Extensive airway hemangiomas in two patients without beard hemangiomas.

Airway hemangiomas are most often seen in association with cutaneous hemangiomas involving the "beard area." We report two unusual cases of extensive airway hemangiomas developing in patients with facial hemangiomas predominantly involving the upper face, emphasizing the need to consider the possibility of airway hemangiomas even in the absence of "beard area" hemangiomas.

Comparison of electrodesiccation and potassium-titanyl-phosphate laser for treatment of dermatosis papulosa nigra.

BACKGROUND: There is a lack of randomized split-face studies investigating treatments for dermatosis papulosa nigra (DPN) in dark skin. OBJECTIVE: To compare the efficacy, safety, and tolerability of potassium-titanyl-phosphate (KTP) laser with efficacy, safety, and tolerability of electrodesiccation in the treatment of DPN in subjects with Fitzpatrick skin phototypes IV to VI. METHODS: Fourteen subjects with Fitzpatrick skin phototypes IV to VI were randomized to receive two KTP laser treatments 4 weeks apart to half of the face. The contralateral half received two electrodesiccation treatments 4 weeks apart. Response was evaluated by photography reviewed by blinded dermatologists at 4 weeks after the second treatment. A treatment quality questionnaire about side effects and cosmetic outcome was also administered. RESULTS: Difference in improvement of DPN between the KTP side and the electrodesiccation side per each rater (p=.99, p=.54) and per raters combined (p=.50) did not reach statistical significance. There was no treatment difference for subjective effectiveness (p=.06) or subjective confidence improvement (p=.99), although there was a significant treatment difference for subjective discomfort (p=.002) in favor of KTP. Both treatments were well tolerated without significant adverse effects. CONCLUSIONS: Although treatment of DPN with KTP laser and electrodesiccation are comparable in efficacy, KTP laser is preferable for patient comfort.

Identification of autoantigens in psoriatic plaques using expression cloning.

To search for autoantigens in psoriatic plaques, we screened cDNA libraries of plaque epidermis with psoriatic serum samples. This approach has been highly successful in identifying tumor antigens, but has not been widely applied to autoimmune disease. We identified 11 autoantigens including three with prominent reactivity and plausible disease relevance. These are keratin 13 (K13), heterogeneous nuclear ribonucleoprotein-A1 (hnRNP-A1), and a previously uncharacterized protein, FLJ00294. Serum antibody screening for these demonstrated reactivity in 40%, 38%, and 27% of psoriasis patients, respectively. Most positive samples reacted with all three, and we found that this was due to cross-reactivity among them. Enzyme-linked immunospot assay (ELISPOT) analysis of psoriatic peripheral blood T cells confirmed that these autoantigens are also recognized by T cells. This demonstrates that this is a feasible method to identify autoantigens in an autoimmune target tissue, and suggests that these antigens warrant further study in psoriasis. Furthermore, but peripheral blood of normal controls reacted to these autoantigens with essentially the same frequencies as patients, suggesting that psoriatics may have not only an immune system which is capable of reacting to certain autoantigens, but also to a skin immunoregulatory alteration which allows this normal reactivity to develop into abnormal inflammation.

Food allergy and atopic dermatitis: separating fact from fiction.

The relationship between food and atopic dermatitis (AD) is complex. A common misunderstanding is that food allergies have a significant impact on the course of AD, resulting in uncontrolled attempts at elimination diets and undertreatment of the skin itself. Studies have shown that only a small portion of cutaneous reactions to food in the form of late, eczematous eruptions will directly exacerbate AD in young infants who have moderate-to-severe AD. Given the low frequency of food allergies actually inducing flares of AD, the focus should return to appropriate skin therapy, and identification of true food allergies should be reserved for recalcitrant AD in children in whom the suspicion for food allergy is high. A different relationship between food and AD involves delaying or preventing AD in high-risk infants by exclusive breastfeeding during the first 4 months of life. Finally, the skin barrier defect in AD may allow for easier and earlier sensitization of food and airborne allergens; therefore, exposure of food proteins on AD skin may act as a risk factor for development of food allergies.

Infantile hemangiomas with minimal or arrested growth: a retrospective case series.

