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  • Author: Zorica Perić-Hajzler x
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Zorica Perić-Hajzler, Lidija Kandolf-Sekulović and Lidija Zolotarevski

Abstract

Psoriatic onycho-pachydermo-periostitis has been recognized as an uncommon subset of psoriatic arthritis, and to date, only a few cases have been reported. In general, psoriatic onycho-pachydermo-periostitis is regarded as a unique variant of psoriatic arthritis, but its pathology and pathophysiology are not well understood. Although psoriatic onychopachydermo- periostitis is usually found in patients with psoriasis, it can also be found in patients without psoriatic skin lesions. It is characterized by psoriatic nail changes (usually onycholysis), painful swelling of the soft tissue close to the distal phalanges, and radiographic changes of the distal phalanges with periosteal reaction and bone erosions. We present a 58-year-old man with a 3-year history of deformation, thickened nails and pustules on the skin of his fingers and toes, and painful redness of the nail bed accompanied with pain in small joints. The family history was negative. After confirmation of the diagnosis, methotrexate: 15 mg weekly, was initiated which led to symptoms improvement. Treatment of psoriatic onycho-pachydermo-periostitis is difficult. It is based on treatment modalities used for other forms of psoriatic arthritis, such as sulphasalazine, methotrexate, and anti-tumor necrosis factor antibody therapy with adalimumab and etanercept. Nonsteroidal anti-inflammatory drugs are usually ineffective. Retinoids, subungual cyclosporine and corticosteroid therapy also showed inefficient. In our patient, methotrexate has shown efficacy in symptom improvement.

Open access

Zorica Perić-Hajzler, Lidija Zolotarevski, Dušan Šofranac and Lidija Kandolf Sekulović

Abstract

Lichen planus is an acquired inflammatory disease of the skin, mucous membranes and nails. It is characterized by pruritic polygonal livid papules. The disease was first described by Erasmus Wilson in 1869. It is primarily a disease of adults, and it usually occurs between the ages of 30 and 60, without gender predominance. The exact incidence and prevalence of this disease are unknown, but it is thought to affect less than 1% of the general population (0.14 to 0.80%) (1).

A 63-year old male patient was admitted to our Department with itchy erythematous papules and plaques which appeared a month before admission. On admission, numerous erythematous and livid papules and plaques of polygonal shape up to 5 mm in diameter were present in the lines of Blaschko, along the left lower extremity, left side of the trunk and the left upper arm (Figures 1-3), while mucous membranes, nails and scalp were spared.

Blaschko-linear distribution of skin lesions was first described by a German dermatologist Alfred Blaschko in 1901 in his work ”The distribution of nerves in the skin and their relationship to diseases of the skin”. In 1978, Happle first published that genetic mosaicism was the cause of these peculiar skin changes (1,4,6). Although knowledge of mosaicism in the skin was further elucidated in articles of several authors (Taieb in 1994, Bolognia in 1994, Heide 1996), the exact mechanism and molecular basis for the development of Blashcko linear distribution has not been fully clarified yet (5). Blaschko lines may be related to X-linked, congenital and inflammatory dermatoses, and they may be found in several skin conditions like segmental forms of atopic dermatitis, erythema multiforme, pemphigus vulgaris, vitiligo, and granuloma annulare. This is a case report of a patient with a rare form of lichen planus, with typical clinical manifestations and with Blaschko-linear distribution. Lichen planus in the lines of Blaschko was also described in several other dermatoses: lichen striatus, lichen sclerosus, morphea, porokeratosis of Mibelli, mucinosis follicularis and psoriasis vulgaris. The treatment included topical corticosteroids under occlusion, due to comorbidities, with satisfactory response. Other options include, topical calcineurin inhibitors, intralesional and systemic corticosteroids, retinoids, phototherapy and in resistant cases that severely affect the quality of life methotrexate, cyclosporine and thalidomide.