Lichen sclerosus (LS) is an uncommon chronic inflammatory skin disorder with a predilection for the anogenital area, characterized by porcelain white papules, plaques and atrophic patches. We report a prepubertal, 12-year-old girl who presented with chronic, disseminated pearly, flat-topped papules, plaques and atrophic patches located on the trunk, limbs and in the anogenital area, consistent with LS based on clinical and histologic findings. Potent and ultrapotent topical corticosteroids should be considered as first-line treatment. The ultraviolet A1 (UVA1) and calcipotriol for extragenital lesions, as well as calcineurin inhibitors for anogenital lesions, are other treatment options for pediatric LS. Lichen sclerosus is an uncommon chronic inflammatory skin disorder with a predilection for the anogenital area, characterized by porcelain white papules, plaques or atrophic patches. It was first described in 1887, by Hallopeau. Since then, many synonyms have been in use, notably 'guttate scleroderma,' 'white spot disease,' 'dermatitis lichenoides chronica atrophicans" or Csillag's disease,' 'kraurosis vulvae,' 'vulvar dystrophy, 'balanitis xerotica obliterans', 'lichen albus or Von Zumbusch Disease', or 'lichen sclerosus et atrophicus'. Since not all cases of LS et atrophicus exhibit atrophic tissue, 'et atrophicus' was dropped and replaced by 'lichen sclerosus,' which is now used for genital and extragenital lesions (1). LS is mostly seen in females with two peak ages of presentation: prepubertal girls and postmenopausal women. The prevalence rate of vulvar LS ranges between 1:70 to 1:1000 in women and 1:900 in children (2, 3).The patogenesis of LS has not yet been completly elucidatet; however, genetic factors and autoimmunity have been implicated. The most common autoimmune diseases associated with LS were autoimmune thyroiditis (12 %), alopecia areata (9%), vitiligo (6%), and pernicious anemia (2%) (4, 5). Hormonal, environmental, and infectious factors have also been implicated as possible causes of this disease.Initially, the lesions are pearly, flat-topped papules that coalesce into plaques, over time becoming atrophic patches. Telangiectasias, follicular plugging and bullous lesions may also be seen. The most common location is the anogenital area, although extragenital lesions can be present, mostly affecting buttocks, breasts, submammary area, neck, back, chest, axillae and wrists. Oral mucosal involvement has also been reported. Extragenital lesions are rare, especially in children.We report a prepubertal female child with LS with genital and disseminated extragenital lesions. Case ReportA 12-year-old prepubertal girl with a 12-month history of numerous mildly pruritic hypochromic lesions distributed on the trunk, limbs, and genital area was referred to the University Clinic of Dermatology, Skopje, Macedonia. She had visited several physicians due to these complaints which were misdiagnosed as vitiligo, tinea, psoriasis and verrucae planae. She used various ointments such as

Tuberous sclerosis complex is a multisystem, autosomal dominant disorder affecting children and adults, which results from mutations in either of two genes, TSC1 (encoding hamartin) or TSC2 (encoding tuberin). Tuberous sclerosis complex often causes disabling neurologic disorders, including epilepsy, mental retardation, and autism. Major features of the disease include dermatologic manifestations, such as facial angiofibromas, renal angiomyolipomas, and pulmonary lymphangiomyomatosis. We report a 20-year-old woman with epilepsy and subnormal intelligence, who was admitted for evaluation of multiple facial papules that have gradually increased in number over the past 15 years. She had been previously diagnosed with tuberous sclerosis complex based on findings of cardiac ventricular rhabdomyomas, tuberosclerotic nodules of glial proliferation in the cerebral cortex, and renal angiomyolipoma. The facial papules were angiofibromas, confirming the clinical presentation of tuberous sclerosis complex. Detailed examination of the skin and mucosa revealed Shagreen patches, nontraumatic subungual and gingival fibroma, all features of tuberous sclerosis complex. A multidisciplinary team approach was used for diagnosis and medical care of tuberous sclerosis complex in order to treat many organ systems affected by tuberous sclerosis in our patient. The patient received antiepileptic medications, while rapamycin was recommended.

Kyrle's disease (KD) is a rare variant of acquired perforating dermatosis (APD), associated with systemic diseases in adults, particularly chronic kidney disease (CKD) and diabetes mellitus (DM). Hyperkeratotic papules of KD are clinically important as a sign of a systemic disorder that are often misdiagnosed, so clinicopathological correlation is needed to establish the diagnosis. We report a rare case with longstanding KD, associated with endstage renal disease (ESRD) on hemodialysis for 25 years that was repetitively misdiagnosed as folliculitis, excoriations and prurigo nodularis. The KD skin changes started to develop during earlier stages of CKD, before the kidney disease was suspected, and continued to appear in flares with extensive lesions when the systemic disease in the background was not under control. Dermoscopy revealed a 3-zonal concentric pattern, characterized by bright whitish scales in the centre, a structureless whitish-grey area surrounding the central crusts, and a peripheral, rose/brown pigmentation. Histopathological examination revealed moderately acantathotic epidermal invagination filled with a keratotic plug admixed with cellular debris and neutrophils, and a modest parakeratosis. Our goal is to emphasize that the accurate and timely diagnosis is vital to be able to monitor patients for a life threating, systemic disease such as kidney failure, and attaining better management of the dermatological status which can become longstanding and hindering, as well as to outline the importance of the multidisciplinary approach to improve outcomes in patients affected by KD.