The overall prognosis of BPDCN is dismal The

The overall prognosis of BPDCN is dismal. The median survival is approximately 12–14 months, and the 5-year-survival rate is 0–6%. The initial clinical presentation of BPDCN may seem deceptively indolent and often responds to multiagent chemotherapy. However, relapse with rapid extracutaneous dissemination, with or without leukemia, occurs in the vast majority of patients with an average disease-free time of 9–11 months. Reports of efficacy of treatments in patients with BPDCN indicate that acute lymphoblastic leukemia/lymphoma-type therapy results in a better outcome than acute myeloid leukemia-type therapy, despite a closer relationship between pDCs and myeloid cells. Long lasting remissions or curable cases have been rarely reported in pediatric patients and younger patients who received aggressive acute leukemia type therapy and allogeneic stem cell transplantation. A multivariate analysis from Bekkenk et al also revealed that an age <40 years, high AMG 925 of deoxynucleotidyl transferase, presentation of only cutaneous lesions, and aggressive treatment using an acute leukemia protocol are associated with a more favorable prognosis. Our BPDCN case presented with early cutaneous manifestations, including bruise-like lesions, which were accompanied by unusual nasopharyngeal involvement. Although a complete response was observed after initial chemotherapy with regression of nasal and skin lesions, long-term follow-up is necessary due to the high relapse rate, rapid leukemic dissemination, and poor prognosis of the disease. Cutaneous lesions are often the first prominent presentation of BPDCN and show a great variability in their morphologic and phenotypic features. The diagnosis of BPDCN requires exhaustive examination by expert clinicians and histopathologists. Dermatologists should be aware of the clinical presentations of such hematopoietic neoplasms because the early diagnosis of the disease and appropriate treatment is important to improve the outcome.
Introduction Becker\'s nevus, also known as Becker\'s melanosis or pigmented hairy epidermal nevus, was first reported by Becker in 1949. It is characterized by a light or dark brown patch with a sharply outlined but irregular border and always shows hypertrichosis after puberty in male patients. Becker\'s nevus may sometimes be associated with developmental anomalies, including hypoplasia of the ipsilateral breast, other cutaneous or muscular abnormalities, and skeletal defects. This phenotype is now described as Becker\'s nevus syndrome. Here, we describe a patient with Becker\'s nevus syndrome with bilateral skin involvement as follows.
Case Report An 18-year-old man was admitted to our hospital presenting with brownish hairy macula on the right scapular, lateral back region (Figure 1A), prothorax, upper limb, thigh (Figure 1B), and rump (Figure 1C) together with the posterior left upper arm (Figure 1D). The lesion started when he was 3 years old, increasing proportionally in size with the growth of the child. Hypoplasia of the whole right upper limb and ipsilateral breast were also observed (Figure 1B). There was no family history of similar disorders. Physical examination revealed neither impaired sensation of skin lesions nor enlarged cubital nerve. Laboratory examination showed no abnormality in routine tests. Magnetic resonance imaging (MRI) of the right upper limb showed congenital dislocation of the capitulum radius. A skin biopsy specimen obtained from the patient\'s forearm revealed slight acanthosis, hyperpigmentation of the basal layer without melanocytes increasing in number, and a superficial and sparse perivascular lymphohistiocytic infiltrate in the dermis (Figure 2A and B). Based on the clinical and histopathologic characters, a diagnosis of Becker\'s nevus syndrome was formed. The patient was being followed without any special treatment.
Discussion Becker\'s nevus syndrome is included within the group of epidermal nevus syndromes, together with nevus sebaceous syndrome, nevus comedonicus syndrome, phakomatosis pigmentokeratotica, Proteus syndrome, and CHILD (congenital hemidysplasia with ichthyosiform erythroderma and limb defects) syndrome. Becker\'s nevus often presents as a unilateral, asymptomatic, and irregular tan-brown patch. The associated anomalies tend to be a regional correspondence to the nevus and are mostly ipsilateral. Various associated developmental abnormalities or structural defects have been described along with Becker\'s nevus, including ipsilateral breast hypoplasia, supernumerary nipples, short limb or other forms of limb asymmetry, scoliosis, hemivertebrae, cleft vertebrae, spina bifida occulta, pectus excavatum, and patchy hypoplasia, etc.