Mirjana Paravina

Cheilitis Glandularis Apostematosa in a Female Patient – a Case Report

Abstract 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. Sažetak Heilitis (Cheilitis) inflamatorno je oboljenje rumene zone usana (vermiliona) koja se nalazi na prelazu kože u sluzokožu. Heilitisi koji nastaju kao samostalna oboljenja mogu biti površni ili duboki. Duboki su Cheilitis glandularis i Cheilitis granulomatosa. Cheilitis glandularis (CG) retka je bolest koja najčešće zahvata donju usnu i karakteriše je nodularno uvećanje, redukovani mobilitet i everzija usne. Kliničke varijante su CG simplex (Puente and Acevedo), CG suppurativa superficiallis (Baelz-Unna) i CG suppurativa profunda seu CG apostematosa (Von Volkmann). Mi prikazujemo bolesnicu sa dubokom supurativnom formom heilitisa na obema usnama, kod koje je sistemska primena antibiotika, prema antibiogramu, i kortikosteroida, uz lokalnu terapiju, dovela do znatnog poboljšanja.

Brooke spiegler syndrome-a case report

Brooke Spiegler syndrome (BSS) is a rare autosomal dominant hereditary disease with variable penetration.1,2 It is more common in females,3 usually in second and third decades of life.4 Brooke Spiegler syndrome is manifested by the appearance of three types of tumors. Cylindroma’s are different number and size, from few to multitude, covering the entire scalp, usually without any symptoms. Typical trichoepithelioma in the form of yellowish papules and small tumorous nodules are usually on the face, and they cause psychosocial problems in patients due to location.5 Spiradenoma, in the form of individual tumorous formation due to the location is most frequently localized in the upper part of the trunk, upper extremities, and rarely on the chest.

Lichen Planopilaris, Dijagnosis and Therapy Challenge - Case Report

Background: Lichen planopilaris is relatively rare inflammatory disorder that results in cicatricial alopecia. It is a rare, cutaneous form of lichen planus which affects hair follicles, most commonly on the scalp area. It is caused by an autoimmune disorder that leads to follicular destruction and permanent hair loss. Case history: We are presenting the case of a patient who is 21 years old and who suffers from cicatricial alopecia and follicular hyperkeratosis of the parietal region of the scalp. Initially, she was diagnosed with seborrheic dermatitis and has been treated with local corticosteroid therapy for several months by her doctor. She was admitted to the Department of Dermatology with lessions located on the scalp. A clinical examination revealed exudative erythema with prominent circumferential hair follicle openings and the presence of sticky, yellowish crusts and scales whose removal caused bleeding. There were a several locations with significant cicatricial alopecia which look like porcelain. Dermoscopy showed perifollicular squamas. Routine laboratory analyses and immunological analysis were in normal range. Finally, after taking a biopsy and histopathological findings, we have come to a differential diagnosis which may include fibrous and suppurativ follicular disorders, as well as lichen planopilaris. The patient was treated with keratolytic lotions, combination of corticosteroid preparation and salicylic acid applied topicaly, and tacrolimus 1% cream which was used twice per day for several months. This terapy was successful, crusts did not return and progression of the disease was stopped.

A case report of a female patient with Brooke-Spiegler syndrome

Brooke–Spiegler sindrom (BRSS) retka je autozomno dominantna nasledna bolest s varijabilnom penetracijom (1, 2). Češće se javlja kod žena (3), obično u drugoj i trećoj dekadi života (4). Brooke–Spiegler sindrom se manifestuje pojavom tri vrste tumora: multipli cilindromi, trihoepiteliomi i spiradenomi. Svi tumori kože počinju da se javljaju nakon dvadesete godine, prvo na poglavini, a zatim i na drugim delovima tela (5). To su cilindromi, različitog broja i veličina, od nekoliko do multiplih promena, koji prekrivaju čitavu poglavinu, obično bez subjektvnih simptoma. Tipični trihoepiteliomi, u formi žućkastih papula i malih nodula, obično se nalaze na licu i uzrok su psihosocijalnih problema obolelih zbog njihove lokalizacije (6). Spiradenomi u formi pojedinačnih tumora najčešće su lokalizovani na gornjim delovima trupa, gornjim ekstremitetima i retko na grudima.

