Improvement of Unilateral Breast Hypoplasia With Oral Spironolactone in a Patient With Becker Nevus Syndrome.

Becker nevus (BN) is a benign cutaneous smooth muscle hamartoma that presents with a hyperpigmented patch or plaque with or without hypertrichosis.1 BN may be associated with ipsilateral breast hypoplasia or other musculoskeletal abnormalities, an association which has been termed Becker nevus syndrome (BNS).

Cutaneous Small/Medium CD4+ Pleomorphic T-Cell Lymphoma-Like Nodule in a Patient With Erythema Chronicum Migrans.

CD4+ small/medium pleomorphic T-cell lymphoma is a relatively rare subtype of cutaneous lymphoproliferative disorder with an indolent clinical behavior. The place of this condition among lymphomas is debatable. The authors describe a rare case of the direct association of CD4 small/medium pleomorphic T-cell lymphoma-like solitary nodule with Borrelia burgdorferi infection in a 5-year-old boy, discuss the reactive nature of this condition, and emphasize the importance of clinicopathological correlation.

Treatment for Infantile Hemangiomas: Selection Criteria, Safety, and Outcomes Using Oral Propranolol During the Early Phase of Propranolol Use for Hemangiomas.

OBJECTIVE: Despite the increasing popularity of propranolol for treatment of infantile hemangioma (IH), there is need for further evidence of efficacy and safety. This study is a retrospective review of one institution's experience treating IH with propranolol using a standard protocol. METHODS: Between 2009 and 2014, patients with IH were evaluated for treatment with propranolol. Exclusion criteria included a history of hypoglycemia, respiratory disorders, and cardiovascular disorders. Propranolol, 2 mg/kg/d, was initiated during 48-hour inpatient stay. Weight and complications were monitored. Appearance was assessed by Visual Analog Cosmetic Scale (VACS) via serial photography. RESULTS: Twenty-three patients were treated with propranolol. Average age at initiation of therapy was 14.9 weeks. Twenty-two lesions were on the head and neck, and 1 was on the trunk. Average treatment duration was 54.3 weeks (range 24-148 wk). Treatment was confirmed to be complete in 23 patients at the time of review (91.3%). Two patients were lost to follow-up. Posttreatment color, size, and VACS improved significantly (P < 0.05). There was no significant difference between first and most recent weight. Two patients experienced hypoglycemia, 1 during a diarrheal illness and 1 during inpatient treatment initiation. CONCLUSION: The authors present a series of patients with IH safely treated with 2 mg/kg/d of propranolol. Using a strict protocol, few complications were observed. Patients achieved significant reduction in size and improvement of the overall appearance of IH.

Amplification of mutated NRAS leading to congenital melanoma in neurocutaneous melanocytosis.

The mechanisms behind malignant progression in patients with giant nevi are largely unknown. Here, we aim to describe novel genetic findings and explain possible mechanisms resulting in the most severe form of neurocutaneous melanocytosis. Detailed histological (biopsy and post-mortem) studies, tissue culture, and high-resolution cytogenetic analysis, including chromosome and array comparative genomic hybridization, Ion AmpliSeq Cancer Panel, and Sanger sequencing, were performed on tissues from a white male who succumbed at 17 months of age to congenital melanoma associated with a bathing-trunk nevus. We also used quantitative PCR to quantitatively assess the expression of NRAS among normal cells, including fibroblast and melanocytes, as well as melanoma cells from our patient. Full autopsy documented tumors in the brain, spinal cord, lung, liver, testis, bone marrow, and, retrospectively, in the placenta. Next-generation sequencing and chromosome microarray in our patient revealed novel findings, including duplication of a mutated NRAS gene, leading to an aggressive clinical course and disseminated disease. Quantitative PCR showed a five-fold increase in NRAS expression in the melanoma cell line when compared with normal melanocytes. Finally, three amino acid-changing germline variants were detected: homozygous TP53 p.P72R, heterozygous KIT p.M541L, and homozygous KDR (VEGFR2) p.Q472H. These genes are involved in malignancy and other potentially relevant pathways, such as mast cell and melanocytic signaling, as well as angiogenesis. These findings provide novel insights into the biology of congenital melanocytic proliferations, showing that amplification of mutated NRAS seems to represent a new genetic mechanism leading to melanoma in the context of neurocutaneous melanocytosis.

