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146

A Case of Extensive Alba

Deborah Lee, M.D., Ju-Hyun Kang, M.D., Sang-Hyun Kim, M.D., Jong-Keun Seo, M.D., Ho-Suk Sung, M.D., Seon-Wook Hwang, M.D. Department of , Busan Paik Hospital, College of Medicine, Inje University, Busan, Korea

Pityriasis alba (PA) is a common benign disease, characterized by hypopigmented macules or patches on the face, usually seen in children. However, two uncommon variants exist, a pigmenting type and an extensive type. Extensive PA is rare. The tend to be less scaly, more persistent, more generalized, more symmetrical, and more frequently seen over the trunk and less so over the face. We report a child who had extensive PA lesions. (Ann Dermatol (Seoul) 20(3) 146∼148, 2008)

Key Words: Extensive, Hypopigmented,

INTRODUCTION months ago, they started as small hypopigmented macules over the chest and gradually increased in Pityriasis alba (PA) is a common disease, usually number and size and spread to the entire body. The seen in children. The lesions are frequently limited lesions were asymptomatic and were accentuated to the face, but the neck, arms, and shoulders can with sun exposure. There was no under 1 also be involved . Initially the typical consists Wood's light. On physical examination, the lesions of an irregular, round or oval, pink patch, some- were symmetrically distributed over the whole body times with an elevated, and slightly erythematous and consisted of large hypopigmented patches with border, varying in size from a few millimeters to a indistinct borders. Laboratory examinations, in- few centimeters. After a few weeks, the usually fades, leaving a whitish macule covered by fine scales2. Atypical forms of PA are the extensive and pigmenting types. Extensive PA is characterized by typical PA lesions distributed in a generalized fashion. We present a case of extensive PA, which has not yet been reported in Korean literature.

CASE REPORT

A 4-year-old boy presented with multiple hypo- pigmented patches over his whole body (Fig. 1). 18

Received February 19, 2008 Accepted for publication March 17, 2008 Reprint request to: Deborah Lee, M.D., Department of Dermatology, Busan Paik Hospital, College of Medicine, Inje University, 633-165, Gaegeum-dong, Busanjin-gu, Busan Fig. 1. Multiple hypopigmented, non-scaling, and non- 614-735, Korea. Tel: 82-51-890-6135, Fax: 82-51-897-6391, coalescing patches with indistinct borders involving the E-mail: [email protected] whole body. A Case of Extensive Pityriasis Alba 147

