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Open access

Lidija Kandolf-Sekulović

Abstract

Toxic epidermal necrolysis is an idiosyncratic drug reaction which manifests with extensive epidermal detachment due to the massive keratinocyte apoptosis, mucous membrane involvement, and potentially lethal outcome. It is caused by adverse reactions to drugs, mostly idiosyncratic, unpredictable and independent of the applied dose, which develops 7-21 days after initiation of the drug, and is most commonly caused by the following drugs: sulfonamides, allopurinol, carbamazepine, phenobarbitone, phenytoin and oxycam group of nonsteroidal anti-inflammatory drugs. The treatment outcome depends on several factors, while older age, multiple drug use, late exclusion of the drug inducing toxic epidermal necrolysis, raised serum levels of urea, creatinine and cytopenia are poor prognostic indicators which are rated in SCORTEN scoring which proved to be of great help in the assessment of disease outcome. The basic approach to the treatment is early diagnosis, immediate suspension of the probable inducing drug, and emergency transport to the closest burn center, since treatment in burn units is associated with a lower risk of infection and mortality of these patients. Exclusion of the drug that induced toxic epidermal necrolysis, and supportive therapy, is the first and only therapy for which there is a consensus in different centers. Various forms of adjuvant therapy are also applied: in France, supportive therapy is a standard of care, in Germany it is short-term use of high-dose corticosteroids, while in USA, in the last decade high-dose intravenous immunoglobulins are the most widely accepted treatment modalities. Case reports and small patients’ series described therapeutic effects of plasmapheresis, cyclosporine and other immunosuppressants. In conclusion, elimination of the possible causal agent, rapid transport to the burn unit, and multidisciplinary approach to treatment are of utmost importance for favorable outcome of the disease with 20-30% mortality rate. An update on diagnosis and the treatment of toxic epidermal necrolysis is provided in this review.

Open access

Lidija Kandolf-Sekulović and Tatjana Radević

Abstract

Adverse drug reactions may be defined as undesirable clinical manifestations resulting from administration of a particular drug; this includes reactions due to overdose, predictable side effects, and unanticipated adverse manifestations. Adverse drug effects on the skin are among the most frequent reactions and, according to a study, account for approximately 14% of all adverse drug reactions. However, the incidence of cutaneous adverse effects in general population is unknown. Systemic drug administration results in various cutaneous adverse reactions, and medications used in the treatment of skin diseases themselves have their own adverse effects. Adverse drug reactions include a wide range of effects, from harmless exanthema of short duration, urticaria to systemic cutaneous reactions such as drug rash with eosinophilia and systemic symptoms (DRESS) or toxic epidermal necrolysis. Exanthematous eruptions and urticaria are the two most common forms of cutaneous drug reactions. Less common include fixed eruptions, lichenoid, pustular, bullous and vasculitis reactions. The most severe cutaneous and mucosal adverse drug reactions are epidermal necrolysis, which is usually drug-induced, DRESS syndrome, and acute generalized exanthematous pustulosis. Therefore, the diagnostic of adverse drug reactions requires a detailed history of drug intake and development of skin disorders, excellent knowledge of clinical presentations for a wide range of drug-induced skin reactions as well as of the very medications being taken by patients. In addition to details on drug intake, it is necessary to learn about taking herbal and alternative preparations, which may also cause adverse reactions. A drug started within 6 weeks of the development of disorders is considered the most common cause of adverse reaction, as well as drugs taken periodically but regularly. Once a reaction has occurred, it is important to prevent future similar reactions with the same drug or a cross-reacting medication. Early withdrawal of all potentially responsible drugs is essential, particularly in case of severe drug reactions.

Open access

Tanja Tirnanić, Dimitrije Brašanac and Lidija Kandolf Sekulović

Abstract

The term “nevi of special sites” refers to melanocytic nevi of specific anatomic locations including the breast, axillae, umbilicus, genitalia, flexural areas, acral surfaces, ear, scalp and the conjunctiva. Nevi from these anatomic sites display sometimes dermoscopic and histological features of melanoma, resulting in unnecessarily high rates of excisions and re-excisions. Some authors have categorized nevi excised in the axillary, breast, umbilical and perineal areas as the nevi of the milk line. Two patients, a 32-year-old female and 23-year-old male with breast and periumbilical pigmented lesions, presented to our Department during 2017. Dermoscopy revealed features that were highly specific for melanoma. Excisional biopsies were done and histopathology revealed benign nevi with present site-related atypia. Irregular blotches, non-uniform radial streaks, blue-gray veil, and regression are the most specific features of melanoma of the breast and flexural areas. Excision is always recommended in pigmented lesions on the breast and flexural areas, which exhibit these features. However, larger studies are needed to define specific criteria required to distinguish special-site nevi from melanoma.

Open access

Lidija Cvetković Jordanov, Lidija Kandolf Sekulović, Željko Mijušković, Lidija Zolotarevski and Radoš Zečević

Abstract

Concomitant occurrence of psoriasis and bullous pemphigoid was described in less than 100 cases in the literature. The co-occurrence affects the treatment approach of patients. We present a case of a 58-year-old man with psoriasis presenting with erythematous plaques, tense bullae, erosions and fever up to 39°C. Direct and indirect immunofluorescence and histopathological examination confirmed the diagnosis of bullous pemphigoid. In our case, bullous eruptions were successfully treated with oral methylprednisolone and dapsone, and psoriasis with narrowband ultraviolet B phototherapy and acitretin.

In conclusion, the etiopathogenesis of the coexistence of these two entities remains unknown, but it may be related to relatively high incidence of psoriasis and bullous pemphigoid, respectively. Both conditions are immunologically mediated and combined immunosuppressive regimens, directed at cellular and humoral immune responses, usually result in clinical 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.

