Giulia Odorici

Autoantibodies in psoriatic patients treated with anti-TNF-α therapy.

TNF‐α inhibitors have been associated with induction of autoantibodies and autoimmune diseases. We retrospectively evaluated the incidence of autoantibodies ANA, ENA, anti‐dsDNA, the occurrence of clinical symptoms and possibly related treatment failure.

A 36-week retrospective open trial comparing the efficacy of biological therapies in nail psoriasis

Nail psoriasis occurs in about 50% of psoriatic patients and can be psychologically devastating since it appears on visible areas. Up to now there is no evidence about what biological drugs is the most effective on nail psoriasis.

Sex and the PASI: patients affected by a mild form of psoriasis are more predisposed to have a more severe form of erectile dysfunction

Psoriasis is a multi‐systemic disease involving the skin and joints, but it is also characterized by endothelial dysfunction, which may cause sexual impotence and erectile dysfunction (ED), an embarrassing disease frequently neglected by dermatologists.

Cutaneous collagenous vasculopathy: report of a case

an itchy facial rash. His medical history was unremarkable apart from acne vulgaris as a teenager. On physical examination, striking tumid erythematous papules, plaques and nodules were seen predominantly on the lower cheeks, and scaly erythematous plaques over the central forehead, upper cheeks, chin and neck (Fig 1a). The eyebrows, beard and scalp appeared normal with no obvious hair loss, and the cutaneous findings were limited to the head and neck. The differential diagnoses considered included granulomatous rosacea, sarcoidosis and lupus erythematosus. Pending histological confirmation, twicedaily topical 0.1% tacrolimus ointment was commenced. On histological examination of an incisional biopsy taken from the left pre-auricular area, a prominent perifollicular inflammatory cell infiltrate was seen, comprised mainly of lymphocytes, with mucinous degeneration of the follicular epithelium (Fig. 2a,b). There was no significant lymphocyte atypia, and the lymphoid immunoprofile was normal. Follicular destruction and granulomatous inflammation were absent. Fungal, bacterial and mycobacterial cultures were negative, as was direct immunofluorescence. Alcian blue staining confirmed the presence of intrafollicular mucin deposits (Fig 2c). Findings were consistent with a diagnosis of benign FM. On review 10 days later, the patient’s facial eruption had dramatically improved (Fig 1b). Full blood count, erythrocyte sedimentation rate, lactate dehydrogenase, human T cell lymphotropic virus (HTLV)1 and HTLV2 serology, and lymphocyte immunophenotyping were all normal or negative. T-cell gene rearrangement studies confirmed a polyclonal population, and a final diagnosis of primary idiopathic FM was made. Treatment with topical 0.1% tacrolimus ointment was tapered over the following 4 weeks, and remission has been maintained for > 1 year. A single case of recalcitrant FM successfully treated with 1% pimecrolimus cream has previously been reported. The response to tacrolimus ointment in our case was unusually rapid, and spontaneous resolution of lesions cannot be excluded. The risk of activating what may be considered a premalignant condition remains a concern, and further studies evaluating the efficacy, safety and tolerability of topical tacrolimus ointment in FM are required before it can be recommended.

Bosentan and Extracorporeal Photochemotherapy in Eosinophilic Fasciitis Synergistic Action or Fortuitous Coincidence

