Nedzmidin Pelivani

Alopecia Areata Universalis Elicited during Treatment with Adalimumab

Adalimumab is a fully humanized recombinant anti-tumour-necrosis-factor (TNF-α) monoclonal antibody which has been approved for rheumatoid arthritis, active ankylosing spondylitis, psoriatic arthritis and Crohn’s disease. We report a case of alopecia areata (AA) universalis occurring 6 months after administration of adalimumab monotherapy in a patient with a long-standing history of psoriatic arthritis and psoriasis. The diagnosis was confirmed by a scalp biopsy which showed a peribulbar infiltrate of both CD4+ and CD8+ T cells, CD1a+ dendritic cells as well as CD68+ and CD163+ macrophages. In addition, immunofluorescence staining for TNF-α was found in the mononuclear cell infiltrate. This case suggests a complex role of TNF-α in the induction of AA.

Detection of linear IgE deposits in bullous pemphigoid and mucous membrane pemphigoid: a useful clue for diagnosis

Summary Background  Bullous pemphigoid (BP) is an autoimmune subepidermal blistering disease of the skin associated with IgG autoantibodies to BP180 and BP230, while mucous membrane pemphigoid (MMP) comprises a heterogeneous group of autoimmune blistering diseases characterized by a predominant mucous membrane involvement and scarring tendency associated with an autoantibody response to various autoantigens, including BP180. While the pathogenicity of IgG autoantibodies to BP180 has been demonstrated in BP, the role of IgE autoantibodies in mediating tissue damage in BP and MMP is unclear.

Amicrobial Pustulosis-Like Rash in a Patient with Crohn’s Disease under Anti-TNF-Alpha Blocker

Amicrobial pustulosis of the folds (APF) is a recently described entity characterized by relapsing pustular lesions predominantly involving the cutaneous flexures and scalp. This disease typically occurs in association with systemic lupus erythematosus and a variety of other autoimmune diseases. We here describe an APF-like pustular eruption predominantly affecting the scalp, face and trunk, occurring during long-term infliximab treatment for Crohn’s disease. Immunohistochemical staining of skin biopsy specimens for myxovirus resistance protein A, a marker for type 1 interferon-inducible proteins, showed increased staining in the epidermis and dermal mononuclear inflammatory infiltrate. Our observation further extends the spectrum of cutaneous adverse reactions potentially related to anti-tumor necrosis factor-α, the clinical context in which APF can occur as well as its clinical presentations.

Giant Cellulitis-like Sweet Syndrome, a New Variant of Neutrophilic Dermatosis

BACKGROUND Neutrophilic dermatoses comprise a wide spectrum of inflammatory diseases with overlapping features characterized histologically by the presence of an aseptic neutrophilic infiltrate in the epidermis, dermis, and/or hypodermis and are often associated with systemic inflammatory and neoplastic disorders. OBSERVATIONS We describe 3 patients with an unusual neutrophilic dermatosis characterized by relapsing episodes of fever, widespread infiltrated plaques with bullous appearance, and variable involvement of the arms, legs, abdomen, and/or trunk. Light microscopy studies showed marked edema of the papillary dermis with an inflammatory infiltrate consisting mainly of mature neutrophils. All 3 patients were morbidly obese, and workup revealed underlying cancer in 2 cases: myeloma and breast carcinoma. Management of the underlying disease resulted in long-term remission of the skin disease. CONCLUSIONS The clinicopathologic features in our 3 cases best correspond to a widespread giant cellulitis-like form of Sweet syndrome. Knowledge of this newly observed unusual variant of Sweet syndrome within the broad spectrum of neutrophilic diseases is important for its prompt and proper management.

