Familial leiomyomatosis: a review and discussion of pathogenesis.

Multiple cutaneous leiomyomas of pilar origin have long been recognized to have an autosomal dominant inheritance. While the skin tumors are relatively uncommon and benign, women of affected families often develop uterine fibroids with associated infertility, pain and bleeding. In addition, a subset of these families harbors a predisposition to papillary renal cell carcinoma. Germline mutations in a recently identified classical tumor suppressor gene encoding fumarate hydratase are observed in these individuals. Appropriate screening measures for associated disorders are mandatory.

Langerhans cell histiocytosis in a child while in remission for acute lymphocytic leukemia.

The occurrence of Langerhans cell histiocytosis (LCH) and malignancy in the same patient is rare. When LCH occurs concomitantly with acute leukemia, distinct temporal patterns often exist; acute myelogenous leukemia (AML) typically succeeds LCH, whereas acute lymphocytic leukemia (ALL) usually precedes it. We report a case of LCH developing in a child while in remission for ALL. Unique features of this case include the disseminated nature of the LCH and the death of the patient from LCH rather than ALL.

Dysplastic nevi of the scalp and forehead in children.

To determine if there is a significant difference in the relative frequency and degree of atypia of sporadic dysplastic nevi from the scalp, face, and neck area in children as compared with nevi from the rest of the body, we reviewed 99 consecutive biopsy specimens of melanocytic nevi from the scalp, face, and neck areas in children less than 18 years of age and compared them with 95 consecutive cases of nevi from other areas of the body in children of the same age. Large numbers of the nevi biopsied from the scalp (13 of 31; 41.93%) and forehead (2 of 10; 20%) were dysplastic. The number of dysplastic nevi from the neck (1 of 58; 1.72%) was not assessed as very different from the incidence found in other regions of the body, where 7 dysplastic nevi (7.36%) from a total of 95 nevi were found. Of the 13 dysplastic nevi from the scalp, 9 showed minimal atypia and 4 showed moderate atypia. No nevi with severe atypia were found. Many pigmented nevi from the scalp and forehead in children in this study were dysplastic. This finding points out the importance of examining the scalp of children for the presence of dysplastic nevi. The majority of nevi from the neck were common nevi.

Managing pediatric atopic dermatitis.

Although atopic dermatitis is a very common inflammatory skin condition in children and results in many pediatric healthcare visits, its exact cause is unknown. No single laboratory test can reliably diagnose atopic dermatitis, but a relatively simple set of diagnostic criteria was recently validated for use by practicing physicians. Because existing remedies for atopic dermatitis do not cure the disorder, a program of disease control and management should be pursued. Patients and their caregivers should be advised that current therapies are primarily preventive and palliative. However, a comprehensive plan that includes routine general skin care, medical management of symptoms, identification and avoidance of aggravating factors (including psychological factors), and attention to quality-of-life issues can reduce the occurrence of skin flares. Successful treatment of acute flare-ups can be achieved with appropriate use of topical corticosteroids, but occasionally children afflicted with severe atopic dermatitis require more intensive therapies (e.g., ultraviolet light exposure systemic corticosteroids, and cyclosporine) that need close physician monitoring. Physicians must remain mindful of the psychological and quality-of-life burdens imposed on children with atopic dermatitis and their families and tailor treatments to the needs of each individual patient.

New and emerging therapies in pediatric dermatology.

Many of the dermatologic conditions for which children seek medical attention are caused by infectious organisms. Several medications have recently become available or are on the horizon for the treatment of pediatric skin infections and infestations. Treatment of tinea capitis with fluconazole, itraconazole, and terbinafine, antibiotic therapy for staphylococcal skin infections, cidofovir for the treatment of verrucae vulgaris and molluscum contagiosum and ivermectin for scabies and head lice are discussed.

Herpes gestationis in a mother and child.

Herpes gestationis (pemphigoid gestationis) is a rare autoimmune disease that appears during pregnancy or in the immediate postpartum period. We report the cases of a mother and neonate with immunofluorescence confirmed herpes gestationis both of whom had extensive cutaneous involvement.

Dye rashes.

Physicians may administer intravenous dyes to patients, most commonly to delineate vascular or urinary anatomy, without an appreciation of the potential hazards associated with these compounds. We report two cases in which skin eruptions followed the intravenous administration of the dyes fluorescein and methylene blue; these eruptions were the same colors as the dyes. In our first patient, urticaria, which was yellowish in color and fluorescent under a Wood's lamp, occurred after the administration of fluorescein. In the second patient, painful blue macules appeared randomly on the forearm within 15 seconds after methylene blue was injected into a free-flowing intravenous cannula on the dorsal aspect of the hand.

A randomized, vehicle-controlled trial of tacrolimus ointment for treatment of atopic dermatitis in children. Pediatric Tacrolimus Study Group.