Sir, Eosinophilic fasciitis (EF) is a rare connective-tissue disorder clinically characterized by symmetrical and painful swelling associated to progressive induration and thickening of both skin and soft tissues, especially on the extremities. In contrast to other connective-tissue diseases, Raynaud’s phenomenon, sclerodactily, and abnormal nailfold capillaroscope readings are usually absent as antinuclear antibody and extractable nuclear antigen. Furthermore, peripheral eosinophilia, hypergammaglobulinemia, and a high erythrocyte sedimentation rate can be found. Despite the absence of a consensus on EF diagnostic criteria, the association of both clinical and histological characteristics (ie, fascia thickening intermingled with an eosinophilic infiltrate) leads to the diagnosis. EF therapy has not yet been standardized. However, besides systemic steroids, currently considered as the elective treatment, immunosuppressive drugs (such as cyclosporine and methotrexate) are successfully administered to nonresponder patients. Moreover, both PUVA (psoralen combined with ultraviolet A) and extracorporeal photopheresis (ECP) have been reported in recalcitrant cases. Herein, we describe a EF case treated with ECP and bosentan. A 50-year-old man presented with 1-year history of eosinophilia, malaise, arthromyalgia, and erythematous, edematous plaques (Figure 1) on the lower limbs. On the same areas, he complained of stiffness and swelling, as well. At blood examination, a slight eosinophilia 0.71 10/mm (9.8%) and lymphocyte percentage decrease (11.3%) were observed. In addition, positivity for antinuclear antibodies (1:1280) was found. To rule out hematological disorders, bone marrow biopsy and chest and abdomen computed tomography scan were performed with no sign of an internal disease. A biopsy on the leg revealed normal epidermis, edema in the medium and deep dermis, and muscular fascia thickened with an infiltrate of plasma cells (Figure 2). Despite the absence of the muscular component in the biopsy, the clinical–pathological correlation suggested EF diagnosis. Moreover, nail-fold capillaroscope readings were negative. Oral methylprednisolone (0.5 mg/kg/d) was administered in association with PUVA therapy (4 times a week). Two months afterward, 2 small (<0.5 cm) nummular and painful ulcers were observed on the left leg and hydrocolloid plaques were applied. Four months after the start of therapy, no improvement was observed, so PUVA therapy and oral steroids were stopped. ECP was started, scheduled in 1 cycle (on 2 consecutive days at 2-week intervals for 3 months, thereafter scheduled every 4 weeks for 10 months). The disease improved slightly after 4 cycles. However, the 2 ulcers did not improve. Bosentan therapy was administered (125 mg/d, increased 1 month later to 250 mg/d). Three months afterward, the stiffness had subsided, the plaques turned brownish, and ulcers appeared smaller. Twelve months from the start of both ECP and bosentan treatment, neither EF nor 529651 IJLXXX10.1177/1534734614529651The International Journal of Lower Extremity Wounds research-article2014

Interdigital Psoriasis of the Feet (Psoriasis Alba): Not a Distinct Form of Psoriasis

Background: Interdigital psoriasis (IP) of the feet is often missed and is commonly mistaken for interdigital fungal infection. Objective: To assess the characteristics and the clinical presentation of IP, in order to better understand if IP should be considered a distinct form of psoriasis or not. Methods: We performed a 1-year observational study on 164 psoriatic patients, affected by moderate to severe cutaneous psoriasis and undergoing systemic therapy, examining each patient between the digits of both feet. In every suspected case of IP, differential diagnosis with interdigital fungal infection was excluded by direct microscopic examination of skin scrapings, by culture and by skin biopsy. Results: We suspected IP in 7 of the 164 patients. IP was confirmed in 6 patients and in the other one a diagnosis of tinea pedis was made. Conclusion: IP proved to be not rare or atypical since IP localized between the toes usually presents as characteristic whitish and sodden plaques or patches. Such a diagnosis should be considered in all patients presenting characteristic lesions especially if these have a negative fungal culture, are resistant to antimycotic treatment and involve patients with a history of psoriasis.

Elephantasis Nostras Verrucosa and Psoriasis: Only a Coincidence?

Sir, A 60-year-old man came to our observation because of hyperkeratotic plaques on the lower limbs, of 8 weeks duration. On physical examination, his legs showed indurated, cobblestone-like, grayish lesions, pink verrucous projections, and nonpitting edema (Figure 1). Kaposi–Stemmer sign was evident. Few psoriatic plaques were present on the trunk and the upper limbs. The patient was affected by morbid obesity (body mass index 41 kg/m). His medical history was relevant for heart failure, venous insufficiency, lymphedema, autoimmune hypothyroidism, and psoriasis. At the time of the first visit, he was receiving furosemide 25 mg/d, enalapril 20 mg/d, thyroxine 100 μg/d, citalopram 20 mg/d, acetyl salicylic acid 100 mg/d, omeprazole 20 mg/d, acitretin 10 mg/d, emollients, and calcipotriol/betamethasone dipropionate ointment. The patient referred a worsening of the peripheral edema and effort dyspnea in the past week. A chest X-ray was taken and revealed pulmonary congestion due to congestive heart failure. He was hospitalized and blood tests were all within normal limits, except for an elevated reactive C protein (45 mg/L). An arteriovenous color Doppler ultrasonography (model MyLab40, Esaote, Genova, Italy) using a 7.5 to 12 MHz linear array transducer confirmed mild to moderate venous insufficiency. Filarial elephantiasis was excluded on the basis of the anamnesis and a diagnosis of elephantiasis nostras verrucosa (ENV) was made. Intravenous furosemide treatment was started, followed after 72 hours by the oral administration of the drug, in association with the increase of oral acitretin to 50 mg/d (0.4 mg/kg/d). Daily cleaning of the legs with chlorhexidine 2% aqueous solution was done to prevent over-infections. The patient initially used compressive bandages and after 2 weeks began to carry graduated support stockings (compression index 23-32 mm Hg). At follow-up visit, 2 months later, the patient had lost 15 kg and the lesions had resolved almost completely (Figure 2). Elephantiasis nostras verrucosa is a rare condition considered an exaggerated form of secondary lymphedema that usually involves lower extremities, deformed by progressive fibrosis of the skin. The term “nostras” refers to elephantiasis involving individuals resident in areas where filariasis is not endemic. ENV is clinically characterized by hard, thick, warty, or papillomatous projections and cobblestone-like lesions, with nonpitting edema. ENV mainly affects the lower limbs, even though it has been reported in external genitals, abdomen, buttocks, upper limbs, and face. The diagnosis of ENV is based on clinical history and physical examination. Skin biopsy, computed tomography, magnetic resonance imaging, lymphangiography, and 486156 IJLXXX10.1177/1534734613486156The International Journal of Lower Extremity WoundsSavoia et al research-article2013

