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Bullous systemic lupus erythematosus is a rare distinctive subepidermal bullous disease seen in patients with systemic lupus erythematosus (SLE). It has characteristical clinic, pathologic, and immunologic findings including antibodies to type VII collagen, laminin 332, laminin 331, and bullous pemphigoid antigen 230. Clinical presentation combined with histopathology, immunological testing, and concomitant diagnosis of SLE according to the criteria of American College of Rheumatology, are required to distinguish bullous SLE from these bullous diseases. In patients with bullous SLE, SLE disease progression and complications may be worse. Cicatricial pemphigoid is a chronic subepidermal blistering disease which is characterized by erosive lesions of mucous membranes and skin. Pathogenesis of cicatricial pemphigoid is characterized by linear deposition of Immunoglobulin G, A, or complement 3 along the epithelial basement membrane zone. The main target antigens are bullous pemphigoid antigens 180-230, laminin 331-332, type VII collagen, and β-4 integrin subunit. Cicatricial pemphigoid may lead to serious complications such as blindness and airway obstruction. Herein, clinical, histological, immunopathological features, the diagnosis and treatment of bullous SLE and cicatricial pemphigoid diseases are mentioned to raise awareness among the dermatologists about this important but rare heterogeneous bullous disease.

Özet Yaşam kalitesi, toplumların ulaşmayı amaçladığı en önemli genel hedeflerden birisi olup günümüzde yaşam kalitesini ölçmek için pek çok yöntem kullanılmaktadır. Bu ölçümler, dermatolojik durumlar için de özel bir öneme sahiptir; çünkü dermatolojik hastalıklar, kişilerin duygudurum ve sosyal yaşamını etkilemektedir. Bununla birlikte psikojenik faktörler birçok deri hastalığının patogenezinde rol oynamaktadır. Görülebilir bir cilt rahatsızlığı yaşayan insanlar, etraflarındaki kişilerden olumsuz tepkiler alabilirler ve bunun sonucunda sosyal ilişkilerden kaçınma davranışı sergileyebilirler. Tüm bunlar bireylerin yaşam kalitesini şekillendiren birer unsurdur. Bu derlemede dermatolojide kullanılan yaşam kalite ölçeklerine değinilmiştir. Anahtar Kelimeler: Dermatoloji, yaşam kalitesi, değerlendirme. QUALITY of LIFE in DERMATOLOGY Quality of life is a term that populations desire to reach in high levels. There are various types of scales developed for measuring this concept. These measurement methods have special attention for dermatological situations because these situations effect lots of parameters including mental status and sociality. Some unfortunate mental and emotional problems may play roles in the pathogenesis of dermatological diseases. Furthermore, people with some apparent skin diseases may avoid contacting with communities because of negative reactions. All these effect and formalise the quality of life. In this review, it is aimed to talk over scales about quality of life in dermatological diseases.

Acquired epidermolysis bullosa is a rare subepidermal bullous disease characterized by autoantibodies to type VII collagen, the major component of anchoring fibrils.Although the exact pathophysiologic mechanism remains unclear, reduction or perturbation of the anchoring fibrils results subepidermal blister formation and clinical features such as skin fragility, blisters, erosions, scars, milia and nail loss. Acquired epidermolysis bullosa includes various clinical manifestations resembling genetic epidermolysis bullosa, bullous pemphigoid, cicatricial pemphigoid, Brunsting-Perry pemphigoid and linear immunoglobulin A bullous dermatosis. Numerous treatment options are available but patients are often refractory to treatment. Linear immunoglobulin A bullous dermatosis is another subepidermal bullous disease characterized by the accumulation of IgA antibodies in lamina densa or sublamina densa region of the basement membrane and neutrophil-rich infiltrates in histopathology. It can be seen both in children and adults. The form seen in children usually begins under the age of 5 and it is called chronic bullous disease of childhood. The classical presentation is annular/polycyclic plaques and papules with blistering on perioral and perineal regions, giving a "cluster of jewels" appearance. The adult form is often seen after the fourth decade and clinical features are similar to those of dermatitis herpetiformis, bullous pemphigoid or cicatricial pemphigoid.

