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CORRESPONDENCE
Year : 2011  |  Volume : 56  |  Issue : 4  |  Page : 460-461
Linear porokeratosis with follicular involvement


Department of Dermatology and Department of Pathology, Surat Municipal Institute of Medical Education and Research, Near Sahara Darwaja, Umarwada, Surat, Gujarat, India

Date of Web Publication10-Sep-2011

Correspondence Address:
Jignesh B Vaishnani
Department of Dermatology and Department of Pathology, Surat Municipal Institute of Medical Education and Research, Near Sahara Darwaja, Umarwada, Surat, Gujarat
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0019-5154.84715

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How to cite this article:
Vaishnani JB, Bosamiya SS, Sapariya BJ, Udhreja PR. Linear porokeratosis with follicular involvement. Indian J Dermatol 2011;56:460-1

How to cite this URL:
Vaishnani JB, Bosamiya SS, Sapariya BJ, Udhreja PR. Linear porokeratosis with follicular involvement. Indian J Dermatol [serial online] 2011 [cited 2019 May 25];56:460-1. Available from: http://www.e-ijd.org/text.asp?2011/56/4/460/84715


Sir,

Porokeratosis, [1],[2] first described in 1893 by Mibelli, is a heterogeneous group of disorders of abnormal keratinization and affects the keratinous tissue, with variety of clinical presentation and potential for malignant transformation. [3] Exact heritable pattern has not been identified. It appears in infancy or childhood and boys are more frequently affected. Besides different clinical subtypes, e.g., porokeratosis of Mibelli (PM), disseminated superficial, disseminated superficial actinic porokeratosis (DSAP), linear porokeratosis, porokeratosis palmaris et plantaris disseminata (PPPD), palmo-planter punctate porokeratosis (PPPP), other varieties like pustular, pruritic papular, verrucus/porokeratosis ptycotropica, giant, follicular, bullous, hypopigmented and erosive, have been described. Exact pathogenesis is not known but an abnormal early keratinocyte apoptosis accompanied by dysregulation of terminal differentiation (keratinization) has been suggested for pathogenesis of cornoid lamella. [4] Development of the invasive squamous cell carcinoma, basal cell carcinoma and Bowen disease has been reported in all variants except punctate porokeratosis. Linear porokeratosis is particularly more susceptible to malignant degeneration. [1],[2],[3]

A 27-year-old female presented with asymptomatic small, elevated skin lesions on right upper limb. Lesions gradually increased in number and size over the period of 1 year. Cutaneous examination revealed multiple well-defined, brown-black to skin color papules and annular lesions with peripheral keratotic ridges [Figure 1], distributed linearly on flexure side of right upper arm, forearm, shoulder and axillae. Palms, soles and other body areas were free from lesions. There was no family history of similar lesion. Histopathology of skin biopsy showed cornoid lamella which is a column of tightly packed parakeratotic cells with pyknotic nuclei in epidermis and absence of granular layer below cornoid lamella. Here, cornoid lamella was localized to hair follicle [Figure 2] and [Figure 3]. Although cornoid lamella is a classical feature of porokeratosis, it may be seen in viral warts, icthyosis and other nevoid hyperkeratotic conditions.

Clinically, various stains like gentian violet, povidone-iodine and Giemsa have been used to stain cornoid lamella. Paint the suspected lesion/s with stain, then remove the stain with alcohol; elevated keratotic ridge retains the stain and is helpful in clinical differentiation from seborrheic keratoses, stucco keratoses, disseminated granuloma annulare, atrophic annular lichen planus. [5]
Figure 1: Papules and annular lesions (border stain with Giemsa stain) on forearm

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Figure 2: Tightly packed parakeratotic cells with pyknotic nuclei extending into hair follicle and absence of granular layer (H and E stain, ×400)

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Figure 3: Parakeratotic cells involving hair follicle (H and E stain, ×400)

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Irrespective of clinical presentation, histopathological features of all subtypes are identical and include cornoid lamella along the peripheral rim. Cornoid lamella is most prominent in PM. Besides keratotic ridge, cornoid lamella may involve hair follicle as well as acrosyringium. Follicular localization of cornoid lamella is uncommon and described only in association with other subtypes such as DSAP, PM, and exclusive follicular cornoid lamella in follicular porokeratosis. [6],[7],[8] Follicular and non-follicular epidermal involvement may be due to multifocal origin of the abnormal keratinization within involved epidermis. It is not known whether lesion starts primarily form hair follicle or there is secondary involvement of follicular epidermis. Clinically involvement of hair follicle has been described in DSAP and PM. [7]

