|Year : 2022 | Volume
| Issue : 1 | Page : 53-54
Congenital lichenoid porokeratosis in a child
Subhra Dhar1, Shikhar Ganjoo2, Samujjala Deb3
1 Chief of Lab and Consultant Histopathologist, WIZDERMPATHLAB, Kolkata, West Bengal, India
2 Department of Dermatology and STD, SGT Medical College, Hospital and Research Institute, Gurugram, Haryana, India
3 Department of Dermatology, Mission Hospital, Durgapur, West Bengal, India
|Date of Submission||12-Jun-2021|
|Date of Acceptance||14-Sep-2021|
|Date of Web Publication||31-Dec-2021|
Associate Professor, Department of Dermatology and STD, SGT Medical College, Hospital and Research Institute, Gurugram, Haryana
Source of Support: None, Conflict of Interest: None
Linear porokeratosis is a rare disease that can be congenital or may occur typically during infancy and childhood. It presents clinically as streaks or plaques of reddish-brown papules along the Blaschko lines. The presence of cornoid lamella is pathognomonic on histopathology. We present the case of a 3-year-old female child with classical lesions over the left upper and lower limbs.
Keywords: Cornoid lamella, lichenoid reaction pattern, linear porokeratosis
|How to cite this article:|
Dhar S, Ganjoo S, Deb S. Congenital lichenoid porokeratosis in a child. Indian J Paediatr Dermatol 2022;23:53-4
| Introduction|| |
Porokeratosis is a clonal expansion of keratinocytes which differentiate abnormally but are not truly neoplastic. All forms of porokeratosis show a thin column of parakeratosis, known as the cornoid lamella which represents the active border. Congenital lichenoid porokeratosis is a histopathological variant of linear porokeratosis which presents with the features of lichenoid tissue reaction including the presence of lymphocytic infiltrate, vacuolization of spinosum layer, and pigmentary incontinence. It presents as streaks or plaques of reddish-brown papules along limbs or side of the trunk and head and neck following Blaschko lines, indicating somatic mosaicism. There is a risk of malignant change, particularly squamous cell carcinoma associated with linear porokeratosis.
| Case Report|| |
A 3-year-old otherwise healthy girl born to nonconsanguineous parents presented with multiple annular plaques along the palms and left upper and lower limb present since birth [Figure 1]a, [Figure 1]b, [Figure 1]c. To start with, there were few lesions on the left palm, which gradually increased in number and dimensions to acquire the present status. The rest of the cutaneous examination was within normal limits.
|Figure 1: (a-c) Multiple annular plaques along the palms and left upper and lower limb present since birth|
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A punch biopsy was performed from the margin of a lesion on the left forearm. Notable histological findings were hyperkeratotic and papillomatous epidermis. The invagination between the papillomatous projections of epidermis showed a parakeratotic column with the parakeratotic cells having pyknotic nuclei [Figure 2]a and [Figure 2]b. The underlying epidermis showed irregular keratinocytes with pyknotic nuclei and perinuclear edema. The upper layer of the stratum spinosum demonstrated vacuolization. Granular cells were absent beneath the parakeratotic column but present elsewhere, showing coarse and abundant keratohyalin granules. The dermis had mild lymphohistiocytic infiltrate. The histopathological findings confirmed the diagnosis of congenital lichenoid porokeratosis. The child was started on oral acitretin at a dose of 0.5 mg/kg body weight and showed a good clinical response at follow-up.
|Figure 2: (a and b) Hematoxylin and eosin-stained section of the skin showing hyperkeratotic and papillomatous epidermis. The invagination between the papillomatous projections of epidermis showed a parakeratotic column with the parakeratotic cells having pyknotic nuclei. The underlying epidermis showed irregular keratinocytes with pyknotic nuclei and perinuclear edema. The upper layer of stratum spinosum demonstrated vacuolization. Granular cells were absent beneath the parakeratotic column|
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| Discussion|| |
Linear porokeratosis is an uncommon variant of porokeratosis, which may be congenital or develop during adulthood. It has been listed as a rare disease by the Office of Rare Diseases of the National Institute of Health. Congenital lichenoid porokeratosis is an extremely rare entity, with very few reports across the globe.
Linear porokeratosis can be further classified as localized linear porokeratosis and systematized linear porokeratosis based on the extent of involvement. Clinically, it presents as streaks or plaques of reddish-brown plaques along the Blaschko lines with a surrounding raised rim which represents the cornoid lamella on histopathology. Congenital lichenoid porokeratosis is a rare histological variant of linear porokeratosis showing a lichenoid tissue reaction pattern. Histopathological examination shows mild lymphocytic infiltrate, vacuolization of spinosum layer, and pigmentary incontinence in addition to pathognomonic feature of cornoid lamella.
The disease may be the outcome of genetic mosaicism. The most significant complication is malignant degeneration (basal cell carcinoma or squamous cell carcinoma), which necessitates periodic follow-up and vigilant monitoring. Other complications include ulcerations, bony abnormalities, and onychodystrophy.,,
Differentials that need to be kept in mind include linear lichen planus, verrucous epidermal nevus, verrucous stage of incontinentia pigmenti, and linear Darier's disease among a few. Treatment modalities apart from oral acitretin include topical calcipotriol, topical tretinoin, and physical treatment modalities such as cryotherapy and ablative laser therapy.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient's parents have given their consent for his images and other clinical information to be reported in the journal. The patient's parents understand that name and initials will not be published and due efforts will be made to conceal identity.
Financial support and sponsorship
Conflicts of interest
There are no conflicts of interest.
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[Figure 1], [Figure 2]