Zrinjka Paštar

Case of concurrent epidermolysis bullosa acquisita and anti‐p200 pemphigoid – how to treat it?

Epidermolysis bullosa acquisita (EBA) is an uncommon, chronic, acquired, autoimmune, subepidermal blistering disease. The etiology of the disease is unknown. EBA antibodies bind to type VII collagen within anchoring fibrils at the dermo-epidermal junction. This results in a decreased number of anchoring fibrils, which therefore do not anchor the epidermis and its underlying basement membrane to the papillary dermis. To date, five clinical presentations of EBA have been described. 1 In general, the clinical features of classical EBA include increased skin fragility and skin lesions on the areas predisposed to trauma. EBA also affects the mucous membranes. The first two cases of EBA were reported and distinguished from dystrophic epidermolysis bullosa by Elliot in 1895. We present a patient with clinical, histological and immunological features of a mixture of EBA and anti-p200 pemphigoid.

Verruciform xanthoma in recessive dystrophic epidermolysis bullosa Hallopeau-Siemens.

Epidermolysis bullosa is divided into three major types according to the level of cleavage: Epidermolitic EB simplex, junctional EB, and dystrophic dermolitic EB. 2–7 The most comprehensive classification scheme, based on the recommendation of the Subcommittee of the National EB Registry (USA) and an international consensus group, has attempted to incorporate molecular and clinical findings and combine certain phenotypes that cannot be distinguished by modern molecular analyses. 3,4

Cutaneous Adverse Drug Reactions Caused by Antituberculosis Drugs

Multidrug antituberculosis regimen is associated with diverse clinical patterns of cutaneous adverse drug reactions (CADR), ranging from mild and moderate such as pruritus, maculopapular exanthems, lichenoid eruptions, fixed drug eruptions and urticaria to severe and even life threatening ones like acute generalized exanthematous pustulosis (AGEP), Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN). These adverse reactions to antituberculosis drugs are commonly observed adverse events. This is of particular importance for high HIV prevalence settings and developing countries where tuberculosis is common infection resulting in higher occurrence rate of these reactions. There is still significant heterogenity in definition and classification of CADR, as well as diversity in treatment modalities following adverse reactions and rechallenge management. The aim of this review is to discuss clinical presentation, occurrence of CADR caused by antituberculosis drugs, to identify risk factors for intolerance of the standard therapy as well as to draw attention to importance of multi-disciplinary approach, early detection, prompt diagnosis and in time management of antituberculosis drugs associated CADR. CADR can cause significant treatment interruption and alteration, resulting in increased risk of treatment failure, drug resistance, relapses and increased risk of complications including even lethal outcome. Finally, it can be concluded that it is of great importance to identify the best possible treatment and preventive regimens in order to enable continuity of the antituberculosis therapy to the full extent.

Intertriginous eruption induced by terbinafine: a review of baboon syndrome.

A 60-year-old otherwise healthy man presented with a 1-week history of a mildly stinging erythematous eruption involving the buttocks and the flexural areas, including the axillae and the groins. His past medical history was significant for a recently diagnosed tinea pedis for which he was prescribed terbinafine HCl 125 mg tablets twice a day for 2 weeks about 10 days prior. On the third day of initiating treatment with terbinafine, a flexural rash was noted affecting the axillae. Despite the rash, he continued taking the medication for another few days, but as the rash worsened and progressed to involve the groins and upper inner thighs, he consulted his GP who immediately stopped the patient’s medication and referred him to a dermatologist. Physical examination revealed an erythematous eruption symmetrically involving the axillae, sides of the trunk, midsternal groove, antecubital fossae, groins, and the perianal and gluteal areas. It appeared as large welldemarcated dusky red patches with central confluence (Fig. 1). Superficial peripheral desquamation was also noted particularly around the axillae (Fig. 1). There were no raised or advancing margins at the periphery, and the superficial scrapings from the multiple affected sites showed no fungal elements on microscopy of the potassium hydroxide-treated samples. He was otherwise well, and there was no recent history of any upper respiratory tract infection, pharyngitis, or fever. He had not taken any other medications recently except terbinafine. According to his previous medical record, he had not been prescribed topical and/or oral terbinafine before. There was no personal and/or family history of atopic diatheses or any other drug hypersensitivity. His routine blood and urinalyses were unremarkable. Antistreptolysin titer was also within normal limits. Patch tests, carried out with terbinafine HCl, prepared in petrolatum and water as a vehicle, at different concentrations (5, 10, and 30% according to the guidelines of the International Contact Dermatitis Research Group) remained negative after 48 and 72 hours. Histopathologic examination of the biopsy from the affected sites showed a mixed, predominantly mononuclear perivascular infiltrate in the dermis. After clinicopathologic correlation, a diagnosis of drugrelated baboon syndrome (BS) or symmetrical drugrelated intertriginous and flexural exanthema (SDRIFE) was made. A drug provocation test was refused by the patient. He was treated with saline compresses and mild topical steroids. The eruption appeared to have cleared with desquamation when the patient was seen at the follow-up examination after 1 week.