Cutaneous Sarcoidosis in a patient with left Hilar calcification of the lungs - A Case Report

Abstract Sarcoidosis is an acquired idiopathic granulomatous disease, which is characterized by noncaseating epithelioid granulomas in organs and tissues. Most frequently it affects the lungs, liver, lymph nodes, skin, eyes and other organs. The cutaneous lesions appear in 20 - 30% of patients with systemic manifestations, and in 25% of them they appear without systemic manifestations. Based on the histopathological characteristics, cutaneous lesions are divided into specific, characterized by cutaneous granuloma, and non-specific, which are not granulomatous. Moreover, they can be classified as typical and atypical. We are presenting a female patient with unilateral hilar calcification of the lungs, who exhibited plaque skin lesions typical for sarcoidosis, with a specific granulomatous histology and a favorable response to corticosteroid and antimalarial therapy.

Scleromyxedema (Arndt-Gottron Syndrome): a Case Report

Abstract Lichen 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, confluent, 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, fibroblast 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 fibrosis in the middle dermis, increased fibroblasts; 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 purified IgG paraprotein itself has no direct effects on fibroblast 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.

Yellow Nail Syndrome - a Case Report

Abstract Yellow nail syndrome is a rare disease of unknown etiology. It is clinically characterized by a triad of yellow nails, lymphedema at one or more sites, and chronic respiratory disease (bronchitis, bronchiectasis and rhinosinusitis). All nails may be affected, but some may be spared. The nail plates are yellowish green, thickened, occasionally with transverse ridging and onycholysis, with increased longitudinal and transversal over-curvature, with partial or complete separation of the nail plate from the nail bed, without lunula and cuticle and slow nail growth rate. The lymphedema is usually peripheral, affecting the lower limbs, or in the form of pleural effusion. This is a case report of a 47-year-old female patient who presented with nail changes at the age of 40; two years later the patient developed lymphedema of the lower limbs, and a year later a chronic respiratory disease. The affected nails were yellow to yellow-gray, with thickened nail plates separated from the nail bed, ingrown in the perionychium, without lunula. At the same time, additional examinations revealed the following associated conditions: edema of talocrural joints in both legs, chronic obstructive bronchitis, bronchial asthma, chronic rhinitis with bilateral nasal polyposis, labile arterial hypertension. Apart from the management of chronic respiratory disease, oral vitamin E capsules (200 mg 3 times a day) and topical vitamin E solution were administered over 15 months. The nails began to grow, and the newly grown nails were of normal pigmentation. In conclusion, we present a case of an adult female patient with yellow nail syndrome, and a recognized association of peripheral edema and chronic pulmonary disease. The patient had a typical clinical picture, all the nails were affected, but showed a favorable response to systemic and topical vitamin E therapy.

