Treatment of facial molluscum contagiosum with trichloroacetic acid.

Molluscum contagiosum (MC) virus is a common cutaneous infection in the pediatric population, most commonly affecting school-aged children. Spontaneous clearing of lesions usually occurs over time; however, treatment is often sought due to cosmetic significance, pruritus, or concerns of transmission and autoinoculation. Chemical destruction with cantharidin, which is derived from blister beetle extract, is very safe and highly effective, making it the treatment of choice in the pediatric population. However, treatment of facial lesions or those in the diaper area are not recommended with this agent. Trichloroacetic acid is a safe and effective agent frequently utilized in dermatologic practice, most commonly in the treatment of verrucae. We have successfully used topical trichloroacetic acid to treat facial molluscum contagiousum and present the following technique for proper application.

Segmental hemangioma of infancy complicated by life-threatening arterial bleed.

Infantile hemangiomas (IHs) are the most common benign vascular tumors of childhood. IH of "segmental" morphology, are clusters of hemangiomas with a configuration involving a broad anatomic territory of skin. They are the least common of all types and generally larger than regular hemangiomas, morphologically characterized as plaque-like lesions. Head and neck segmental hemangiomas have a higher risk of causing life-threatening complications and of having associated structural anomalies, i.e., PHACES syndrome (Posterior fossa malformations, hemangiomas, arterial anomalies, coartation of the aorta and other cardiac defects, eye abnormalities and sternal clefting or supra abdominal raphe). We present a patient with a segmental IH over the right anterior neck complicated by ulceration and life threatening arterial bleeding. Although segmental hemangiomas of head and neck have high incidence of ulceration, fortunately life threatening bleeding events are rare with only 7 previously recorded cases. We recommend that large, neck IH be followed closely for evidence of ulceration and that MRI/MRA be performed to adequately assess their vascular supply. Direct extension of the ulceration into arterial vessels is a possibility and can lead to severe bleeding. Life-threatening bleeding is an unusual complication of IH and may represents a surgical emergency. In such cases we recommend a multidisciplinary approach to their treatment.

Macrocephaly-capillary malformation: a report of three cases and review of the literature.

Macrocephaly-Cutis Marmorata Telangiectatica Congenita (M-CMTC) is a rare syndrome that was first delineated as distinct from Cutis Marmorata Telangiectatica Congenita (CMTC) in 1997. Since that time, there have been over 75 cases reported in the literature, though few are in the dermatology literature. The syndrome is characterized by macrocephaly, neonatal hypotonia, developmental delay, segmental overgrowth, syndactyly, asymmetry, connective tissue defects, and vascular stains. We report three new patients seen at the University of Miami Genodermatoses Clinic with features of M-CMTC. We believe the skin findings in our patients and in the previously published cases of M-CMTC are more consistent with capillary malformations rather than true CMTC. Therefore, we agree with recent publications that this condition be renamed Macrocephaly-Capillary Malformation (M-CM). The differential diagnoses for patients with M-CMTC include Klippel Trenaunay Syndrome (KTS) and Proteus or Proteus-like syndromes. Given the significant prognostic and likely genetic differences among these conditions it is important to distinguish M-CMTC from these syndromes.

Severe combined immunodeficiency (SCID)-associated dyschromia with subsequent repigmentation: a report of two patients.

Severe combined immunodeficiency (SCID) often presents with mucosal infections, cutaneous infections and eczematous rashes. We present two patients with history of SCID diagnosed at an early age who experienced diffuse dyschromia associated with their bone marrow and stem cell transplants. Dyschromias may be caused by numerous factors including medications, genetics, environmental contacts, or as a sequela of underlying chronic disease. These case reports describe progressive repigmentation to original skin color after the occurrence of dyschromia in two patients with SCID.

Excision and grafting of palmoplantar keratoderma.

