|LETTER TO EDITOR
|Year : 2015 | Volume
| Issue : 2 | Page : 40-42
Facial porokeratosis of Mibelli mimicking discoid lupus erythematosus in young female
Mukunda Ranga Swaroop1, Shravandanahalli Nagendrakumar Manas1, Kuchangi Channabasavaprabhu Nischal2, Haleuoor Borelingegowda Basavaraj1, Belagola Dasevegowda Sathyanarayana1, Periasamy Vasudevan1
1 Department of Dermatology, Adichunchanagiri Institute of Medical Sciences, Bangalore, Karnataka, India
2 Consultant Dermatologist Nirmal Skin and Hair Clinic, Bangalore, Karnataka, India
|Date of Web Publication||6-Jan-2016|
Mukunda Ranga Swaroop
Department of Dermatology, Adichunchanagiri Institute of Medical Sciences, Bangalore, Karnataka
Source of Support: None, Conflict of Interest: None
|How to cite this article:|
Swaroop MR, Manas SN, Nischal KC, Basavaraj HB, Sathyanarayana BD, Vasudevan P. Facial porokeratosis of Mibelli mimicking discoid lupus erythematosus in young female. Indian J Dermatopathol Diagn Dermatol 2015;2:40-2
|How to cite this URL:|
Swaroop MR, Manas SN, Nischal KC, Basavaraj HB, Sathyanarayana BD, Vasudevan P. Facial porokeratosis of Mibelli mimicking discoid lupus erythematosus in young female. Indian J Dermatopathol Diagn Dermatol [serial online] 2015 [cited 2021 May 10];2:40-2. Available from: https://www.ijdpdd.com/text.asp?2015/2/2/40/173416
Porokeratosis represents a rare, benign, heterogeneous group of genodermatoses having abnormal keratinization, with an autosomal-dominant mode of inheritance. Porokeratosis of Mibelli (PM) was first described in 1893 by Vittorio Mibelli. Since then many clinical variants, both localized and generalized forms, have been described.  Clinically, it presents with annular plaques with atrophic center and hyperkeratotic edge, mainly over the extremities. The histopathology is characterized by keratin-filled epidermal invagination with a column of parakeratotic cells called the cornoid lamella. Exclusive facial porokeratosis is very rare, with very few cases being reported.  We describe a young woman with hyperkeratotic, hyperpigmented annular plaques distributed over the cheeks and the bridge of the nose, mimicking discoid lupus erythematosus (DLE).
A 24-year-old woman presented with complaints of dark, raised skin lesions over the nose and cheeks for the past 9 years. To begin with, she observed asymptomatic dark raised lesions which gradually increased in size with central white scarring over the period of next two years There was no history of photosensitivity, recurrent fever, Raynaud's phenomenon, or joint pains. The family history was not significant. On examination, hyperpigmented annular plaques with hyperkeratotic margins were found distributed on the cheeks in butterfly pattern and over the bridge of the nose. Annular plaques revealed central scarring and atrophy with mild scaling [Figure 1] and [Figure 2]. Differential diagnoses of DLE, porokeratosis, lupus vulgaris, and sarcoidosis were considered. Dermoscopy revealed an annular plaque with a peripheral ridge and a central crater-like depression, the ridge showing scaling in the center. There was depression along the ridge unmasked by removal of the scale. A biopsy from the annular plaque on the chin showed mild acanthosis with focal interface dermatitis, focal keratin-filled invagination, and cornoid lamella, composed of a vertical mound of parakeratosis with underlying dyskeratotic cells [Figure 3] and [Figure 4] The diagnosis of PM was confirmed. The patient was prescribed a topical sunscreen and tretinoin 0.1% for 6 months. There was xerosis and scaling with topical tretinoin application. The lesions healed with atrophy and depigmentation.