OBJECTIVE: To describe clinical characteristics of infantile hemangiomas with minimal or arrested growth (IH-MAGs). DESIGN: Retrospective case series. SETTING: Ambulatory referral center at the University of California, San Francisco. Patients Infantile hemangiomas with minimal or arrested growth were defined as infantile hemangiomas with a proliferative component equaling less than 25% of their total surface area. The patients must have been at least age 2 months at the initial visit or on follow-up. Forty-two eligible patients with 47 IH-MAGs were included in the study. MAIN OUTCOME MEASURES: Medical record review was performed for demographic and gestational information, lesion size, and clinical appearance, presence of proliferation, complications, coexisting classic infantile hemangiomas, and morphologic subtype classified as localized, segmental, or indeterminate. RESULTS: Infantile hemangiomas with minimal or arrested growth manifested most commonly as fine or coarse telangiectatic patches. Proliferation was present in 30% (14 of 47 IH-MAGs), usually as small papules at the periphery of these hemangiomas. Sixty-eight percent (32 of 47 IH-MAGs) of them were present on the lower body. Seventeen patients had classic infantile hemangiomas at another body site. Comparison of distribution of sites of IH-MAGs showed a 26-fold (95% confidence interval, 1.9-351.5; P = .01) likelihood of having IH-MAGs on the lower body compared with classic infantile hemangiomas. CONCLUSIONS: Infantile hemangiomas with minimal or arrested growth have a distinct clinical appearance and a unique predilection for the lower body. Recognition of IH-MAGs will help in more accurate diagnosis of vascular birthmarks of infancy, and the presence of IH-MAGs in an individual patient does not exclude the proliferative potential of other infantile hemangiomas that may be present.

Propranolol in the management of airway infantile hemangiomas.

OBJECTIVE: To report our experience with propranolol in managing airway infantile hemangiomas. DESIGN: Case series of 3 consecutive patients who had extensive, symptomatic airway infantile hemangiomas treated with propranolol. SETTING: Tertiary academic medical center. PATIENTS: Three infants with facial cutaneous hemangiomas who developed stridor that progressed to respiratory distress, which according to laryngoscopic examination results was confirmed to be caused by extensive subglottic hemangiomas. These patients underwent follow-up during their course of therapy, ranging from 3 weeks to 15 months. RESULTS: Patient 1 failed to respond to systemic corticosteroids, laser ablation, and intravenous vincristine for her airway hemangioma and had to undergo tracheotomy. She was given propranolol after her tracheotomy and had a significant reduction in her subglottic airway obstruction. Patient 2 developed progressive stridor secondary to airway hemangioma at age 6 1/2 months following tapering of systemic corticosteroids prescribed for her periorbital hemangioma. Systemic corticosteroids were restarted with the addition of propranolol. The stridor improved within 24 hours, and she was able to be weaned off corticosteroids. Patient 3 was also treated with initial combined therapy of systemic corticosteroids and propranolol. He had a significant reduction in stridor within 24 hours and was weaned off corticosteroids. CONCLUSIONS: Our 3 patients had severe respiratory symptoms related to their airway infantile hemangiomas. In the first patient, propranolol was used when other treatments were ineffective or associated with intolerable adverse effects. In the second and third patients, propranolol was part of a dual regimen that resulted in rapid resolution of airway symptoms and allowed for quicker weaning of corticosteroids.

p63 in Primary Cutaneous Carcinosarcoma.

Primary cutaneous carcinosarcomas (PCCs) are rare malignant neoplasms that are characterized by biphasic epithelial and mesenchymal differentiation. When the biphasic nature is not evident, immunohistochemical studies may be important in the diagnosis of PCCs. Although AE1/AE3 is frequently used to demonstrate the epithelial component, it may not be strongly expressed in epithelial cells that are not well-differentiated. p63 is a protein homologue of p53 that is expressed in poorly differentiated epithelial cells. We report 3 cases of PCC. The clearly epithelial areas of each tumor were frequently positive for both markers, whereas the sarcomatous areas were negative for both markers. Epithelial cells that were poorly differentiated and not easily identifiable were positive for p63 but negative for AE1/AE3. Of interest, transitional areas showed positivity for p63 alone. These 3 cases suggest that the use of both p63 and routine cytokeratin markers such as AE1/AE3 can increase the sensitivity for distinguishing epithelial cells over a range of differentiation states, which we propose will aid in the diagnosis of PCCs. In addition, the staining pattern of AE1/AE3 and p63 in these cases further supports the conversion theory of PCC.

Profound loss of T-cell receptor repertoire complexity in cutaneous T-cell lymphoma.

Cutaneous T-cell lymphoma (CTCL) is a malignancy of skin-homing T cells. A major feature of CTCL is profound immunosuppression, such that patients with advanced mycosis fungoides or Sézary syndrome have been compared with patients with advanced HIV disease and are susceptible to opportunistic infection. The etiology of this immunosuppression is unclear. We analyzed peripheral blood T cells of patients with CTCL with stage I to IV disease, using a sensitive beta-variable complementarity-determining region 3 spectratyping approach. Our data revealed a profound disruption of the complexity of the T-cell repertoire, which was universally observed in patients with advanced disease (stages III and IV), and present in up to 50% of patients with early-stage disease (stages I and II). In most patients, multiple monoclonal and oligoclonal complementarity-determining region 3 (CDR3) spectratype patterns in many different beta-variable families were seen. Equally striking was a reduction of normal T cells (as judged by absolute CD4 counts) across multiple beta-variable families. In general, CTCL spectratypes were reminiscent of advanced HIV spectratypes published elsewhere. Taken together, these data are most consistent with a global assault on the T-cell repertoire in patients with CTCL, a process that can be observed even in early-stage disease.