Keloidal basal cell carcinoma: should it be considered a distinct entity?

Keloidal basal cell carcinoma (KBCC) is an extremely rare and still debated variant of the commonest malignant neoplasm of the skin [1, 2]. We observed a neoplasm with the characteristics of KBCC and would like to share our opinion, reporting a case that could better explain this entity. A 59-year-old man was referred to us because of a nodule in the right preauricular region (Figure 1). The patient had a history of previously excised basal cell carcinomas (BCCs) on the trunk, but the pre-existing scars were not keloidal or hypertrophic. The lesion had started 18 months before as a progressively enlarging papule. On physical examination it appeared as a nodule associated with a whitish translucent and slightly depressed patch without clearly defined edges; palpation revealed a firm skin texture that extended irregularly beyond the visible changes. Dermoscopy of the patch revealed fine telangiectases and scar-like depigmentation, while the nodule had the appearance of a keloid, even though the patient strongly denied antecedent traumas in this area. The lesion was excised with wide surgical margins. The histological examination showed a neoplasm formed by a BCC and an adjacent keloid, while higher magnification showed aggregations of basaloid cells between thick collagen bundles in haphazard array (Figure 2). Morphological aspects (e. g. absence of follicular cysts, clefts between neoplastic aggregations and stroma, basaloid palisading cells, scarce and edematous stroma, continuity with epidermis, necrosis) permitted to exclude neoplasms such as trichoblastoma [3], while other benign adnexal neoplasms were ruled out with immunohistochemistry. Staining with BCL-2 and BerEP4 confirmed the presence of a BCC. A final diagnosis of KBCC was made. In 1996 Requena et al. described KBCC as a new clinicopathological variant of BCC, reporting 2 cases of BCC with unique histological features [1]; after that only 3 other cases concerning this neoplasm were described [4–6] until 2009, when Jones et al. reported a 1.6 % frequency of keloidal collagen in BCC, suggesting that KBCC should not be considered a distinctive clinicopathological entity [2]. KBCC is not very well known among dermatologists. Whether or not it is a distinct entity remains debatable. The Clinical Letter following arguments have been advanced to support it as a separate entity:  Low frequency of KBCC and the unusual clinical presentation: only 5 cases have been reported to date and in none of them was a clinical diagnosis of BCC possible [1, 4–6].  Lack of described dermoscopic features [1, 2, 4–6].  A peculiar histology; in particular KBCC differs histologically from morpheaform BCC (mBCC), since the mBCC is not associated with well-circumscribed, keloidal collagen bundles, but show narrow strands or cords of BCC, which are embedded in an ill-defined, dense fibrous stroma [4].

Chronische Graft‐versus‐Host‐Erkrankung am männlichen Genitale: eine unterschätzte Manifestation