Fig. 2. (A) Histologic examination of the hypopigmented patch revealed hyperkeratosis, focal spongiosis in the and perivascular inflammatory infiltration (H&E, ×200). (B) Irregularly distributed were positive for S-100 protein in the basal layer (S-100 protein, ×200). cluding chest PA, CBC, LFT, and UA were within pigmenting and an extensive PA. Pigmenting PA is normal limits. Potassium hydroxide (KOH) mount a variant that may be associated with superficial from a hypopigmented lesion was negative. The boy infection. Extensive PA is an entity had a history of xesosis for 2 years but no family characterized by typical PA lesions distributed in a history of extensive pityriasis alba or atopic der- generalized fashion, and seen more commonly in matitis. adults. Extensive PA is not preceded by erythema. Histopathological examination of the hypopig- The lesions tend to be less scaly, more persistent, mented patch revealed hyperkeratosis, acanthosis in and completely asymptomatic4. In addition, they are the epidermis, and perivascular inflammatory cells more generalized, more symmetrical, and more infiltration in the upper (Fig. 2A). On frequently seen over the trunk and less so over the immunoperoxidase staining, the irregularly distri- face5. In our patient, the lesions were not preceded buted melanocytes in the basal layer were positive by erythema and not scaly, completely asympto- for S-100 protein (Fig. 2B). Although the patient matic, and generalized. was treated with topical twice a day for 1 The pathogenesis of PA is still controversial. month, no regression of the lesions were observed. Weber et al studied the etiopathogenesis of PA6. They reported, the disease was more accentuated when the patient had poor hygiene and sun DISCUSSION exposure habits. And another important element in the development of the disease was associated with PA is a common disorder usually seen in the xerosis presenting in individuals with atopic children. PA often starts as a pink patch with an diathesis5. PA is also linked to vitamin deficiencies, elevated border varying 0.5 to 5 cm in diameter. lower serum levels of copper, wind, and soap6,7. After several weeks, the patch fades, leaving a paler The histologic changes in specimens are spot covered by a powdery white scale. This spot usually nonspecific1. PA has areas of hyperkeratosis, will progress to a smooth hypopigmented macule parakeratosis, acanthosis, and small amounts of that persists for about 1 year. The lesions are spongiosis. In the early stage, there is follicular frequently present on the face, arms, and shoulders. plugging and atrophic sebaceous glands. Perivascular Most patients are children between 6 and 16 years lymphocytic infiltrates and edema are evident in the of age. Boys are thought to be affected more dermis. The intermediate stage is characterized by frequently. There are two uncommon variants, a the damage to the hair follicle and spongiotic Annals of Dermatology 148 D Lee, et al. Vol. 20, No. 3, September 2008 edema. Late stage PA shows a finding of typical REFERENCES chronic and irregularly distributed mela- 4 nization . The histologic finding of our patient 1. Zaynoun ST, Aftimos BG, Tenekjian KK, Bahuth showed late stage PA. N, Kurban AK. Extensive pityriasis alba: a histolo- In the differential diagnosis, it is necessary to gical histochemical and ultrastructural study. Br J consider post-inflammatory , tinea Dermatol 1983;108:83-90. versicolor, , and depigmentosus. Post- 2. Zaynoun S, Jaber LA, Kurban AK. Oral inflammatory hypopigmentation can be ruled out photochemotherapy of extensive pityriasis alba. due to the absence of a preceding dermatitis. Tinea Preliminary report. J Am Acad Dermatol 1986;15: versicolor can be distinguished by KOH mount of 61-65. the lesion. In vitiligo, the contrast between normal 3. Dhai S, Kanwar AJ, Dawn G. Pigmenting pityriasis and affected skin is greater than that of PA. Nevus alba. Pediatr Dermatol 1995;12:197-198. depigmentosus can be distinguished from PA 4. Lin RL, Janniger CK. Pityriasis alba. Cutis 2005;76: because 92.5% are present before the age of 2 and 21-24. have well-defined borders. In this case, the skin 5. Sandhu K, Handa S, Kanwar AJ. Extensive pity- lesion presented at the age of 3 without preceding riasis alba in a child with . Pediatr dermatitis. KOH mount from the lesion was Dermatol 2004;21:275-276. negative. There was no fluorescence under Wood's 6. Blessmann Weber M, Sponchiado de Avila LG, light. The lesions were hypopigmented patches with Albaneze R, Magalhães de Oliveira OL, Sudhaus indistinct borders. BD, Cestari TF. Pityriasis alba: a study of patho- Treatment for patients with PA is assuring genic factors. J Eur Acad Dermatol Venereol 2002; patients that the disorder is self-limiting and not 16:463-468. dangerous. Clinicians should recommend measures 7. Bassaly M, Miale A Jr, Prasad AS. Studies on pity- that limit an individual's exposure to possible etio- riasis alba: a common facial skin lesion in Egyptian logic factors, such as decreasing sun and wind children. Arch Dermatol 1963;88:272-275. exposure, regular use of , and reducing the 8. Galadari E, Helmy M, Ahmed M. Trace elements frequency and temperature of baths. Accepted in serum of pityriasis alba patients. Int J Dermatol treatments include emollients and lubricants. Exten- 1992;31:525-526. sive PA has been found to resolve with - 9. Fujita WH, McCormick CL, Parneix-Spake A. An UVA therapy2. In recent studies, exploratory study to evaluate the efficacy of pime- cream 1% and ointment 0.1% were crolimus cream 1% for the treatment of pityriasis proved as effective treatment5 for typical PA9,10. alba. Int J Dermatol 2007;46:700-705. To the best of our knowledge, our case is the first 10. Rigopoulos D, Gregoriou S, Charissi C, Konto- case of extensive PA in Korean literature. Therefore christopoulos G, Kalogeromitros D, Georgala S. we report this rare and interesting case of extensive Tacrolimus ointment 0.1% in pityriasis alba: an PA. More experience in this rare condition and trial open-label, randomized, placebo-controlled study. of various treatment modalities are needed. Br J Dermatol 2006;155:152-155.