|Figure 1: Hyperkeratotic, hyperpigmented annular plaques distributed on cheeks and bridge of nose in butterfly pattern|
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|Figure 2: Annular plaques reveal central scarring and atrophy with mild scaling|
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|Figure 3: Biopsy reveals mild acanthosis with focal interface dermatitis and focal keratin-filled invagination (H and E 100×)|
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|Figure 4: Cornoid lamella composed of vertical mound of parakeratosis with underlying dyskeratotic cell (H and E 400×)|
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Porokeratosis is a chronic progressive disorder of keratinization. The mode of inheritance is autosomal-dominant, but some sporadic cases have also been reported. It has a slight male preponderance (3:2), usually beginning in childhood or adolescence, and is more prevalent in Caucasians. , The exact etiopathogenesis is unknown but multiple factors are proposed, such as chronic sun exposure, ultraviolet (UV) radiation, trauma, hepatitis B and C infection, human immunodeficiency virus infection (HIV) and immunosuppression. , Clinically, porokeratosis is classified into the localized forms, which include PM, linear porokeratosis (LP), and punctate porokeratosis, and the disseminated forms including disseminated superficial actinic porokeratosis (DSAP), superficial disseminated porokeratosis, and porokeratosis palmaris et plantaris disseminata (PPPD). In addition, other uncommon morphological variants such as facial, giant, punched out, hypertrophic, verrucous, and reticulate porokeratosis have also been reported. ,,
Porokeratosis usually occurs on the extremities or the trunk. Exclusive facial lesions are rare, although 15% of DSAP patients manifest with concomitant facial lesions.  Facial porokeratosis is considered a distinct subset of PM related to actinic damage, and is most commonly distributed over the nose and perinasal region. ,, Hence, it is known by other names, such as solar facial porokeratosis, localized actinic nasal porokeratosis, and localized actinic facial porokeratosis. , Nabai et al. in 1979 first reported two patients with facial porokeratosis.  Since then, about 50 cases of facial porokeratosis have been reported in the literature. Sharquie et al. reported the largest series of 15 cases of solar facial porokeratosis, of whom the majority of patients were young women (age range 19-37 years) and the nose was the commonest site involved.  Mehregran et al.  and Gutierrez et al.  reported 7 and 6 cases of facial porokeratosis, respectively. There have been other isolated reports of this variant documented in the literature. ,,,, Some other variants, such as a case of congenital facial LP and four cases of follicular facial porokeratosis, have also been documented. , In our patient, the lesions were distributed over the malar area, with atrophic center and hyperpigmented margin with mild scaling, thereby resembling DLE. Classically, DLE presents with annular lesions having central atrophy and peripheral hyperpigmentation with scaling, whereas porokeratosis manifests as central atrophy with hyperkeratotic edge.
Clinically, PM needs to be differentiated from the following: DLE, lupus vulgaris, sarcoidosis, Bowen's disease, annular lichen planus, lichen sclerosus et atrophicus, pityriasis rubra pilaris, acrokeratosis verruciformis, Darier's disease, seborrheic keratosis, linear scleroderma, actinic keratosis, xerosis, elastosis perforans serpiginosa, and inflammatory linear verrucous epidermal nevus. ,
Histopathologically, PM has the hallmark cornoid lamella, a column of a parakeratosis arising within the invagination of the epidermis. The granular layer is focally diminished and keratinocytes are dyskeratotic. A few cases of facial porokeratosis have microscopically shown follicular localization of cornoid lamellae, a histological feature seen in various form of porokeratosis including PM, DSAP, and follicular porokeratosis. 
PM has a chronic, indolent course with good overall prognosis, but with a risk of malignant transformation (Bowen's disease, squamous-cell carcinoma, basal cell carcinoma, and diffuse large B-cell lymphoma) of 7.5-11%, though this is not observed in facial porokeratosis. , The duration and size of the lesion, age of the patient, large lesions over the extremities, and the linear form and multiple patterns of porokeratosis are factors associated with increased risk of malignant transformation. , Porokeratosis in association with Gardner syndrome, lichen planus, diabetes mellitus, CAP syndrome (craniosyntosis, anal anomalies, and porokeratosis), Bloom's syndrome, and cystic fibrosis has also been reported. 
The various modalities of treatment available are as follows: Topical 5-fluorouracil, topical and systemic retinoids, topical and oral corticosteroids, keratolytics, topical vitamin D3 analogues, 5% imiquimod, 3% topical diclofenac sodium, lasers (CO 2 ablation, Nd:YAG, and 585 pulse dye laser), Grenz ray radiation, cryotherapy, dermabrasion, surgical excision, photodynamic therapy, and electrodesiccation. ,, Patients must also be counselled regarding photoprotection and long-term follow-up. Our patient responded well to sunscreen and topical tretinoin 0.1%.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.
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[Figure 1], [Figure 2], [Figure 3], [Figure 4]