Neutrophilic dermatoses in two children with idiopathic neutropenia: association with granulocyte colony-stimulating factor (G-CSF) therapy.

Painful neutrophilic skin lesions were observed in two children receiving granulocyte colony-stimulating factor (G-CSF) for treatment of idiopathic neutropenia. A girl with cystic fibrosis and cyclic neutropenia developed an erythematous papular eruption without fever or neutrophilia 7 months after commencing therapy with G-CSF. A skin biopsy specimen revealed microscopic, sterile, neutrophilic abscesses. A boy with chronic neutropenia and recurrent inflammatory skin lesions developed multiple erythematous nodules following administration of G-CSF. A biopsy specimen showed neutrophilic panniculitis. We believe that these skin eruptions belong to a spectrum of neutrophilic dermatoses that can be induced or aggravated by G-CSF therapy.

Pruritus vulvae in prepubertal children.

BACKGROUND: Vulvar pruritus is a common complaint in females of all ages. However, little has been published on pruritus vulvae in children as a primary symptom. OBJECTIVE: Our purpose is to review the causes and treatments of vulvar pruritus in prepubertal girls and to retrospectively evaluate the causes and outcomes of the premenarchal children we studied. METHODS: The records of 44 premenarchal girls with vulvar pruritus were reviewed, and follow-up interviews were performed by telephone. RESULTS: Thirty-three patients (75%) had nonspecific pruritus. Lichen sclerosus, bacterial infections, yeast infection, and pinworm infestation were seen in a minority of patients. At follow-up, pruritus had cleared in 15 patients, been alleviated in 13, and remained the same in 4. CONCLUSION: Most of our patients had nonspecific pruritus, which was alleviated by or cleared with better hygiene and avoidance of irritants. In prepubertal girls, poor hygiene and irritants such as soap are major contributors to pruritus vulvae. All patients may benefit from following hygienic measures and irritant precautions in addition to specific therapy directed at underlying causes.

Typical halo nevi in childhood: is a biopsy necessary?

We sent questionnaires to pediatric dermatologists inquiring whether anyone had seen a child with halo nevi that developed into melanoma. None of the respondents reported having seen such a development. We conclude that typical halo nevi in children are usually benign and a skin biopsy is probably not necessary.

Lymphangioma circumscriptum treated with pulsed dye laser.

Lymphangioma circumscriptum is a lymphatic malformation that involves the skin and may extend to subcutaneous tissue and muscle. Treatment of these lesions is challenging. Surgical excision may be performed but recurrence is common. Herein we report a child with a symptomatic lymphangioma circumscriptum that was treated with pulsed dye laser with good results. For superficial lymphatic malformations containing blood, pulsed dye laser might be considered as a treatment option.

A child with spider bite and glomerulonephritis: a diagnostic challenge.

A previously healthy 7-year-old white boy presented to St. Louis Children's Hospital with a 1-day history of headache, malaise, temperature of 38.7 degrees C, and a progressively erythematous, tender calf with central dusky purpura. On the morning of admission, his mother noticed a 2-mm crust on the patient's right calf with a 3-cm x 3-cm area of surrounding erythema. No history of recent trauma or bite was obtained. He had suffered two episodes of nonbloody, nonbilious emesis during the last day. In addition, over the previous 12 h, he presented brown urine without dysuria. His mother and brother had suffered from gastroenteritis over the previous week without bloody diarrhea. On initial physical examination, there was a 6-cm x 11-cm macular tender purpuric plaque with a central punctum on the right inner calf, which was warm and tender to the touch, with erythematous streaking towards the popliteal fossa (Fig. 1). The inguinal area was also erythematous with tender lymphadenopathy and induration, but without fluctuance. Laboratory studies included an elevated white blood cell count of 20, 800/microL with 6% bands, 86% segs, and 7% lymphocytes, hemoglobin of 12.5 g/dL, hematocrit of 35.1%, and platelets of 282,000/microL. The prothrombin time/activated partial tissue thromboplastin was 10. 4/28.0 s (normal PT, 9.3-12.3 s; normal PTT, 21.3-33.7 s) and fibrinogen was 558 mg/dL (normal, 192-379 mg/dL). Urinalysis showed 1+ protein, 8-10 white blood cells, too numerous to count red blood cells, and no hemoglobinuria. His electrolytes, blood urea nitrogen (BUN), and creatine were normal. The urine culture was negative. Blood culture after 24 h showed one out of two bottles of coagulase negative Staphylococcus epidermidis. The patient's physical examination was highly suggestive of a brown recluse spider bite with surrounding purpura. Over the next 2 days, the surrounding rim of erythema expanded. The skin within the plaque cleared and peeled at the periphery. The coagulase negative staphylococci in the blood culture were considered to be a contaminant. Cefotaxime and oxacillin were given intravenously. His leg was elevated and cooled with ice packs. The patient's fever resolved within 24 h. The lesion became less erythematous and nontender with decreased warmth and lymphadenopathy. The child was discharged on Duricef for 10 days. Because the patient experienced hematuria rather than hemoglobinuria, nephritis was suggested. In this case, poststreptococcal glomerulonephritis was the most likely cause. His anti-streptolysin-O titer was elevated at 400 U (normal, <200 U) and C3 was 21.4 mg/dL (normal, 83-177 mg/dL). His urine lightened to yellow-brown in color. His blood pressure was normal. Renal ultrasound showed severe left hydronephrosis with cortical atrophy, probably secondary to chronic/congenital ureteropelvic junction obstruction. His right kidney was normal.