Palmoplantar keratodermas may present to the clinician with an extremely broad series of clinical findings. Management has also taken on a wide variety of medical and surgical modalities. The literature seems to provide evidence that optimum management consists of surgical excision with skin grafting. It is believed that this will eliminate all of the underlying tissue and associated skin appendages, which are believed to be the source of this abnormal skin entity. We present a case of a patient in which tangential excision with delayed split-thickness skin grafting was performed after initial application of an acellular dermal matrix (Integra). Unfortunately, there was nearly immediate recurrence of this disease, and we, therefore, suggest a more aggressive approach to the initial excision.

Patch testing: another steroid-sparing agent to consider in children.

The burden of children suffering from chronic severe dermatitis and especially from undiagnosed confounding allergic contact dermatitis can be overwhelming for not only the patients and their families but also for their dermatologists. We report our protocol for pediatric patch testing and three pediatric cases, with severe and recalcitrant dermatitis unresponsive to systemic therapies, in whom comprehensive patch testing played a key role in management. Comprehensive patch testing allowed for the culprit allergens to be detected and subsequently avoided. With strict compliance, we were able to cease the use of systemic immunosuppressive therapy. These cases illustrate the importance of patch testing in recalcitrant dermatitis within the pediatric population.

Addison's disease, diffuse skin, and mucosal hyperpigmenation with subtle "flu-like" symptoms--a report of two cases.

Addison's disease, or chronic adrenocortical insufficiency, is the overproduction of adrenocorticotropic hormone by the pituitary gland as a compensatory mechanism for decreased cortisol production by the adrenal glands. Classically, patients affected with Addison's disease develop weakness, anorexia, electrolyte imbalances: decreased sodium and chloride with increased serum potassium resulting in hypotension, and hyperpigmentation of the skin and mucous membranes. Herein this case report, we focus on the subtle findings of diffuse hyperpigmentation and intermittent but repetitive "flu-like" symptoms in two patients to correctly identify the diagnosis of Addison's disease effectively and efficiently.

A rare presentation of squamous cell carcinoma in a patient with PIBIDS-type trichothiodystrophy.

The clinical presentation of trichothiodystrophy type F includes photosensitivity, ichthyosis, brittle hair, intellectual impairment, decreased fertility, and short stature, often referred to as the PIBIDS syndrome. While many of these patients demonstrate features also found in xeroderma pigmentosum patients, including similar nucleotide excision repair gene defects and photosensitivity, PIBIDS patients rarely demonstrate cutaneous malignancies. This case report demonstrates the rare presentation of squamous cell carcinoma developing in a PIBIDS patient.

Costello syndrome with severe nodulocystic acne: unexpected significant improvement of acanthosis nigricans after oral isotretinoin treatment.

We report the case of 17-year-old female diagnosed with Costello syndrome. Genetic testing provided a proof with G12S mutation in the HRAS gene since 3 years of age with a presentation of severe nodulocystic acne on her face. After 2 months of oral isotretinoin treatment, improvement in her acne was observed. Interestingly, an unexpected significant improvement of acanthosis nigricans on her neck and dorsum of her hands was found as well. We present this case as a successful treatment option by using oral isotretinoin for the treatment of acanthosis nigricans in Costello syndrome patients.

Propranolol vs corticosteroids for infantile hemangiomas: a multicenter retrospective analysis.

