Blastic Plasmacytoid Dendritic Cell Neoplasm, a Very Rare Hematological Malignancy With Initial Cutaneous Involvement: A Case Report.

Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a very rare and aggressive hematologic malignancy, arising from plasmacytoid dendritic cells (pDCs). BPDCN frequently has, at least initially, exclusively cutaneous presentation. We present a 45-year-old woman with a 3-month history of rapidly evolving violaceous patches, infiltrated plaques, and bruise-like tumefactions, disseminated on her face and upper trunk.

Serum Zinc Concentration in Patients with Alopecia Areata.

Alopecia areata is an autoimmune non-scarring disease in which the exact mechanism that induces loss of immune privilege is unknown. Zinc is important for DNA stability and repair mechanisms that are essential in maintaining normal hair growth. Zinc deficiency has been investigated as an important factor in many autoimmune diseases, and may have a possible role in the aetiopathogenesis of alopecia areata.

Mastocytosis in children: a single-center long-term follow-up study.

Background: Mastocytosis is a heterogeneous group of rare disorders characterized by the accumulation of clonal mast cells in organs such as the skin and bone marrow. The diagnosis of cutaneous mastocytosis (CM) is based on clinical findings, positive Darier's sign, and histopathology, if necessary.

Methods: Medical records of 86 children with CM diagnosed during a 35-year long period were reviewed.

Level and avidity of antineutrophil cytoplasmic antibodies specific to lactoferrin are useful biomarkers in systemic lupus erythematosus.

Objectives: To evaluate specificity, level, and avidity of antineutrophil cytoplasmic antibodies (ANCA) in systemic lupus erythematosus (SLE). There are no studies of ANCA avidity in SLE.

Methods: Level (ELISA) and avidity (ELISA) of myeloperoxidase (MPO-), proteinase 3 (PR3-), lactoferrin (LF-), cathepsin G, elastase (EL-), and bactericidal/permeability increasing protein (BPI)-ANCA in 142 SLE patients were studied.

Annular lichenoid dermatitis of youth-Recurrent case of rare skin disease treated with cyclosporine.

Annular lichenoid dermatitis of youth (ALDY), first described in 2003, represents an uncommon entity whose etiopathogenesis is still debated. Futhermore, the optimal treatment for ALDY is yet to be established. We report a 9-year-old girl who presented with annular and oval erythematous lesions mostly on her trunk, with several lesions on the neck, groin, flanks, and upper extremities.

Combined intravenous pulse and topical corticosteroid therapy for severe alopecia areata in children: Comparison of two regimens.

There is no universally accepted treatment for severe pediatric alopecia areata (AA). This prospective study comprised 73 patients (aged 1-18 years) with severe AA (>30% of scalp surface area): 37 received 1-day intravenous dexamethasone pulses (1-DP) and 36 received 3-day pulses (3-DP), monthly, for 6-12 months. Also, all patients applied topical clobetasol propionate under plastic wrap occlusion.

Penicillin-induced Cutaneous Necrotizing Eosinophilic Vasculitis with Cryofibrinogenemia.

Cutaneous necrotizing eosinophilic vasculitis (CNEV) is a rare type of vasculitis. Eosinophilic vasculitis is a necrotizing vasculitis with eosinophilic vascular infiltration, in which eosinophils mediate vascular damage in the disease process. We present a case of an 18-year-old girl who developed palpable purpura and hemorrhagic bullae over the lower extremities associated with itching, 7 days after the commencement of penicillin therapy.

Pregnancy-Induced Sweet's Syndrome Treated with Infliximab.

Objective: To present a case of relapsing and resistant Sweet's syndrome that developed during pregnancy together with an onset of Crohn's disease, showing complete resolution with the use of infliximab.

Clinical Presentation And Intervention: A 30-year-old pregnant woman presented with fever, skin lesions, and diarrhea. Skin biopsy confirmed neutrophilic dermatosis and she was diagnosed with Crohn's disease after endoscopy.

Orofacial granulomatosis in a 12-year-old girl successfully treated with intravenous pulse corticosteroid therapy and chloroquine.

Orofacial granulomatosis, a rare disease in childhood, is characterized by orofacial swelling in the absence of systemic disease. We report the case of a 12-year-old girl with asymptomatic erythematous infiltration of her upper lip, cheeks, and chin that had persisted for more than 2 years; biopsy confirmed granuloma formation. Because a large area was affected, intralesional corticosteroids were inappropriate and six cycles of 3-day intravenous pulse corticosteroid therapy (dexamethasone 1.

Combined oral pulse and topical corticosteroid therapy for severe alopecia areata in children: a long-term follow-up study.

There are no widely accepted therapy protocols for severe alopecia areata (AA). We treated 65 children/adolescents with AA affecting >30% of scalp. Fourty-three percent of patients had AA plurifocalis (AAP).

Importance of low serum DNase I activity and polyspecific anti-neutrophil cytoplasmic antibodies in propylthiouracil-induced lupus-like syndrome.

Objective: To study the role of deoxyribonuclease (DNase) I activity and ANCA in propylthiouracil (PTU)-induced lupus-like syndrome (LLS).

Methods: We compared 36 SLE patients with 17 PTU-induced LLS patients diagnosed from 2008 to 2014. We studied ANCA profile (MPO, PR3, lactoferrin, CTG, elastase, bactericidal/permeability-increasing protein), anti-dsDNA, anti-ENA, anti-nucleosome, anti-histone, anti-C1q, anti-aCL, complement components, cryoglobulins and serum DNase I activity.

Stevens-Johnson syndrome and toxic epidermal necrolysis: a 20-year single-center experience.

Background: Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are life-threatening diseases that are most frequently caused by drugs.

Objectives: The purpose of this study was to summarize 20 years of experience with SJS and TEN in the largest dermatology clinic in Serbia.

Methods: The study included 38 patients treated during the period 1993-2012.

Granulomatosis with polyangiitis (Wegener's granulomatosis) in children: report of three cases with cutaneous manifestations and literature review.

Granulomatosis with polyangiitis (GPA), also known as Wegener's granulomatosis, is a rare disease in childhood. Of 39 GPA patients that we diagnosed during a 20-year period, only 3 (7.7%) were younger than 18 years.

Acrokeratosis paraneoplastica Bazex syndrome associated with esophageal squamocellular carcinoma.

Background: Acrokeratosis paraneoplastica Bazex (APB) is a very rare disease in the group of obligate paraneoplastic dermatoses, associated mostly with squamous cell carcinoma of the upper aerodigestive tract and metastatic cervical lymphadenopathy. The disease is characterized by violaceous erythemosquamous changes on the acral regions. This entity was first reported by Bazex in 1965.

Pagetoid reticulosis of Woringer-Kolopp.

Pagetoid reticulosis of Woringer-Kolopp is a rare form of cutaneous T-cell lymphoma that primarily affects middle-aged males. It is characterized by the presence of one or several scaly patches and plaques with an acral distribution. We present a case of a 58-year-old woman, otherwise healthy, with a 5-month history of asymptomatic, hyperkeratotic plaques on the hands and feet.