associated with systemic, even lethal manifestations, fi broblast proliferation and accumulation of acid mucopolysaccharides of the hyaluronic acid. Serum IgG class paraproteinemia is always present and it can be detected in all patients after appropriate or repeat testing (1,2,3).A case of an adult male with IgG paraproteinemia, in whom lichenoid papules evolved to scleromyxedema after two years, is reported. L ichen myxedematosus, also known as papular mucinosis, is a primary diff use cutaneous mucinosis. It is a rare cutaneous myxedematous condition characterized by formation of numerous lichenoid papules. Scleromyxedema, or ArndtGottron syndrome, is a rare, confl uent, papular and sclerotic variant of lichen myxedematosus, which is characterized by diff use thickening of the skin that underlies the papules. Th e condition is AbstractLichen myxedematosus, also known as papular mucinosis, is a primary diffuse cutaneous mucinosis. It is a rare cutaneous myxedematous condition characterized by formation of numerous lichenoid papules. Scleromyxedema, also known as Arndt-Gottron syndrome, is a rare, confl uent, papular and sclerotic variant of lichen myxedematosus, characterized by diffuse thickening of the skin underlying the papules. The condition is associated with systemic, even lethal manifestations, fi broblast proliferation and accumulation of acid mucopolysaccharides of the hyaluronic acid. Serum IgG class paraproteinemia is always present and it can be detected in all patients if appropriate or even repeat testing is used. Herein, we present a 67-year-old patient with a 2-year history of skin problems. He had no health problems other than hypertension and diabetes, wich were both diagnosed 15 years before. On examination, the patient exhibited sclerodermoid lesions with diffuse pseudo-sclerodermatous thickening of the exposed skin, microstomia and sclerodactyly-like changes; on the face, there were numerous solid, shiny 2 -4 mm in diameter skin-coloured lichenoid papules, scattered across the forehead, glabellar area, nasolabial folds, perioral region, ear lobes and the neck. Histopathological examination revealed: highly distinctive collagenosis and fi brosis in the middle dermis, increased fi broblasts; collagen bundles with irregular arrangement and fragmentation; alcian blue-positive deposits with appearance consistent with acid mucins. Serum protein electrophoresis detected IgG lambda paraproteinemia. The patient was treated with systematic corticosteroids during 9 months with subsequent introduction of methotrexate and showed satisfactory results. The etiology of scleromyxedema remains unknown, since the purifi ed IgG paraprotein itself has no direct effects on fi broblast proliferation. In scleromyxedema, numerous therapeutic modalities are proposed, unfortunatelly with limited effects. In colclusion, we report a case of an adult male with lichenoid papules; after a two-year progression, they evolved into scleromyxedema and exhibited well response to conventional therapy.
Pachydermodactyly is a rare, benign form of digital fibromatosis, characterized by asymptomatic and progressive, periarticular and usually symmetrical soft tissue finger swelling, specifically on the lateral aspects of the proximal interphalangeal joints mainly of the second, third, and fourth fingers; it mostly affects young adolescents and is probably due to repeated mechanical injury of the skin (such as repeated clasping or rubbing of crossed fingers), sometimes as a result of obsessive-compulsive disorder, which must be distinguished from obsessive “chewing pads”. This paper presents a male patient aged 19, who presented with first symptoms at the age of 12, and was diagnosed with periarticular hypertrophy: localized soft tissue thickening around the proximal interphalangeal joints of all fingers except the thumbs; slight hypertrophy of the skin; absence of subjective complaints; normal joint function. Dermatological status on admission revealed: symmetrical soft tissue swelling of all fingers of both hands except the thumbs at the level of the proximal interphalangeal joints; normal appearance of the distal parts of all fingers; thickening at the level of the proximal interphalangeal joints, bilateral, almost symmetrical hypertrophy (ulnar and radial) of phalanges of the affected fingers except both index fingers, affecting only the ulnar side. The skin lesions were pain-free on palpation, with homogeneous texture and elastic consistency, freely movable over underlying structures. The affected joints showed no functional deficit. The test results, sonography, radiography and histopathology confirmed our clinical diagnosis-pachydermodactyly. The review of the currently available literature, published between 1973 and 2014, including 99 papers and 160 patients, provided important insight into the characteristics and variations of the disease.
Cheilitis is an inflammatory condition of the vermilion border of the lips, which is the junction between the skin and the mucosa. Cheilitis may arise as a primary disorder of the vermilion zone; the inflammation may extend from the nearby skin, or less often from the oral mucosa. Primary cheilitis lesions are either superficial or deep. Deep types include cheilitis glandularis (inflammatory changes and lip gland swelling), and granulomatous cheilitis (chronic swelling of the lip due to granulomatous inflammation mostly of unknown origin). Cheilitis glandularis is a rare condition that mostly affects the lower lip and it is characterized by nodular enlargement, reduced mobility and lip erosion. Based on clinical presentation, cheilitis glandularis may be classified into three subtypes: simplex (described as Puente and Acevedo), superficial suppurative (described by Baelz-Unna), and the most severe type - deep suppurative, also known as cheilitis glandularis apostematosa (Volkmann’s cheilitis) characterized by deep-seated inflammation forming abscesses and fistulous tracts. This is a case report of a female patient with a deep suppurative type of cheilitis affecting both lips. Treatment with systemic antibiotics (using antibiogram tests), corticosteroids and topical therapy resulted in significant improvement.
Brooke Spiegler syndrome is rare autosomal dominant hereditary disease with variable penetration. It is more common in females, usually in the second and third decades of life. The syndrome manifests with the appearance of three types of tumors: multiple cylindromas, trichoepitheliomas and spiradenomas. We presented a female patient, 54 years of age. The first changes occurred at the age of 22, first on the forehead, and then on the scalp. She has a positive family history for this condition, obtained by anamnesis (mother, two sisters, brother and daughter). We presented a dermatologic status before and after surgical intervention. Pathohistological finding of the removed tumors: cylindroma. After surgical intervention, the patient's quality of life has improved. New surgical procedures are planned.
Lichen planus is an immune, infl ammatory reaction with characteristic clinical and histological lesions. It is a benign disorder, often chronic or recurrent, characterized by fl at-topped, pink to purple, shiny pruritic polygonal papules on the skin, or milky white reticular papules on the visible mucous membranes. Hypertrophic lichen planus is a chronic form of lichen planus with marked epidermal hyperplasia and intense pruritus. It is characterized by symmetrical hypertrophic plaques, usually located on the pretibial or perimalleolar regions. Lesions are often resistant to treatment. This paper presents a patient with a giant form of verrucous lichen planus on the lower extremities, with a chronic course and resistance to various forms of therapy (keratolytics, local and intralesional corticosteroids, radiotherapy, systemic antibiotics, cryotherapy). Signifi cant improvement was seen after 8-month treatment with etretinate (initial dose of 75 mg per day, with progressive reduction to 10 mg per day). Etretinate therapy resulted in a signifi cant regression of the disease. 6 (2): 73-80 M. Paravina Lichen planus hypertrophicus Ključne rečiLichen planus + fi ziopatologija + terapija; Etretinat; Ishod lečenja Serbian Journal of Dermatology and Venereology 2014; 6 (2): 73-80 M. ParavinaLichen planus hypertrophicus
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