Early morphea mimicking acquired port-wine stain.

We report the case of a 2.5-year-old girl with linear morphea initially diagnosed as an acquired port-wine stain (PWS). She underwent three treatments to the right face using the pulsed dye laser (PDL) before sclerotic changes were observed and the correct diagnosis was confirmed with histopathology. Treatment using the PDL reduced the skin erythema but did not prevent subsequent sclerosis. The sclerosis became most prominent superior to the patient's right ear in an area not treated using the laser. A review of the English-language medical literature identified no cases of morphea triggered using a PDL, but there were several reports of early morphea misdiagnosed as an acquired PWS. Briefly, we review those cases, as well as morphea subtypes, and comment on how the pathophysiology of morphea may lend itself to an early underrecognized inflammatory presentation, delaying diagnosis.

Crusted scabies misdiagnosed as erythrodermic psoriasis in a 3-year-old girl with down syndrome.

Scabies is a highly contagious infestation with the Sarcoptes scabiei var hominis mite. The variety of clinical presentations make timely, accurate diagnosis problematic. We report the case of a 3-year-old girl with Down syndrome and crusted scabies initially misdiagnosed as erythrodermic psoriasis.

Congenital erosive and vesicular dermatosis with reticulated supple scarring: unifying clinical features.

BACKGROUND: Congenital erosive and vesicular dermatosis (CEVD) healing with reticulated supple scarring, a condition usually observed in premature neonates, presents at birth with vesicles and erosions. Lesions typically heal within a few months, leaving behind scarring with a distinctive supple and reticulated texture. OBJECTIVES: We sought to merge existing literature with new cases to further define CEVD. METHODS: We analyzed 19 previous reports of CEVD and added 9 additional patients; we identified unifying characteristics of this cohort. RESULTS: In 28 total cases, notable features included: preterm birth (79%), nail abnormalities (46%), hyperthermia/hypohidrosis (46%), a history of maternal chorioamnionitis (43%), alopecia (43%), neurodevelopmental and ophthalmologic abnormalities (36% each), tongue atrophy (29%), or a combination of these. Patients with CEVD may be prone to postnatal herpetic superinfections. Previously unreported findings included: erosive lichen planus, digital tip gangrene, and hydronephrosis. LIMITATIONS: The small patient sampling makes it difficult to define diagnostic criteria. As certain findings are associated with prematurity, it is unclear to what extent these features result from CEVD, premature birth, or another intrauterine pathology. CONCLUSIONS: Although rare, CEVD should be considered in the differential diagnosis of neonatal vesicles/erosions in the context of a negative infectious workup. This review strengthens the spectrum of CEVD features, thus facilitating its recognition by clinicians.

Eccrine squamous metaplasia and periadnexal granulomas: new cutaneous histopathologic findings in cardiofaciocutaneous syndrome.

Cardiofaciocutaneous syndrome is a rare genetic disorder characterized by dysmorphic facial features and neurologic, cardiac, ophthalmologic, and dermatologic findings. Previously reported skin and hair findings in cardiofaciocutaneous syndrome include sparse, slow-growing curly hair, atopic dermatitis, ichthyosis, follicular hyperkeratosis, and keratosis pilaris. We report the case of a 4-year-old boy who has cardiofaciocutaneous syndrome with previously unreported histopathologic findings of eccrine squamous metaplasia and periadnexal granuloma.

Chronic recurrent multifocal osteomyelitis (CRMO) and synovitis, acne, pustulosis, hyperostosis, and osteitis (SAPHO) syndrome with associated neutrophilic dermatoses: a report of seven cases and review of the literature.

A growing body of literature has identified the association between neutrophilic dermatoses and multifocal, aseptic bone lesions in children, termed chronic recurrent multifocal osteomyelitis (CRMO). Classically, patients present with swelling, pain, and impaired mobility of the affected area, with skin lesions developing concurrently or in the future. Bone biopsy reveals inflammatory changes consistent with infectious osteomyelitis, but cultures and histologic staining invariably fail to identify an infectious source. Patients are refractory to antibiotic therapy, but dramatically respond to systemic steroids and may need to be maintained on low-dose steroids to prevent relapse. Numerous authors have suggested that CRMO and synovitis, acne, pustulosis, hyperostosis, osteitis (SAPHO) syndrome lie along the same clinical spectrum. In fact some believe that CRMO is the pediatric presentation of SAPHO. The two syndromes share numerous characteristics, including osteitis, a unifocal or multifocal presentation, hyperostosis, and pustulosis, which all occur in a generally healthy individual. Our seven patients, five of whom were diagnosed with CRMO, and two of whom were diagnosed with SAPHO syndrome further strengthen the idea that CRMO and SAPHO syndrome do indeed lie along the same clinical spectrum. In addition, we include two rare cases of pediatric Sweet's syndrome with evidence of pathergy.

Neonatal sporotrichosis.

Sporotrichosis is caused by the dimorphic fungus, Sporothrix schenckii. Classically, infection occurs after implantation of the organism into the skin by abrasion of a puncture wound by contaminated thorns, hay, or sphagnum moss. Cats are also a commonly recognized source of sporotrichosis. In children, fixed cutaneous lesions are more common than lymphocutaneous lesions, and the face is a frequent location for infection. We present a neonate with a fixed cutaneous facial lesion and both pre-auricular and cervical lymphadenopathy who developed signs of infection at 3 weeks of age. This patient is currently the youngest reported case of sporotrichosis in the literature. The patient's family denied any trauma and denied contact with flowers, gardens, and cats; thus, the source of infection remains unknown.