Treatment of Verrucous Epidermal Nevus by Radiofrequency Ablation: a Case Report

Abstract Verrucous epidermal nevi are noninflammatory, congenital, cutaneous hamartomas composed of keratinocytes, abnormal clone(s) of cells that reflect genetic mosaicism arising from different somatic mutations. Some of these mutations are well recognized, but some are still unidentified. Molecular techniques are used for identification and classification of molecular causes of certain epidermal nevi, whereas all verrucous epidermal nevi are divided into epidermolytic and non-epidermolytic types. They are typically present at birth, but may appear during childhood, even later. Their prevalence in adults ranges from 0.1 to 0.5%, equally affecting both sexes, and about 1 in 1000 newborns. Warty, brown papules without inflammation distinguish verrucous nevi from other epidermal nevi, while presence at birth and persistance distinguish verrucous epidermal nevi from linear viral warts. Epidermolytic and non-epidermolytic verrucous epidermal nevi are almost always hard to distinguish, except by histology. As a rule, verrucous epidermal nevi are asymptomatic, they have a benign course, except occasionally, and therapy is mostly used for cosmetic reasons. Simple excision is usually the treatment of choice. Topical agents are rarely curative, as well as surgery which is associated with relapses, unless both epidermis and the underlying dermis are removed or destroyed at the same time. We present a case of an otherwise healthy 21-year-old female patient, who presented with a solitary congenital verrucous cauliflower-like lesion in the right zygomatic region of the face. The lesion was present from birth. Due to its gradual growth during years, the lesion became a great esthetic and functional problem for this young patient. There was no family history of similar or any other tumorous skin lesions in the family. On examination, the patient had a solitar unilateral, well defined yellowish cauliflower-like verrucous lesion confined to the right malar side of the face. The lesion was distributed along the lines of Blaschko extending horizontally, from its wider 1.5 cm cauliflower-like part on the right zygomatic region, towards its tail-like 0.5 cm thick end on the preauricular region, in approximately 3 cm long tail-like manner without crossing the midline. Since the patient refused biopsy, no exact differentiation between epidermolytic and non-epidermolytic nevi was possible. The diagnosis of verrucous epidermal nevus was based on history and clinical presentation, as a diagnosis of exclusion. Due to the fact that patients with epidermolytic verrucous epidermal nevi are at risk of parenting a child with bullous ichthyosiform erythroderma, the patient was counseled on this risk, and on the possibility of first-trimester antenatal diagnosis. The lesion was successfully treated by radio-wave surgery.

CREST Syndrome - a Limited Form of Systemic Scleroderma: a Case Report and Literature Review

Abstract Systemic scleroderma (SSc) is a multisystem disease with microvascular abnormalities, autoimmune disorders, excessive collagen production and deposition, and fibrosis of the skin and internal organs. According to the simplest, though incomplete classification, there are two forms of SSc: diffuse and limited (formerly acrosclerosis). CREST syndrome is a subtype of limited SSc, characterized by: calcinosis, Raynaud’s phenomenon, esophageal dysfunction, sclerodactyly, and telangiectasia. We present a patient with all the features of the CREST syndrome, which appeared at the age of 43 and lasted for 23 years. The patient presented with a gradual development of symptoms during the first ten years, from Raynaud’s phenomenon, skin sclerosis, calcinosis, telangiectasia, and esophageal dysmotility. The diagnosis was based on clinical findings and relevant diagnostic procedures. The article presents a literature review on the epidemiology, etiology, pathophysiology, clinical manifestations, various attempts at classification, diagnostic criteria, and therapeutic modalities. When classifying systemic scleroderma into two main types — diffuse and limited, with CREST syndrome as a variant of the latter, it should be pointed out that both types represent clinical forms of systemic sclerosis, share similar visceral involvement, laboratory abnormalities and course which is variable, as was the case in our patient.

Pityriasis Rubra Pilaris: A Report of Two Cases and Literature Review

Abstract Pityriasis rubra pilaris (PRP) is an idiopathic inflammatory hyperproliferative chronic dermatosis characterized by: perifollicular coalescing papules with central keratotic acuminate plugs gradually submerged in sheets of erythema; perifollicular erythema with islands of unaffected skin; palmoplantar keratoderma; diffuse desquamation which typically spreads from the head down to the feet. The cause of the condition is unknown, but possible etiological factors include: vitamin A deficiency, trauma, infections, autoimmune mechanisms, and malignancies. Taking into account different age of onset, clinical course, morphology and prognosis, there are six different types of the disease: two in adults (classical and atypical); three in children (classical, circumscribed and atypical); one in individuals infected with human immunodeficiency virus. This paper presents two male patients with clinical symptoms of classical PRP, 53 and 69 years of age at the onset of the disease, with rapid generalized involvement, typical erythematous perifollicular papules, islands of unaffected skin, palmoplantar hyperkeratosis with a waxy appearance and nail changes. The diagnosis was based on clinical findings and histopathologic analysis. Apart from topical therapy with emollients, corticosteroids and keratolytics, they received systemic retinoids and corticosteroids, which resulted in improvement of skin lesions. It is extremely important to consider the possible triggering factors, establish the diagnosis as soon as possible and begin proper treatment.