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Terra Firma Forme Dermatosis T AD CASE REPORT The Early Onset Case of Terra Firma Forme Dermatosis Erken Başlangıçlı Terra Firma Forme Olgusu Pınar Özuğuz1 , Seval Doğruk Kaçar1 , Gulcan Saylam Kurtipek2 , Fatma Akyurek 2 1 Afyon Kocatepe University, Faculty of Medicine, Department of Dermatology, Afyon, Turkey 2 Konya Training and Research Hospital, Department of Dermatology, Konya, Turkey Abstract Terra firma-forme dermatosis (TFFD) is an idiopathic and uncommon disorder of keratinization in which patients present with acquired, dirt-like lesions that are resistant to washing. TFFD consists of 55% of the pediatric and 45% of the adult patients. It commonly involves neck, trunk and umbilicus. Although the affected patients present with typical lesions, the disorder is not well-known by pediatrician and dermatologists. The lesions can be easily removed with gentle swabbing with 70 % isopropyl alcohol pads. We describe a 3-monthold girl with TFFD, whose lesions started when she was one month old, therefore is the youngest patient thus far reported with this condition. Key Words: Terra firme forme dermatosis, early onset, dirty neck syndrome, acantosis nigricans Özet Terra firma-forme dermatoz (TFFD), yıkamaya dirençli olan kir-benzeri lezyonlar ile mevcut olan nadir görülen idyopatik bir keratinizasyon hastalığıdır. TFFD\'nin %55\'ini pediyatrik olgular, %45\'ini erişkin hastalar oluşturur. Genellikle boyun, gövde ve göbekte görülür. Hastalığın lezyonları tipik olsa da, hastalık Pediyatrist ve dermatologlar tarafından iyi bilinmemektedir. Lezyonlar% 70 izopropil alkollü pamukla hafif silme ile temizlenir. Biz burada, bir aylık iken başlayan 3-aylık bir kız hastayı, şimdiye kadar bildirilen en küçük olgu olmasından dolayı, sunuyoruz. Anahtar Kelimeler: Terra firme forme dermatozu, erken başlangıç, kirli boyun sendromu, akantozis nigrikans T erra firma-forme dermatoz (TFFD) is a benign dermatosis whose name derives from the Latin words terra firma, meaning “solid ground.” The condition was first described in 1987 by Duncan et al. (1). Synonym of this condition is “Duncan’s dirty dermatosis”. The etiology of TFFD is unknown. Lesions may appear verrucous, reticulate, and stuck-on, often affecting the neck, trunk, and umbilicus. Affected patients are often adolescents or young adults, with no gender predilection (2). The lesions can be easily removed with gentle swabbing with isopropyl alcohol pads. Although only of cosmetic significance, if unrecognized, may cause anxiety and lead to unnecessary investigations. Here we present a 3month-old girl with TFFD, which is the youngest reported patient. * A 3-month-old healthy girl presented with brown lesions on the knee and dorsum of foot and outer malleolus for two months (Figure 1). The child was otherwise well, with no history of atopic eczema but she had mild dryness of the skin. She was bathed every other day, and various soaps had been tried. General physical examination revealed no other significant skin lesions. The family reported only minimal improvement with aggressive attempts at peeling and scraping. On physical The case report was presented in 8 during 3-7 May 2013 in İzmir th Ege Dermatology Days Corresponding Author: Asist Prof Pınar Özuğuz Afyon Kocatepe University, Faculty of Medicine, Department of Dermatology 03100 Afyonkarahisar, Turkey Phone number: 02722463333 Fax number: 02722463300 e-mail address: [email protected] Tıp Araştırmaları Dergisi; 2015: 13(3):137-139 Case Report 137 Özuğuz et al Figure 1. The brown lesions on the knee and dorsum of foot and outer malleolus. examination, she had brown, hyperkeratotic plaques approximately 2x2 cm and 1x1 cm in size involving bilateral knee, dorsum of foot and outer malleolus (Figure 1). Firm rubbing of a lesion with a gauze pad saturated with 70% isopropyl alcohol completely removed the pigmentation (Figure 2), confirming the diagnosis of TFFD. We suggested treatment with a moisturizer without urea and a mixture of 1% salicylic acid and petroleum jelly. Discussion TFFD is a recently described cutaneous pigmentation disorder and is easily curable for a physician if aware of its existence. The condition is an uncommon disorder of keratinization in which patients present with dirt-like lesions that are resistant to washing. The cause of