wir berichten über den Fall eines 24-jährigen Mannes, der sich mit asymptomatischen lichenoiden Läsionen am Penis vorstellte. Diese umfassten den proximalen Teil der Eichel und erstreckten sich bis zum koronalen Sulcus. Wegen eines hypoplastischen myelodysplastischen Syndroms (JAK2-Mutation) mit Monosomie des Chromosoms 7 in der Vorgeschichte hatte sich der Patient 16 Monate zuvor einer allogenen hämatopoetischen Stammzelltransplantation (HSZT) unterzogen. Eine Woche nach der Transplantation hatte sich eine milde kutane Graft-versus-Host-Reaktion (GVHD) entwickelt (akute kutane GVHD: Stadium 3, Grad II). Fünf Monate später war eine Lichen-ruber-planus-artige Ulzeration in der Mundhöhle aufgetreten (Abbildung 1 a). Eine Biopsie der Läsion in der Mundhöhle bestätigte die Clinical Letter Diagnose der lichenoiden chronischen GVHD (cGVHD). Seit der Transplantation hatte der Patient regelmäßig Ciclosporin, Prednison, Foscarnet, Misoprostol, Trimethoprim-Sulfamethoxazol, Fluconazol und Pantoprazol eingenommen. Die Behandlung der oben genannten Läsion in der Mundhöhle umfasste Clobetasol-Creme (einmal täglich), 0,1%ige Tacrolimus-Salbe (dermale Anwendung) und Schmalband-UVB-Therapie, was zur Stabilisierung der Erkrankung führte. Nach sechs Monaten war die Läsion in der Mundhöhle unter Hinterlassung einer Hyperpigmentierung abgeheilt. Sechzehn Monate nach der Transplantation entwickelten sich beim Patienten lichenoide Läsionen am Penis (Abbildung 1 b, c). Eine Diagnostik auf sexuelle übertragbare und Autoimmunerkrankungen verlief negativ. Die Biopsie einer Läsion zeigte eine Parakeratose, eine kompakte Orthokeratose, eine fokale Hypergranulose mit einigen apoptotischen Keratinozyten sowie eine fokale vakuoläre Degeneration entlang der dermo-epidermalen Junktionszone und ein mononukleäres Infi ltrat (Lymphozyten und Plasmazellen) in der oberen Dermis direkt unterhalb der Epidermis. Das Vorliegen der Parakeratose, der fokalen Hypergranulose und dem dichten lichenoiden Infi ltrat ließ auf eine cGvHD schließen (Abbildung 2 a, b). Ergänzend zur immunsuppressiven Therapie wurden topische Kortikosteroide (Clobetasol-Creme, 1 x täglich) verordnet. Nach zwei Wochen war die lichenoide Ulzeration am Penis abgeheilt und die topische Therapie wurde durch allmähliche Reduktion der Auftragshäufi gkeit ausgeschlichen. Seitdem trat kein Rezidiv auf. Die GVHD ist eine häufi ge Komplikation nach einer HSZT, die 30–70 % aller HSZT-Patienten betrifft [ 1 ] . Ihre

Chronic graft‐versus‐host disease of the male genitalia: an underrecognized manifestation

We report the case of a 24-year-old man who presented with asymptomatic, lichenoid penile lesions involving the proximal part of the glans and extending to the coronal sulcus. The patient had a history of hypoplastic myelodysplasia (JAK2 mutation) with monosomy of chromosome 7 for which he had undergone allogeneic hematopoietic stem cell transplantation (HSCT) 16 months earlier. One week after the transplant, he had developed mild cutaneous graft-versus-host disease (GVHD), (acute cutaneous GVHD: stage 3, grade II). Five months later, a lichen planus-like ulceration of the oral cavity had appeared (Figure 1 a). A biopsy of the oral lesion confi rmed the diagnosis of lichenoid chronic GVHD (cGVHD). Since the transplantation, the patient had been on cyclosporine, prednisone, foscarnet, misoprostol, trimethoprim sulfamethoxazole, fl uconazole, and pantoprazole. Treatment of the aforementioned oral lesion included clobetasol cream (one application daily), tacrolimus 0.1 % ointment (dermal application), and narrowband UVB therapy, which resulted in disease stabilization. After six months, the oral lesion had healed with hyperpigmentation. Sixteen months after the transplantation, the patient developed lichenoid lesions on the penis (Figure 1 b, c). Autoimmunity markers and markers for sexually transmitted infections were negative. A biopsy of the lesion revealed parakeratosis, compact orthokeratosis, focal hypergranulosis with few apoptotic keratinocytes, focal vacuolar degeneration along the dermoepidermal junction, and a mononuclear infi ltrate (lymphocytes and plasma cells) in the upper dermis just below the epidermis. The presence of parakeratosis, focal hypergranulosis, and a dense lichenoid infi ltrate was suggestive of cGVHD (Figure 2 a, b). Topical corticosteroids (clobetasol cream, one application daily) were added to the immunosuppressive therapy. After two weeks, the lichenoid ulceration of the penis had disappeared and topical therapy was discontinued by gradually reducing the frequency of application. Since then, no relapse has occurred.