Phytophotodermatitis.

Phytophotodermatitis is a phototoxic cutaneous eruption due to skin exposure to furocourmarins combined with ultraviolet light. Bizzare linear patterns, ranging from erythema to bullae with residual hyperpigmentaion, is the clinical clue to this diagnosis. Avoidance of furocoumarins in direct sunlight can prevent recurrences.

Hair follicle nevus of the abdominal skin: an unusual extracephalic presentation.

Hair follicle nevus (HFN) is a rare hamartoma typically diagnosed on the face of infants, where it may clinically mimic an accessory tragus. We report a 6-month-old boy who presented with a congenital fleshy, bilobed papule in the midline of his upper abdomen that upon excision was classified as an HFN based on detailed histopathologic examination. Our report documents a previously undescribed extracephalic location of the HFN and therefore expands the spectrum of clinical presentations of this rare hamartoma.

Phototoxic dermatoses in pediatric BMT patients receiving voriconazole.

We investigated the incidence of phototoxic skin reactions in pediatric BMT recipients treated with voriconazole. Nine out of 40 patients (22.5%), all Caucasian, developed skin lesions in sun-exposed distributions. Dermatologic findings included sunburn-like erythema, pseudo-porphyria, linear papulovesicular lesions, severe erosive cheilitis, dermatoheliosis and lentigines. Patients were treated with sun avoidance, high-potency sunscreens, and topical steroids with significant improvement in all cases. Prolonged voriconazole use requires close monitoring for chronic skin toxicities. Long-term risks including the risk of skin cancer need to be investigated.

Bullous Sweet's syndrome after granulocyte colony-stimulating factor therapy in a child with congenital neutropenia.

We present the case of a 20-month-old boy with congenital neutropenia for which he was being treated with granulocyte colony-stimulating factor (G-CSF) who developed bullous Sweet's syndrome. Because of the challenging and extensive differential diagnosis of an acute bullous eruption in an immunocompromised child, we highlight the importance of a prompt and precise diagnosis before initiation of any systemic therapy in children with Sweet's syndrome.

A 7-week-old Nepali girl with a perianal ulcer: brief report.

Cytomegalovirus (CMV) can rarely present with skin findings. Cutaneous CMV is most often found in patients who are immunocompromised because of acquired immunodeficiency syndrome, lymphoma, or other conditions. We present a rare case of an immunocompetent 7-week-old girl with a perianal ulcer attributed to CMV.

Group A streptococcal panniculitis.

Infectious panniculitis caused by group A beta-hemolytic streptococcus is rare, especially in immunocompetent patients. Its clinical presentation is usually nonspecific, but skin biopsy may provide information on the source. We describe the case of a previously healthy 2-year-old girl who presented with fever, tachycardia, and diffuse erythematous skin nodule; biopsy revealed a deep lobular neutrophilic panniculitis with gram-positive bacteria.

Facial necrosis after endovascular Onyx-18 embolization for epistaxis.

BACKGROUND: Evolution in techniques and equipment has expanded the role, effectiveness, and safety of endovascular transarterial embolization for the treatment of severe epistaxis. Risks from this treatment approach include major ischemic complications. To date, there have been only a few reports of soft tissue necrosis following endovascular embolization for severe epistaxis; none involve the use of Onyx-18. CASE DESCRIPTION: We report the case of a 52-year-old woman who presented with epistaxis that was refractory to medical and surgical management, which lead to endovascular intervention and embolization with Onyx-18. The patient subsequently developed nasal ala and facial necrosis as a result of the procedure. CONCLUSION: We report the use of Onyx-18 for the endovascular embolization of a patient with severe epistaxis and subsequent complications. In cases of severe epistaxis that warrant intervention in the form of embolization, ischemic complications are rare; however, ischemic complications may be unavoidable and should factor into the discussion regarding procedural risks.