Long‐term cyclosporin A in systemic sclerosis

Abstract

Laugier-Hunziker syndrome (LHS) is a rare disorder characterized by melanotic pigmentation of the mouth and lips which is frequently associated with longitudinal melanonychia. Laugier and Hunziker described lenticular melanotic pigmentation of the oral cavity and lips in 1970. 1 Some cases have been described in which the neck, thorax, abdomen, fingers and soles have been involved. Fingernails are more frequently involved than the toenails. 2 Melanonychia may also be seen with or after lichen planus (LP). 3,4 We report a case of LHS associated with actinic LP.A 37-year-old Turkish woman was referred to our clinic with melanotic macules on the face, a pruritic annular plaque on the malar area of her face and hyperpigmented streaks on her nails, which had appeared for 19 years, 10 months and 6 years, respectively.Examination revealed the patient's pigmented macules on the lips, gums, buccal mucosa and midline of the hard palate ( fig. 1a). There were melasma and pinkish annular plaque, with a hyperpigmented centre ( fig. 1b). There were longitudinal melanonychia on her fingernails ( fig. 1c) and toenails. Hyperpigmented slate-grey to brown macular lesions were seen over her back. Examination of intertriginous areas did not reveal any evidence of acanthosis nigiricans. The skin phototype according to Fitzpatrick's scale was IV.Results of routine laboratory tests were within normal ranges, including triiodothyronine (T3), thyroxine (T4) and thyrotropin (TSH). The patient had smoked for 20 years. Her mother had melasma. Histopathological examination of pigmented macula on the buccal mucosa showed an accumulation of melanin in the cells of the basal layer of the acanthotic epidermis and an increased number of melanophages in the upper dermis ( fig. 2). Histopathologically, there was thinning of the epidermis with vacuolar alteration of the basal layer, an artifactural cleft between the epidermis and the lichenoid infiltrate and pigment incontinence in the upper dermis on the annular lesion over the cheek (fig. 3).Since the first description by Laugier and Hunziker, many cases of LHS have been reported. 2,[5][6][7] In this syndrome, the essential lesions are manifested as lenticular-pigmented macules, which have different colours (from grey to brown to blueblack) and a smooth surface. Generally symptoms are absent. The most common sites are the lips (especially the lower lip) and the oral cavity (buccal mucosa and hard palate). Other sites such as labial comissure, the gums the floor of the mouth and the tongue are less frequent. Other locations such as the abdomen and the fingers have been involved. Fingernail involvement can present as a single longitudinal 1-2-mm-wide streak or as a double 2-3-mm-wide longitudinal streak on the lateral parts of the nail plate, as a homogeneous pigmentation of the radial or ulnar half of the nail, and complete pigmentation of the nail. All four types of nail involvement may simultaneously involve one or more nails of the fingers and /or toes. 2 fig. 1 (a) Multiple pigmented macules lo...

The Use of Cyclosporine A in Rheumatology: a 2016 Comprehensive Review

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Calcineurin inhibitors in systemic sclerosis – a systematic literature review

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“…Dunn 35 Gisslinger et al 39 Heickendorff et al 40 Hider et al 41 Ishida et al 43 Mehregan and Su 45 Mérot et al 46 Morton and Powell 47 Nunokawa et al 48 Patrick et al 49 Tooze et al 52 Measuring method during process Ishida et al 43 Morton and Powell 47 Patrick et al 49 Roch et al 51 Tooze et al 52 Vayssairat et al 53 Wörle et al 54 Yocum and Wilder 56 Zachariae and Zachariae 57 Zachariae et al 58 Measuring method during process Constantopoulos et al 34 Mérot et al 46 Nunokawa et al 48 Quartier et al 50 Measuring method during process…”

“…Filaci et al 37 Francès et al 38 Gisslinger et al 39 Ippoliti et al 42 Ishida et al 43 Roch et al 51 Number Pat. Lung findings before therapy N.A.…”