BACKGROUND: A topical formulation of tacrolimus, an immunosuppressant currently marketed for the prevention of rejection after solid organ transplant, is a potential therapeutic agent for atopic dermatitis. OBJECTIVE: We sought to determine the safety and efficacy of tacrolimus ointment in pediatric patients with moderate-to-severe atopic dermatitis. METHODS: In this double-blind, vehicle-controlled multicenter trial, children ages 7 to 16 years were treated with twice daily application of tacrolimus ointment at 1 of 3 concentrations (0.03% [n = 43], 0.1% [n = 49], or 0.3% [n = 44]) or vehicle (n = 44) for up to 22 days, with a 2-week follow-up period. RESULTS: The Physician's Global Evaluation of clinical response showed that 69% (95% confidence interval: 53-82) of patients in the 0.03% tacrolimus ointment group, 67% (95% confidence interval: 52-81) in the 0.1% tacrolimus ointment group, and 70% (95% confidence interval: 54-83) in the 0.3% tacrolimus ointment group, compared with 38% (95% confidence interval: 24-54) in the vehicle group, had a marked to excellent (> or =75%) improvement or clearing of their atopic dermatitis (P =.005, .007, and .004, respectively for the 3 tacrolimus groups compared with the vehicle group). The mean percent improvement for a modified Eczema Area and Severity Index at end of treatment for each of the 3 tacrolimus groups (0.03%, 72%; 0.1%, 77%: and 0.3%, 81%) was significantly better than that of the vehicle group (26%; P <.001). The median percent reduction in pruritus was significantly greater for tacrolimus-treated patients (74% to 89%) than for vehicle-treated patients (51%, P = .027). No serious systemic adverse events were noted, and systemic absorption was minimal. CONCLUSION: Tacrolimus ointment appears to be safe and effective in children with atopic dermatitis.

Mid-dermal elastolysis in an adolescent subsequent to lesions resembling granuloma annulare.

First described by Shelley and Wood in 1977, mid-dermal elastolysis (MDE) is a rare acquired disorder in which there is a bandlike absence of elastic tissue limited to the mid-dermis. In their patient, MDE developed in an area previously involved with recurrent episodes of urticaria. We describe a 15-year-old white girl with well-circumscribed, minimally palpable yellow-white plaques and wrinkling diagnosed histologically as MDE in areas clinically diagnosed 5 years previously as granuloma annulare. As in the first described patient, five years elapsed between clearance of the original skin lesions and the clinical appearance of MDE. To our knowledge, we report the first adolescent case of MDE localized to previous sites of lesions clinically consistent with granuloma annulare and propose that MDE represents an abnormal end-stage reaction to multiple processes.

Nonlipidized juvenile xanthogranuloma: a histologic and immunohistochemical study.

Among all patients with a pathologic diagnosis of juvenile xanthogranuloma (JXG) seen at our institution from 1983 to 1994, we identified five patients with an unusual histologic pattern that differed from the classic juvenile xanthogranuloma (CJXG) with foamy cells and Touton giant cells. Four of these five cases, which we termed nonlipidized juvenile xanthogranuloma (NJXG), were seen in infants. The histologic features include a monomorphic infiltrate with absent or few foam cells and Touton giant cells. There is little inflammation, and mitotic figures are easily found. Four cases exhibit a diffuse sheetlike pattern while one is trabecular. Immunoperoxidase staining was done. All lesions are consistently positive for factor XIIIa as opposed to only focally positive or negative in CJXG and negative in Langerhans cell histiocytosis (LCH). The S-100 was negative. NJXG represents an atypical histologic variant of JXG, which may suggest a malignant or aggressive tumor. The follow-up, however, indicates that these lesions behave in a fashion similar to those of CJXG. The differential diagnosis should be made with LCH, intradermal nevus, and reticulohistiocytosis. The immunoperoxidase findings help to differentiate NJXG from these entities.

EBV-associated Kikuchi's histiocytic necrotizing lymphadenitis with cutaneous manifestations.

The clinical and pathologic findings of Kikuchi's histiocytic necrotizing lymphadenitis may mimic those of malignant lymphoma. We describe a 6-year-old boy with generalized lymphadenopathy, spiking fever, chills, myalgias, malaise, and erythematous, crusted papules. Although cutaneous manifestations have been noted in 16% to 40% of patients with histiocytic necrotizing lymphadenitis, only three publications described skin lesions. The skin lesions and affected lymph nodes revealed histiocytic aggregates, atypical lymphoid cells, karyorrhectic debris, and patchy necrosis. Spontaneous resolution occurred in 2 months. Results of serologic studies, Epstein-Barr virus (EBV) latent membrane protein immunoperoxidase staining, EBER-1 RNA in-situ hybridization, and EBV EBNA-1 DNA polymerase chain reaction implicate EBV as the causative agent.

Congenital smooth muscle hamartoma presenting as a linear atrophic plaque: case report and review of the literature.

Congenital smooth muscle hamartoma usually manifests as a well-circumscribed, hyperpigmented plaque, frequently hypertrichotic, on the trunk or extremities. We report such a lesion in a 7-month-old girl that presented as a linear, mottled, purplish red plaque appearing in areas to be atrophic, involving her right buttock, posterior thigh and leg, and fifth toe. Although the clinical appearance suggested linear morphea, a biopsy specimen had numerous haphazardly oriented bundles of smooth muscle in the reticular dermis. Masson trichrome staining, smooth muscle specific actin, and electron microscopic studies confirmed the smooth muscle nature of the cells. A diagnosis of smooth muscle hamartoma was made. To our knowledge, this linear clinical presentation has not been described previously.