Overview: the state of head lice management and control.

Head lice infestations affect millions of people in the United States. Children become infested more often than adults and account for the largest percentage of infestations. Head lice have not been shown to transfer disease, and they are not associated with serious morbidity. The most common effect of lice infestation is pruritus of the scalp with occasional cutaneous infection caused by scratching. Nevertheless, many schools have "no-nit" policies, which require the dismissal of children from school if nits or lice are found. These policies are ineffective in preventing infestations and result in many missed days of school. Lice infestations are most effectively managed with pediculicides. Pyrethroids are the mainstay of over-the-counter products. Prescription pediculicides include OVIDE (malathion) Lotion, 0.5% and lindane (formerly marketed as Kwell). Resistance to pyrethroids due to misuse and overuse has been documented. Lindane resistance also has been reported, and serious safety issues about lindane have been raised by the Food and Drug Administration. Lindane labeling now includes warnings and several restrictions in its use. Malathion is not associated with major systemic safety issues or the development of resistance within the United States. A contributor to pediculicide resistance is misdiagnosis of lice infestations. Survey data reveal frequent misdiagnosis of infestations in children who do not have live lice. Physicians generally are more likely to misdiagnose infestations than nonhealthcare providers. Misdiagnosis contributes to resistance by causing overuse, and consequently overexposure, of pediculicides. These agents should be used only if live lice or viable nits are discovered. Head lice infestations generally do not contribute to health risks for individuals or the public. The most serious consequence is the social cost of missed school days and the associated cost of lost productivity and wages of parents who must care for children sent home from school. Better diagnosis, more appropriate use of pediculicides, and elimination of no-nit policies will improve the overall management of head lice infestations.

Economic considerations associated with Pediculus humanus capitis infestation.

An estimated 6 to 12 million head lice infestations occur in the United States annually, with children ages 3 to 12 most likely to be affected. There are significant direct costs associated with treatment and indirect costs due to lost time from school. Anecdotal reports suggest that direct costs of treatment are in the hundreds of millions of dollars annually. Indirect costs are also substantial but more difficult to quantify. Examples of indirect costs include missed days from schools that use a "no nit" policy, lost wages for parents who must stay home with children, and costs of daycare for parents who cannot miss work. Contributors to the expense of treating head lice include misdiagnosis, and, consequently unneeded treatment; treatment failure due to misuse of pediculicides or other agents; and developing resistance, particularly to over-the-counter pyrethroid agents. An overview of direct and indirect costs of infestation are included in this review, along with a discussion of factors that lead to misuse and overuse of pediculicides. More accurate diagnosis of head lice infestation may provide the most effective means of controlling the costs of care and ensuring proper use of pediculicides.

Paraneoplastic pemphigus with bronchiolitis obliterans in a child.

Paraneoplastic pemphigus (PNP) is a rare blistering autoimmune disease associated with an underlying neoplasm, mucous membrane erosions, and occasionally bronchiolitis obliterans. Most cases have been reported in adults and the number of childhood cases in the current literature is limited. We describe a young patient with PNP who was initially misdiagnosed as having recurrent Stevens-Johnson syndrome. This patient had an underlying inflammatory myofibroblastic tumor and subsequently developed fatal progressive bronchiolitis obliterans.

Bullous phytophotodermatitis associated with high natural concentrations of furanocoumarins in limes.

BACKGROUND: Phytophotodermatitis is a phototoxic reaction, occurring in skin exposed to sunlight after contact with plants containing furanocoumarins. Typical reactions are mild, showing erythema with post-inflammatory hyperpigmentation. A 6-year-old boy presented with marked, symmetric, painful erythema and edema of both hands that rapidly developed into dramatic bullae covering the entire dorsum of the hands. The history revealed that the hands had been bathed in lime juice for a prolonged period in the preparation of limeade. OBJECTIVE: This report documents an unusual bullous presentation of phytophotodermatitis resulting from contact with furanocoumarins in local limes. This study was conducted to identify and measure the inciting substances from the rind and pulp of the limes. METHODS: Psoralen, xanthotoxin, bergapten, and isopimpinellin content were measured by gas chromatography and high-pressure liquid chromatography RESULTS: The rind contained 6- to 182-fold concentrations of all furanocoumarins measured when compared with pulp. Bergapten was the most abundant substance in the rind. CONCLUSION: Hydration of the skin during the preparation of limeade combined with increased levels of bergapten in local limes to produce a dramatic bullous reaction. We encourage clinicians to consider the possibility of phytophotodermatitis in severe bullous skin reactions.

Atypical pityriasis rubra pilaris associated with arthropathy and osteoporosis: a case report with 15-year follow-up.

Pityriasis rubra pilaris (PRP) is an idiopathic papulosquamous disease that clinically presents with palmoplantar keratoderma and follicular hyperkeratotic papules that coalesce into scaly erythematous plaques. We report a unique case of atypical PRP beginning at 1 year of age with associated severe arthropathy and osteoporosis. We further discuss the clinical and histopathologic aspects of PRP, its possible etiology, and other associated conditions.