Unilateral laterotorasik ekzantem (çocukluk çağının asimetrik perifleksural ekzantemi) genellikle vücudun tek bir bölgesinde birleşerek plaklar oluşturmaya eğilimli, eritemli papüllerle karakterize bir hastalıktır. Sıklıkla lezyonlar tek taraflı aksilla ya da kasıktan başlar. Genellikle 1-5 yaş arası çocuklarda görülür. Etiyolojisi tam olarak bilinmemekle birlikte hastaların %75'inde döküntü öncesinde ateş, ishal, halsizlik, rinit gibi prodromal belirtilerin görülmesi viral ajanların etiyolojide yer aldığını düşündürmektedir. Histopatolojik bulguları nonspesifiktir. Tanı klinik olarak konulmaktadır. Hastalık kendi kendini sınırlayıcı özellikte olup, döküntü 2-6 haftada sekelsiz kaybolur. Üç yaşındaki erkek çocuk sağ koltuk altından gövdeye doğru uzanan çok sayıda, eritemli, kaşıntılı, milimetrik boyutlarda papüller ile polikliniğe getirildi. Unilateral Laterotorasik Ekzantem OlgusuThe Case of Unilateral Laterothoracic Exanthem Öz AbstractUnilateral laterothoracic exanthem (asymmetric periflexural exanthem of childhood) which have a tendency to form plaques in a particular region of the body is characterized by erythematous papules. The lesions usually begin at unilateral axillary or inguinal region. Unilateral laterothoracic exanthem is usually seen in children of 1-5 year-old. Although the etiology is not known very well, viral agents are considered to be the etiologic factor, because in 75% of the patients, prodromal symptoms like fever, diarrhea, fatigue and rhinitis were seen before rash. The histopathologic evidences are nonspecific. The disease is diagnosed clinically. This disease is self-limited and the rash disappears without sequel in 2-6 weeks. In this case, 3 year-old boy was presented with, multiple, erythematous, pruritic millimetric papules which first developed in right axillar region and extended to the same side of abdomen. It was found out that fatigue, loss of appetite, diarrhea before rashes were in the medical history of the patient. The serological examination of the patient's blood showed that ebstein barr virus, cytomegalovirus, rubella and measles immunoglobulin G were positive, immunoglobulin M were negative, parvovirus B19 immunoglobulin G and M were both negative. The patient was treated with topical moisturizer and oral antihistaminic. This case which was diagnosed with unilateral laterotoracic exanthema due to history of the disease, clinical findings and its rarity found fit to be presented.

Lichen aureus is a rare variant of pigmented purpuric dermatosis, which often affects young adults and is localized mainly on the lower extremities. We present here a 31-year-old female who had a solitary golden-brown-color patch on her right leg for 1 year. Punch biopsy of the lesion revealed lymphohistiocytic band-like infiltrate of the papillary dermis with erythrocyte extravasation and hemosiderin deposits. The diagnosis of Lichen aureus was made based on clinicopathologic findings. The dermoscopic findings that support this diagnosis were brownish yellow diffuse coloration of the background, round to oval red dots, globules and patches and twisted red loops. Herein, we present the dermoscopic findings of a 31-year-old female patient with a diagnosis of lichen aureus established with clinical and histopathological features. Keywords: pigmented purpuric dermatosis, lichen aureus, dermoscopy

Otoimmün progesteron dermatiti, nadir görülen ve endojen progesterona karşı gelişen bir hipersensitivite reaksiyonudur. Döngüsel olarak menstrüel siklusun luteal fazında başlayıp, menstrüel kanamanın başlamasından sonra sonlanan, kutanöz ve mukokutanöz bulgularla karakterize bir durumdur. Bu çalışmada, ürtikeryal lezyonlarla seyreden ve otoimmün progesteron dermatiti tanısı konulan 42 ve 49 yaşındaki iki olgu sunulmaktadır. Her iki hastanın da menstrüel kanamadan bir ya da iki hafta önce başlayıp, kanamayla birlikte sonlanan ürtikeryal döküntü öyküsü vardı. Parenteral progesteron preparatı ile yapılan intradermal testler her iki hastada da pozitif olarak saptandı. Hastaların öyküsü, klinik bulguları ve progesteron ile yapılan intradermal testin pozitif olması sonucunda otoimmün progesteron dermatiti tanısı kondu. Bu çalışma, nadir görülen bir hastalık olması nedeniyle tanının atlanabileceğini ve iyi bir anamnezin tanıdaki önemini vurgulamak amacıyla sunulmuştur. Öz AbstractAutoimmune progesterone dermatitis is a rare hypersensitivity reaction that develops against endogenous progesterone. This condition is characterized by cutaneous and mucocutaneous findings that begins cyclically in the luteal phase of the menstrual cycle and ends after the onset of menstrual bleeding. In this study, we present two cases aged 42 and 49 years who were had urticarial lesions and diagnosed with autoimmune progesterone dermatitis. Both patients had a history of urticaria rash that started one or two weeks before the menstruation and ended with bleeding. Intradermal tests with parenteral progesterone preparation were found to be positive in both patients. As a result of patients' history, clinical findings and the positive intradermal test with progesterone, autoimmune progesterone dermatitis diagnosed. It has been aim to emphasize the importance of anamnesis in diagnosis of this rarely seen disease.

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