Surgical treatment of pseudosyndactyly of the hand in epidermolysis bullosa: histological analysis of an acellular allograft dermal matrix.

Recessive dystrophic epidermolysis bullosa is an inherited mechanobullous disorder of skin and mucous membranes. The most striking clinical characteristic of the disease is the formation of blisters following trivial trauma. Repeated cycles of blistering and scarring result in gradual encasement of the hand in an epidermal "cocoon." The authors treated an 11-year-old boy with recessive dystrophic epidermolysis bullosa who presented with hand contractures and interdigital pseudosyndactyly. Treatment included release of contractures and application of a biosynthetic dermal analog. This report is a histological analysis of the dermal matrix 1 year after initial placement of the allograft. Fibroblasts repopulating the dermal allograft had a normal synthetic phenotype and lacked the myofibroblastic features seen in the ungrafted control biopsy. Collagen and elastin in the repopulated dermal allograft had normal dermal orientation and maturity in contrast to the sparse, immature collagen and lack of elastin compared with the dermis of an ungrafted control region. Results of this histological study indicate that treatment of recessive dystrophic epidermolysis bullosa with an acellular human dermal allograft may restore some features of normal dermal architecture. Although the initial results are encouraging, longer follow-up is required before definitive conclusions can be made.

Contact immunotherapy with squaric acid dibutylester for the treatment of recalcitrant warts.

BACKGROUND: Contact immunotherapy has been shown to be effective for warts. Two previous studies on the use of squaric acid dibutylester (SADBE) for warts have reported widely divergent cure rates (10% and 60%). OBJECTIVE: Our purpose was to determine the efficacy of SADBE in the treatment of recalcitrant warts. METHODS: We treated 29 patients with SADBE for warts that were resistant to other therapies. The patient population had warts for a mean duration of 2.1 years. Patients were sensitized with 1% or 2% SADBE in acetone under occlusion, then treated with 0.5% to 5% SADBE applied to their warts every 2 to 4 weeks in the office. RESULTS: Clearing of all warts was seen in 20 of 29 patients (69%), improvement in 3 patients (10%), and no change in 6 patients (21%). For the cured patients, mean duration of treatment was 4.2 months (range, 1 to 12 months) and mean number of treatments was 5.7 (range, 2 to 15). Adverse effects included acute contact dermatitis with 6 patients experiencing blisters and one experiencing hypopigmentation. CONCLUSION: SADBE treatment is worth considering in patients with recalcitrant warts, especially in those who tolerate painful procedures poorly.

Depilation in a 6-month-Old with hypertrichosis: A case report.

Hypertrichosis in the pediatric age group can be troubling to both patients and parents. There is no well-established method for managing this problem in young children. We describe the successful use of a cream depilatory agent for removal of excess hair from the face and body of a 6-month-old girl. Excellent cosmetic results were obtained. The risks and benefits of the use of depilatory cream in young patients are analyzed. Other options for hair removal in children are also reviewed.

The major inherited disorders of cornification. New advances in pathogenesis.

This article provides a synopsis of the major (most common) inherited disorders of cornification. It also reviews the recent advances that have been made for each disorder and their practical applications.

Grouped papules in Hurler-Scheie syndrome.

In a patient with Hurler-Scheie syndrome, a type of mucopolysaccharidosis (I H/S), an initial presentation was grouped papules on the extensor surfaces on the upper portions of the arms and legs. Other physical findings included progressive flexion contractures and mild developmental delay. The patient had deficient alpha-L-induronidase activity, and electron microscopy showed large cytoplasmic vacuoles and lysosomes, consistent with Hurler-Scheie syndrome. Findings of grouped papules have not been previously reported in patients with this syndrome.