Linear porokeratosis [1],[2] is a rare genetically determined disorder with a characteristic linear distribution and can appear at any age. Lesions may arise in infancy or childhood but the onset may be delayed and has a tendency for malignant transformation. Malignant transformation [3] is as high as 19% in linear porokeratosis, while in other types it ranges from 3 to 10%. Linear porokeratosis exists in two forms: In the more common localized form, lesions are unilateral, confined to one extremity and frequently distal in localization. In the rare generalized form, [9] lesions are multiple, affect several extremities and involve the trunk. In the present case of localized form of linear porokeratosis, cornoid lamella is localized to follicle as well as non-follicular epidermis. To the best of our knowledge, follicular localization of cornoid lamella in linear porokeratosis is rare and has been reported recently in a few case studies. [10]

Our patient was treated with topical 5-fluorouracil and advised protection from sunlight. Many drugs including topical 5-fluorouracil, calcipotriol, 5% imiquimod and oral retinoid (Acitretin) have been found effective in the treatment of porokeratosis. Surgical treatments like cryotherapy, dermabrasion, CO 2 laser ablation, shave and curettage, photodynamic therapy, electrodessication, 585 nm flashlamp-pumped pulsed dye laser and frequency-doubled Nd:YAG laser are shown to be helpful in linear porokeratosis. Although topical Diclofenac (3% gel) is found to exert strong anti-neoplastic effects by inducing apoptosis of malignant cells, it is not found effective in the treatment of porokeratosis, but may have a role in prevention of malignancy. [11] Protection from sun light/UV radiation and periodic examination of patient for early detection of the malignant changes is mandatory.

 
   References Top

1.Pierson D, Bandel C, Ehrig T, Cockerell CJ. Benign epidermal tumors and proliferation. In: Bolognia JL, Jurizzo JL, Rapini RP, Thomas DH, Jose MM, Hilaire JS, et al. editors. Dermatology. 1 st ed. London: Mosby (Elsevier science); 2003. p. 1697-720.  Back to cited text no. 1
    
2.Judge MR, McLea WH, Munro CS. Disorder of keratinization. In: Burn T, Breathnach S, Cox N, Griffiths C, editors. Rook's Textbook of dermatology. 7 th ed. Oxford: Blackwell Science; 2004. p. 34.75-34.77  Back to cited text no. 2
    
3.Maubec E, Duvillard P, Margulis A, Bachollet B, Degois G, Avril MF. Common skin cancers in porokeratosis. Br J Dermatol 2005;152:1389-91.  Back to cited text no. 3
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4.Shen CS, Tabata K, Matsuki M, Goto T, Yokochi T, Yamanishi K. Premature apoptosis of keratinocytes and the dysregulation of keratinization in porokeratosis. Br J Dermatol 2002;147:498-502.  Back to cited text no. 4
[PUBMED]  [FULLTEXT]  
5.Thomas CJ, Elston DM. Medical Pearl: Gentian violet to highlight the cornoid lamella in disseminated superficial actinic porokeratosis. J Am Acad Dermatol 2005;52:513-4.  Back to cited text no. 5
[PUBMED]  [FULLTEXT]  
6.Yong AS, Singh M, Goulding JM, Swale VJ. Follicular porokeratosis of Mibelli on the buttocks. Clin Exp Dermatol 2009;34:43-5.  Back to cited text no. 6
[PUBMED]  [FULLTEXT]  
7.de Almeida HL Jr, Guarenti IM, de Castro LA, Rocha NM. Follicular involvement in porokeratosis. J Eur Acad Dermatol Venereol 2007;21:109-11.  Back to cited text no. 7
[PUBMED]  [FULLTEXT]  
8.Pongpudpunth M, Farber J, Mahalingam M. Follicular porokeratosis: Distinct clinical entity or histologic variant? J Cutan Pathol 2009;36:1195-9.  Back to cited text no. 8
[PUBMED]  [FULLTEXT]  
9.Dervis E, Demirkesen C. Generalized linear porokeratosis. Int J Dermatol 2006;45:1077-9.  Back to cited text no. 9
[PUBMED]  [FULLTEXT]  
10.Minami-Hori M, Ishida-Yamamoto A, Iizuka H. Cornoid lamella associated with follicular infundibulum and acrosyringium in porokeratosis. J Dermatol 2009;36:125-30.  Back to cited text no. 10
[PUBMED]  [FULLTEXT]  
11.Marks S, Varma R, Cantrell W, Chen SC, Gold M, Muellenhoff M, et al. Diclofenac sodium 3% gel as a potential treatment for disseminated superficial actinic porokeratosis. J Eur Acad Dermatol Venereol 2009;23:42-5.  Back to cited text no. 11
[PUBMED]  [FULLTEXT]  


    Figures

  [Figure 1], [Figure 2], [Figure 3]

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