Open access

Tatjana Vukanović, Željko Mijušković, Lidija Kandolf-Sekulović, Lidija Zolotarevski and Radoš Zečević

Abstract

Porphyria cutanea tarda is a metabolic disorder that results from a reduced enzymatic activity of uroporphyrinogen decarboxylase. It is the commonest chronic porphyria. Two types of this disease have been reported up to now: acquired (Type 1, 80%) and inherited (Type 2, 20%) an autosomal dominant pattern with low clinical penetrance. Both types are associated with haemochromatosis, alcohol abuse, estrogens, iron overload, hepatitis C virus infection, and halogenated aromatic hydrocarbons causing deficiency of the uroporphyrinogen decarboxylase enzyme in the liver. In this case report we described a 23-year-old woman with increased hair growth on the face and neck, who visited an outpatient dermatology clinic for laser hair removal due to excessive hair growth on the face and neck during the last eight years (Figures 1, 2). Four laser treatments were carried out with incomplete effects. After the fourth laser hair removal treatment, a small sore on the tip of the nose was observed. The patient used oral contraceptive pills during the past 8 months. No additional medications were taken. The diagnosis of porphyria cutanea tarda was confirmed by specific biochemical analyses, since increased excretion of uroporphyrin and coproporphyrin were detected. After discontinuation of drospirenone and ethinyl estradiol (YazÒ tablets) a gradual clinical and laboratory improvement was noticed suggesting a causative role of this drug. There are many published reports discussing and describing estrogens as contraceptive agents, hormone supplements for postmenopausal replacement therapy in females, and adjunctive hormonal therapy in males with prostatic carcinoma, being the probable trigger of porphyria cutanea tarda. However, the mechanisms by which estrogens exert their effects on disease expression have not yet been fully clarified. Conclusion: this case report points to the importance of hypertrichosis as the first manifestation of porphyria cutanea tarda, since it may be a long lasting sign before the onset of other clinical symptoms of the disese

Open access

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

Kristina Kostić, Miroslav Dinić, Željko Mijušković, Lidija Zolotarevski, Lidija Kandolf-Sekulović and Radoš Zečević

Abstract

Neurofibromatosis type I (NF1) is an autosomal dominant, multisystemic disease that usually affects the skin, nervous system and bones. Diagnosis is made by matching at least two of the following 7 diagnostic criteria: six or more caféau- lait macules over 15 mm in diameter, two or more neurofibromas, axillary and/or inguinal freckles, optic glioma, two or more Lisch’s nodules (iris hamartoma), changes in the bones in the form of sphenoid dysplasia, thinning of the cortex of long bones and existence of neurofibromatosis in the first degree relatives. We report three patients, two men and a woman aged 18 to 33 years, in whom the first changes occurred at puberty, and there was no positive family history in any of them. All three patients had café-au-lait spots over 15 mm in diameter and numerous localized neurofibromas on the skin of the trunk and extremities that were histologically verified. In two patients, ophthalmic examinations recorded Lisch’s nodules in the iris. In one of the patients, MRI of the head, revealed presence of oval lesions with diameters of 10-15 mm, which may correspond to neurofibromas, and in the other patient fibrous dysplasia of the femur and tibia were observed. Psychological testing in one patient revealed IQ at the lower limits of average (IQ 68). After the diagnosis of neurofibromatosis type I, the patients were given advice about the disease and a plan for the monitoring and control of possible symptoms, and also the possibility of genetic testing during pregnancy. A multidisciplinary approach is required for diagnosing and monitoring of patients with neurofibromatosis type 1.

Open access

Lidija Kandolf Sekulović, Miroslav Dinić, Tatjana Radević, Radoš Zečević, Lidija Zolotarevski and Vukojica Karličić

Abstract

Cutaneous manifestations of sarcoidosis are present in up to 25% of patients. The manifestatons can be very variable, making this disease one of the „great imitators“ in dermatology. One of its clinical variants is lichenoid sarcoidosis, which is more commonly described in children. We report an adult patient with extensive lichenoid sarcoidosis with a personal history of treated pulmonary tuberculosis, without any evidence of actual pulmonary involvement with sarcoidosis. The main differential diagnosis of lichenoid sarcoidosis of lichen scrofulosorum, so thorough examinations, to exclude active tuberculosis, are essential in theses cases. The patient was succesfully treated with antimalarial drugs, and lowdose systemic corticosteroids.

Open access

Lidija Kandolf-Sekulović, Bojana Cikota, Miroslav Dinić, Dušan Škiljević and Zvonko Magić

Abstract

The diagnosis of erythroderma is challenging, since clinical, histopathological and immunophenotypic findings are insufficient to differentiate between inflammatory and lymphomatous erythroderma. Thus, multiplex PCR was used for T-cell receptor-γ gene rearrangement analysis, in the skin and peripheral blood samples of 24 patients (20 men and 4 women) with erythroderma of varying origin, in order to estimate its diagnostic value. Cutaneous T-cell lymphoma was confirmed in 9, benign inflammatory dermatosis in 12, and idiopathic erythroderma and clonal dermatitis in 3 patients. In the group of patients with erythrodermic cutaneous T-cell lymphoma, the dominant clone was detected in the skin of 8/9, and in none of the patients with inflammatory dermatoses. A dominant clone was found in peripheral blood of 5/6 samples of patients with erythrodermic cutaneous T-cell lymphoma, and in 2/12 patients with inflammatory dermatosis. T-cell receptor-γ gene rearrangement analysis is valuable in differentiation between inflammatory and lymphomatous erythroderma, thus substantially improving the diagnosis of patients with erythroderma.