J Dermatol 2011;56:460-1.
Kudligi C, Bhagwat PV, Giriyan SS, Eshwarrao MS. Unilateral systematized linear porokeratosis: A report of a rare case. Indian J Dermatol 2011;56:452-3.
Neri I, Marzaduri S, Passarini B, Patrizi A. Genital porokeratosis of Mibelli. Genitourin Med 1995;71:410-1.
Gutierrez EL, Galarza C, Ramos W, Tello M, De Paz PC, Bobbio L, et al.
Facial porokeratosis: A series of six patients. Australas J Dermatol 2010;51:191-4.
Malhotra SK, Puri KJ, Goyal T, Chahal KS. Linear porokeratosis. Dermatol Online J 2007;13:15.
Lee Y, Choi EH. Exclusive facial porokeratosis: Histopathologically showing follicular cornoid lamellae. J Dermatol 2011;38:1072-5.
Nabai H, Mehregan AH. Porokeratosis of Mibelli. A report of two unusual cases. Dermatologica 1979;159:325-31.
Moss C, Shahidullah H. Naevi and other developmental defects. In: Burn T, Breathnach S, Cox N, Griffiths C, editors. Rook's Textbook of Dermatology. 8th
ed. Oxford: Blackwell Science; 2010. p. 18.25.
Agrawal SN, Pawar PC, Dhillan PV. Linear porokeratosis: An unusual presentation. Indian J Dermatol 2014;59:318.
Laxmisha C, Thappa DM, Karthikeyan K, Jayanthi S. Linear porokeratosis along Blaschko's lines. Indian J Dermatol 2003;48:167-9.
Das D, Ghosh A, Das A. Unilateral linear porokeratosis. Indian Pediatr 2013;50:981.
Zhang ZH, Xiang LH, Chen LJ, Luo Y, Zheng ZZ. Congenital facial linear porokeratosis. Clin Exp Dermatol 2005;30:361-2.
Sasson M, Krain AD. Porokeratosis and cutaneous malignancy. A review. Dermatol Surg 1996;22:339-42.
Rémond B, Dompmartin A, Mandard JC, Leroy D. Squamous cell carcinoma and disseminated superficial actinic porokeratosis. Ann Dermatol Venereol 1994;121:50-2.
Ma DL, Vano-Galvan S. Squamous cell carcinoma arising from giant porokeratosis. Dermatol Surg 2009;35:1999-2000.
Scola N, Skrygan M, Wieland U, Kreuter A, Gambichler T. Altered gene expression in squamous cell carcinoma arising from congenital unilateral linear porokeratosis. Clin Exp Dermatol 2012;37:781-5.
[Figure 1], [Figure 2]