OBJECTIVE: To determine whether propranolol therapy is safe and effective and superior to oral corticosteroids for treating infantile hemangiomas (IHs). DESIGN: Multicenter retrospective chart review. SETTING: University of Miami and Miami Children's Hospital, Miami, Florida. Patients  The study included 110 patients with IHs. MAIN OUTCOME MEASURES: The percentage of clearance was quantified by documented serial global photography and clinical examinations (length, height, and width) to segregate patients into 2 groups: patients who had clearance of 75% or more and patients who had less than 75% clearance. RESULTS: The mean duration of treatment was 7.9 months for propranolol and 5.2 months for oral corticosteroids. Fifty-six of 68 patients (82%) who were receiving propranolol achieved clearance of 75% or more compared with 12 of 42 patients (29%) who were receiving oral corticosteroids (P < .01). Adverse effects were minimal in the propranolol group: 1 patient had hypoglycemia and 2 patients had a nonspecifice skin eruption that was not associated with propranolol therapy. All 42 patients in the corticosteroid group had 1 or more adverse effects (P < .01). Relapse after discontinuation of propranolol therapy occurred in 2 of the 68 patients; however, both patients responded to propranolol therapy on reinitiation of treatment. Surgical referrals after treatment were required in 8 patients (12%) in the propranolol group and 12 patients (29%) in the oral corticosteroid group (P < .01). CONCLUSIONS: Propranolol therapy was more clinically effective and more cost-effective than oral corticosteroids in treating IHs. It also resulted in fewer surgical interventions and demonstrated better tolerance, with minimal adverse effects, compared with oral corticosteroids. Therefore, propranolol should be considered a first-line agent given its safety and efficacy in the treatment of IHs.

Prevention of chemotherapy-induced alopecia in rodent models.

Alopecia (hair loss) is experienced by thousands of cancer patients every year. Substantial-to-severe alopecia is induced by anthracyclines (e.g., adriamycin), taxanes (e.g., taxol), alkylating compounds (e.g., cyclophosphamide), and the topisomerase inhibitor etoposide, agents that are widely used in the treatment of leukemias and breast, lung, ovarian, and bladder cancers. Currently, no treatment appears to be generally effective in reliably preventing this secondary effect of chemotherapy. We observed in experiments using different rodent models that localized administration of heat or subcutaneous/intradermal injection of geldanamycin or 17-(allylamino)-17-demethoxygeldanamycin induced a stress protein response in hair follicles and effectively prevented alopecia from adriamycin, cyclophosphamide, taxol, and etoposide. Model tumor therapy experiments support the presumption that such localized hair-saving treatment does not negatively affect chemotherapy efficacy.

Generalized erythrodermic pemphigus foliaceus in a child and its successful response to rituximab treatment.

Pemphigus foliaceus is an autoimmune disease that clinically manifests with cutaneous blisters of the superficial skin. The nonendemic or sporadic form of this entity is rare in children and typically presents with a milder, more localized rash that usually follows a benign course of short duration. We describe an affected patient atypical in both her young age and the severity of skin findings. Our patient presented with a full body exfoliative erythroderma at 21 months of age. After an extensive work-up to determine the etiology of her exfoliative erythroderma, direct and indirect immunofluorescence studies confirmed the diagnosis of pemphigus foliaceus. Rituximab therapy was initiated based on the patient's refractory disease course to multiple immunosuppressive agents. Rituximab is a therapeutic monoclonal antibody targeting CD20, an integral membrane protein highly expressed on the surface of pre-B lymphocytes and activated mature B lymphocytes. The patient's skin exhibited marked clinical improvement after the start of rituximab infusions over 12 weeks. Her initial desmoglein 1 antibody level was greater than 1:1280, which decreased to 1:16 after seven rituximab treatments. She has had no skin flares since initiating treatment with rituximab therapy. Based on this clinical and serologic response, the use of rituximab may be helpful in the treatment of pediatric pemphigus foliaceus refractory to mainstays of therapy.

Pityriasis lichenoides and idiopathic thrombocytopenic purpura in a young girl.

Pityriasis lichenoides is an inflammatory skin disorder characterized by erythematous, desquamative papules and plaques. An acute form, pityriasis lichenoides et varioliformis acuta, and a chronic form, pityriasis lichenoides chronica, represent the two ends of the spectrum of this disorder. Most commonly seen in children and young adults, its etiology is unknown. We describe a young patient with concurrent pityriasis lichenoides and idiopathic thrombocytopenia purpura, a previously unreported association.