BACKGROUND:Hydroxyurea (HU) is an antimetabolite agent that interferes with the S-phase of cellular replication and inhibits DNA synthesis, with little or no effect on RNA or protein synthesis. It is used in the treatment of many myeloproliferative disorders (MD) and is particularly a first line treatment drug for intermediate to high-risk essential thrombocythemia. Although safe and very well tolerated by the patients suffering from MD, there have been numerous reports of a broad palette of cutaneous side effects associated with prolonged intake of the medication. These may include classical symptoms such as xerosis, diffuse hyperpigmentation, brown-nail discolouration, stomatitis and scaling of the face, hands, and feet or more serious side effects such as actinic keratosis lesions, leg ulcers and multiple skin carcinomas.CASE REPORT:We report a case of a 52-year-old man, on long-term therapy with HU for essential thrombocytosis, with several concurrent skin lesions. Despite the perennial use of HU, the cutaneous changes were neglected. The local dermatological examination revealed oval perimalleolar ulcer on the right leg, with dimensions 6 x 4 cm, clearly demarcated from the surroundings with regular margins, periulcerous erythema, with very deep and highly fibrinous bed of the ulcer, positive for bacterial infection. The ulcer was treated with topical wound therapy with alginate and parenteral antibiotics. The extended dermatological screening also showed two nummular lesions in the right brachial region, presenting as erythematous papules with sharp margins from the surrounding skin, gritty desquamation and dotted hyperpigmentations inside the lesion. Further dermoscopy and biopsy investigations confirmed a diagnosis of basal cell carcinoma. Nasal actinic keratosis was also noted. The patient was advised for discontinuing or substituting the HU therapy.CONCLUSION:We present this case to draw attention to the various cutaneous side effects that occur with perennial HU use and suggest an obligatory reference to a dermatological consult.

Lichen planus (LP) pigmentosus inversus (LPPI) is a variant of LP, pre

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