Severe cutaneous allergic reactions following topical antifungal therapy

Antifungal creams had been used to treat clinically or mycologically diagnosed infections with dermatophytes or intertrigo. Seven to 21 days after initiation of antifungal therapy, the patients developed widespread eczema as well as erythematous, maculopapular, erythema multiformelike and blistering eruptions, that occurred in addition to intense eczematous reactions at application sites associated with peripheral blood eosinophilia, suggesting drug reactions (Fig. 1). After the eruptions had resolved, patch testing was performed with the European baseline, antimycotic agent, corticosteroid, preservative, and emollient series (Almirall Hermal, Reinbek, Germany) and culprit antifungal and corticosteroid creams used by the patients on Finn Chambers® on Scanpor® tape (SmartPractice, Phoenix, AZ, USA) applied for two days, with readings at D3 and D4. Five patients were sensitized to tixocortol pivalate or prednisolone. Two patients showed sensitization to both corticosteroids and clotrimazole, as well as to the culprit antifungal

Hairy Pinnae after Orchiectomy and Chemotherapy for Testicular Cancer: Acquired Localized Hypertrichosis of the Ears

Acquired localized hypertrichosis has rarely been reported. Here, we describe a patient with localized hypertrichosis of the pinnae that occurred 4 months after orchiectomy and chemotherapy for a testicular carcinoma. To our knowledge, this is the first case of an acquired hypertrichosis of the pinnae after cancer therapy. We propose that in our patient either hypogonadism or the hormonal imbalance caused by the cancer therapy led to the development of the hairy pinnae, perhaps alongside a genetic predisposition for hairy ears.

Trichilemmal Cyst Nevus with a Sebaceous Nevus Component

A 23-year-old man with a typical trichilemmal cyst nevus is reported. This recently described disorder is sufficiently characteristic to differentiate it from sebaceous nevus, nevus comedonicus, porokeratotic eccrine nevus, nevus corniculatus, follicular basaloid hamartoma, Munro’s nevus and Gardner’s syndrome.

A tattoo complicated by allergic contact dermatitis and panniculitis

disease (eight men, eight women, mean age 59.8 years, range 33.4–85.4) and 26 with Darier disease (13 men, 13 women, mean age 65.4, range 30.7–87.6). Eleven of 16 patients (68.8%) affected by Hailey–Hailey disease and 16 of 26 patients (61.5%) with Darier disease admitted to having longitudinal white streaks in one or more nails. The majority of them had white streaks at the time of the survey, whereas one patient with Hailey–Hailey (6.3%) and three with Darier disease (11.5%) stated that this nail sign had occurred only in the past. The observations were confirmed in eight patients based on self-made photos or clinical examination. Longitudinal leuconychia striata in Hailey–Hailey disease was first described by Burge in a study of 38 patients with a prevalence of 70%. Since then, no studies have been performed on this phenomenon, and to our knowledge, only two case reports have focused on it. In Darier disease, leuconychia striata was reported only in one study of 37 patients with a prevalence of 100%. It usually occured in association with other signs such as subungual hyperkeratosis, fragility, splintering, red longitudinal bands and triangular nicking of the distal nail plate. As our aim was solely to study the presence of leuconychia striata, we did not investigate other signs. The pathogenesis of the white stripes is currently not known. In conclusion, we performed the second study on longitudinal leuconychia striata in Hailey–Hailey and Darier disease. We found that nearly two thirds of these patients reported longitudinal white streaks on their finger nails. Our results confirm Burge’s findings but we found a significantly lower prevalence than Zaias and Ackerman.

Penile erosions as first manifestation of pemphigus vulgaris: A misleading presentation

© 2013 The Authors. doi: 10.2340/00015555-1388 Journal Compilation

Hautmanifestationen bei inneren Erkrankungen. Teil 1

Besonders Patienten mit Pruritus und fehlenden Hautveranderungen stellen eine Herausforderung dar. Hierbei mussen cholestatische Lebererkankungen, Niereninsuffizienz, Schilddrusenfunktionsstorungen und lymphoproliferative Erkrankungen ausgeschlossen werden. Einige charakteristische Hautveranderungen sind fakultative oder obligate Paraneoplasien. Sie konnen als erstes Symptom einer Krebserkrankung auftreten.