TFFD is unknown, but is not thought to be caused by a lack of personal hygiene. Fungal and bacterial studies in previous cases have been unhelpful (3). TFFD is a disorder of retantion keratinization with an unknown etiology (4). Although urea containing emollients are thought to play role in the development of this disorder, our patient had no history of usage of a topical agent containing urea and she started lesions when she was only a month old. The disease appears to be more frequent in children and young adults; in other instances, it may start during childhood or early adolescence but present at a later age (5). Clinically, TFFD is characterized by brown-grey and pigmented patches or plaques and macular discoloration located most commonly on the neck and trunk, but also reported on scalp, lips, axilla, chest, umblical area, chest, pubis and arms (4,6-8). The distribution may be localized, generalized or symmetrical (7). Acanthosis nigricans, confluent and reticulate papillomatosis, tinea versicolor, ichthyosis, and granular parakeratosis and inadequate cleansing as the cause of the lesions, Tıp Araştırmaları Dergisi; 2015: 13(3):137-139 Figure 2. The lesion after cleaning with alcohol. such as those seen in dermatosis neglecta and ‘dirty neck syndrome’’ of atopic dermatitis should be excluded (8). In dermatosis neglecta, simple washing with soap and water can remove the pigmentation, whereas in TFFD swabbing with isopropyl alcohol is the only effective method to wipe it off. Therefore, when this condition is suspected, firm, persistent pressure should be applied while rubbing the skin with isopropyl alcohol (4). Because TFFD is readily identified and treated, biopsies have rarely been performed, limiting the availability of histopathological analyses that might shed light on why this condition occurs (9). The reported skin biopsies have revealed a lamellar hyperkeratosis with absence of parakeratosis, but with focal orthokeratosis in whorls, and increased melanin in the hyperkeratotic areas and basal layer. It has been suggested that the condition arises from partial maturation and incomplete development of keratin squames, with retention of melanin (1,10). This histopathological picture may be indistinguishable from other benign papillomatous conditions, such as confluent and reticulated papillomatosis, acanthosis nigricans and epidermal nevi. So biopsy was unnecessary in this condition and we didn’t perform a biopsy from our patient. It is important for pediatricians and dermatologists to recognize TFFD. Recognizing and treating this condition can be gratifying for physicians, who can provide almost instant relief (9). Although TFFD is not harmful, it is critical for both clinicians and dermatopathologists to be aware of this condition in order to avoid misdiagnosis and expensive, painful, unnecessary evaluation, such as blood testing to investigate possible insulin resistance in patients with acanthosis nigricans and biopsy for other differential diagnosis. Furthermore, future studies are required to understand the etiology of TFFD. 138 Terra Firma Forme Dermatosis CASE REPORT References 1. 2. 3. 4. 5. Duncan WC, Tschen JA, Knox JM. Terra firmaforme dermatosis. Arch Dermatol. 1987; 123:567–569. Akkash L, Badran D, Al-Omari AQ. Terra firma forme dermatosis. Case series and review of the literature. J Dtsch Dermatol. 2009;7: 102–107. Pinder VA, Eriyagama S, Saracino A, Moosa Y. Terra firma-forme dermatosis:another cause of reticulate pigmentation. Clin Exp Dermatol. 2012; 37(4):446-447. Browning J, Rosen T. Terra firmaforme dermatosis revisited. Dermatol Online J. 2005; 11: 15. Oztürk F, Kocabaş E, Ertan P, Ermertcan AT. Terra firma-forme dermatosis. Cutan Ocul Toxicol. 2010 Dec; 29(4):303-305. Tıp Araştırmaları Dergisi; 2015: 13(3):137-139 6. 7. 8. 9. 10. Mallari R, Sinclair RD. Terra firma-forme dermatosis of the scalp. Br J Dermatol. 2002; 147:1019. Guarneri C, Cannavò SP. Is this skin diseased, or is it just dirty? JAAPA 2009; 22:17. Berk DR, Bruckner AL. Terra firma‑forme dermatosis in a 4-month-old girl. Pediatr Dermatol 2011; 28:79-81. Berk DR. Terra firma-forme dermatosis: a retrospective review of 31 patients. Pediatr Dermatol. 2012; 29(3): 297-300. Berk DR, Mutizwa MM. Comment regarding the histopathology of terra firma-forme dermatosis. J Cutan Pathol 2012; 39:300-301. 139