Letters to the Editor
A case of facial porokeratosis Mibelli described the lesions of classic porokeratosis in 1893 as one or more localized, chronically progressive, hyperkeratotic, irregular plaques with central atrophy and a prominent peripheral keratotic ridge.1 Exclusively facial porokeratosis is an unusual clinical presentation. A 42-year-old woman had noticed, in her early 20s, the appearance of well-defined small, keratotic papular lesion that had slowly enlarged, located on the left side of the nose and hyperkeratotic papular eruption has grown in the last 4 years. She had also been under fatigue and dysphasic for the past 4 years. The patient reported itching and photosensitivity occasionally. The patient denied any family history of a similar condition. Skin examination showed a 7 × 4-cm size of plaque on the left side of the nose, extending to the cheek and eye. The lesion had irregular, annular with a well-demarcated raised hyperkeratotic border and hyperkeratotic papular eruption (fig. 1). Also, she had koilonychia. Laboratory examinations revealed decreased haemoglobin (8.6 g/dL), ferritin (0.83 ng/mL N: 10 – 90) and elevated total iron-binding capacity (422 µg/ dL N: 200 –300). Other laboratory results were negative or within normal limits. Upper gastrointestinal endoscopic examination showed upper esophageal stricture (Plummer– Vinson syndrome). Histopathological examination of the epidermis revealed hyperkeratosis, parakeratosis and acanthosis, and presence of cornoid lamella in the stratum corneum. In the centre of this keratin-filled invagination raised a parakeratotic column. The granular layer is absent beneath the cornoid lamella. In the upper malpighian stratum, there were some cells that possess an eosinophilic cytoplasma as a result of premature keratinization (fig. 2). Dense mononuclear infiltrate was observed in the dermis. A diagnosis of porokeratosis of Mibelli was made based on clinical appearance and histological examination of a biopsy specimen. Porokeratosis is a disorder of keratinization characterized by an extending keratotic lesion with an atrophic centre and a prominent peripheral ridge with a fissure at its summit. The cornoid lamella, a parakeratotic column, is its hallmark and an essential pathognomonic feature for diagnosis.2 It has been postulated that porokeratosis results from proliferation of an abnormal cellular clone to which several trigger factors have been suggested: irradiation, infective agents, trauma and immunosuppression.3 In our case, Plummer–Vinson syndrome may be one of the trigger factors. The aetiology is still unknown but it has been suggested that the presence of helper T cells and some Langerhans cells in Mibelli’s porokeratosis is evidence for immunological mechanisms induced by antigen presentation.4 The lesions are inherited as an autosomal dominant condition, but frequently sporadic cases are seen. Many
JEADV 2006, 20, 341–362 © 2006 European Academy of Dermatology and Venereology
fig. 1 The clinical appearance of the patient.
fig. 2 The histopathological appearance of the porokeratosis (haematoxylin and eosin stain, original magnification ×40).
treatment modalities have been tried including keratolytic agents, topical 5-fluorourasil,5 topical retinoids, topical imiquimod,6 cryoterapy,7 photodynamic therapy,8 carbon dioxide laser therapy, dermabrasion and excision of the affected area. None of these treatments are universally successful and at present there is no consensus on the optimum approach for this condition. 355
Letters to the Editor
Porokeratosis usually occurs on the trunk or the extremities, and facial lesions are rare; in a review of 165 000 consecutive biopsy specimens, some authors found only seven patients with facial porokeratosis.9 We also emphasize the clinical appearance of these lesions, which may easily be misdiagnosed if not examined correctly. As a consequence of misdiagnosis, the prevalence of these lesions is probably greater than at presently estimated. A Ferahbas,*† S Utas,† C Koc,† O Canoz‡ Departments of †Dermatology and ‡Pathology, Erciyes University, Medical Faculty, Kayseri, Turkey *Corresponding author, Department of Dermatology, Erciyes University, Medical Faculty, 38039 Kayseri, Turkey, tel. +0 90 352 437 49 01/21355; fax +0 90 352 437 76 15/15; E-mail:
[email protected];
[email protected]
fig. 1 Multiple verrucous lesions on the leg.
References 1 Wolff-Schreiner EC. Porokeratosis. In: Fitzpatrik TB, Freedberg IM, Eisen AZ, Wolf K, eds. Dermatology in General Medicine, 5th edn. McGraw-Hill, New York, 1999: 624–630. 2 Jang KA, Choi JH, Sung KJ, Moon KC, Koh JK. The hyperkeratotic variant of disseminated superficial actinic porokeratosis. Int J Dermatol 1999; 38: 204–206. 3 Machado S, Silva E, Pereria O, Sanches M, Massa A. Guess what? Porokeratosis of Mibelli. Eur J Dermatol 2000; 10: 485–486. 4 Jurecka W, Neumann RA, Knobler RM. Porokeratoses: immunohistochemical, light and electron microscopic evaluation. J Am Acad Dermatol 1991; 24: 96–101. 5 McDonald SG, Peterka ES. Porokeratosis (Mibelli): treatment with topical 5-fluorouracil. J Am Acad Dermatol 1983; 8: 107–110. 6 Agarwal S, Berth-Jones J. Porokeratosis Mibelli: successful treatment with 5% imiquimod cream. Br J Dermatol 2002; 146: 338–339. 7 Vergara G, Banuls J, Botella R, Silvestre JF. Porokeratosis of the lower lip. Eur J Dermatol 2002; 12: 500–502. 8 Nayeemuddin FA, Rhodes LE. Topical photodynamic therapy in disseminated superficial actinic porokeratosis. Clin Exp Dermatol 2002; 27: 703–706. 9 Navarro V, Pinazo I, Martinez E, Monteagudo C, Jorda E. Facial superficial porokeratosis. Dermatology 2000; 201: 361. DOI: 10.1111/j.1468-3083.2006.01437.x LETTERS Letters TO theEditor THE Editor EDITOR ?Letters 18 2006 totothe
Multiple verrucae vulgaris in a young woman’s tattoo A healthy 17-year-old white woman received a tattoo on her left leg, consisting of a sun in the colours of dark 356
fig. 2 Histological examination revealing hyperkeratosis, papillomatosis and acanthosis (haematoxylin and eosin stain; original magnification ×10).
blue and red. Three months later, numerous small, skincoloured, acuminate papules within the area of the tattoo were noted. No medical history of personal or familial warts could be found. The patient denied intravenous drug abuse. Physical examination revealed 13 firm, skin-coloured, verrucous 2- to 8-mm papules in the dark-blue-coloured areas of the tattoo (fig. 1). With the clinical diagnosis of verrucae vulgaris, a biopsy specimen was performed. Histological examination showed hyperkeratosis, acanthosis and papillomatosis (fig. 2). The findings of verrucous hyperplasia in this clinical setting confirmed the diagnosis of viral verruca. Laboratory tests for human immunodeficiency and hepatitis virus were negative. The remaining warts were curetted. No recurrence has been noted in a 2-month follow-up. Tattooing is an
JEADV 2006, 20, 341–362 © 2006 European Academy of Dermatology and Venereology
