Steroid-responsive atypical marginal zone hyperplasia of the lip in a child.

Atypical marginal zone hyperplasia (AMZH) is a recently described disease entity seen mainly in children. AMZH most commonly affects tonsils and appendices. Cutaneous AMZH is rare. The authors report here a recurrent AMZH in the lip of a 9-year-old child who presented originally with a lip swelling for approximately 3 months. The lip lesion recurred after each incomplete excision for 4 times. Pathologically, the lesion demonstrated marginal zone B-cell hyperplasia with kappa monoclonality by flow cytometry and immunohistochemistry studies. Lymphoepithelial lesions were noted with involvement of minor salivary glands. Polymerase chain reaction for immunoglobulin heavy-chain gene rearrangement has been repeatedly negative. Polymerase chain reaction for Borrelia species DNA was negative on both paraffin-embedded tissue and plasma. Serum antibodies IgG and IgM for Helicobacter Pylori were positive. A diagnosis of AMZH was made. Two courses of anti H. Pylori therapy did not improve the lip lesion, which completely regressed after a course of prednisone therapy. With differential diagnosis of cutaneous marginal zone lymphoma, the case illustrated diagnostic challenges, especially with recurrent lesions. This is the first case of recurrent cutaneous AMZH that has uncharacteristic kappa light-chain restriction. AMZH should be considered in children with mucocutaneous lesions with features of marginal zone lymphoma.

Polysensitization in recurrent lip dermatitis.

BACKGROUND: In allergic contact dermatitis (ACD), polysensitization consists of positive patch test reactions to three or more unrelated allergens. OBJECTIVE: The purpose of this case report is to document a 27-year-old female with recurrent lip dermatitis, polysensitization, and reactions to a number of clinically relevant exposures. METHODS: Patch testing results revealed positive reactions to tixocortol-21-pivalate (3+), lanolin (3+), neomycin (3+), nickel (1+), hydroxyethyl methylacrylate (3+), bacitracin (3+), and abitol (3+). RESULTS: The patient had seven positive reactions, meeting the criteria for polysensitization. Of known clinical relevance, the patient had applied lanolin-containing lip balms and hydrocortisone- and bacitracin-containing products to the lips, all of which she was strongly reactive to on patch testing. CONCLUSION: Polysensitization occurs when three or more unrelated allergens are positive on patch testing. This case highlights the importance of considering polysensitization in ACD as numerous allergens that were positive on patch testing were clinically relevant.

Food-induced anaphylaxis: mast cells as modulators of anaphylactic severity.

A food-induced anaphylactic reaction can occur within seconds to a few hours following exposure to the causal food allergen and often affects multiple organ systems including gastrointestinal, cutaneous, respiratory, and cardiovascular. A conundrum in the allergy field is that consumption of the same allergen can cause reactions of vastly different severity in separate individuals; one patient may experience a mild non-life-threatening reaction characterized by pruritis of lips or urticaria whereas another may experience a life-threatening reaction that involves respiratory and cardiovascular compromise leading to loss of consciousness and sometimes death. While there are tests available to determine the predictive risk value of a positive food challenge test or clinical reactivity, there is currently no reliable method to distinguish between individuals who are at risk of mild non-life-threatening versus life-threatening reaction. Recent research has significantly advanced our understanding of the involvement of immune pathways in the effector phase of food-induced anaphylaxis; a void remains regarding our understanding of the contribution of these pathways to severity of disease. In this review, we discuss mild non-life-threatening versus life-threatening food-induced anaphylaxis and factors (co-morbidities and immune activation) that predispose individuals to more severe disease. Furthermore, we summarize recent advancements in our understanding of the involvement of underlying immune pathways in systemic and food-induced anaphylaxis in mouse systems and discuss how these pathways may contribute to more severe disease phenotype.

Anti-neutrophil cytoplasmic antibody-positive neutrophilic dermatosis of the dorsal hands.

Neutrophilic dermatosis of the hands is a localized variant of Sweet syndrome (SS). It was first reported in 1995, and is an uncommon condition, with < 100 cases reported to date. The female preponderance, morphological and histological features, and response to treatment are similar to SS, but it differs in its distribution on the body. There may also be a lack of systemic features and inconsistent laboratory findings. Significantly, about half of all cases are associated with haematological problems, i.e. myelodysplasia and leukaemia. Other cases may be associated with ulcerative colitis or solid tumours. We describe a case of a 71-year-old man with neutrophilic dermatoses of the hands, who also had involvement of the lips. There was an associated rise in his anti-neutrophil cytoplasmic antibody level, which corresponded with the activity of the disease.

Letter:Annular atrophic lichen planus of the lip.

Annular atrophic lichen planus is a rare form of lichen planus, clinically characterized by a circumscribed annular configuration. We report herein a 32-year-old female who developed depressed plaques on the lips. Physical examination showed well-circumscribed, erythematous plaques on the lower lip. The border was slightly elevated and the inner portion was depressed and atrophic. Also, a bean-sized well-circumscribed plaque with slightly elevated border was found on the upper lip. A biopsy specimen revealed that the epithelium of the mucous membrane was reduced in thickness and a mononuclear cell infiltration invaded the basement membrane of the epithelium. This is the first case of annular atrophic lichen planus involving the lip. She was treated with 0.1 percent tacrolimus ointment once daily, which resulted in a beneficial effect.

Lip and oral mucosal lesions in 100 renal transplant recipients.

BACKGROUND: Renal transplant recipients (RTRs) appear to be more susceptible to the development of oral mucosal disease and lip cancer as a result of graft-preserving immunosuppressive therapy. However, reports regarding these pathologies other than lip cancer are scarce and not studied in a detailed manner in this patient population. OBJECTIVE: The aim of this study was to determine the prevalence rates and clinical features of lip lesions and oral mucosal lesions (OMLs) in RTRs. METHODS: In all, 100 consecutive RTRs (21 female and 79 male) and 79 healthy age- and sex-matched control subjects (23 female and 56 male) were screened for all pathologic and pseudopathologic lip lesions and OMLs, with special interest on precancerous and cancerous lesions. Information about possible associated risk factors such as smoking and alcohol consumption was also obtained. Dermatologic investigation included clinical observation and direct microscopic examination, culture, and histopathological evaluation when indicated. RESULTS: One or more lip lesions, OMLs, or both were noted in every participant of both groups. Fordyce spots on the lips was the most common lesion in the patient group (73%), followed by diffuse gingival enlargement (39%), fissured tongue (35%), and oral candidiasis (26%). The last 3 disorders were significantly more common in RTRs, whereas the frequency of Fordyce spots in patients and control subjects was similar. No actinic cheilitis, lip cancer, or oral malignancy was observed. LIMITATIONS: This was a relatively small sample size for evaluating precancerous and cancerous lip lesions and OMLs, as they are less frequently observed than benign lesions. CONCLUSIONS: Some of the benign OMLs (oral candidiasis and diffuse gingival enlargement) are increased in RTRs mainly as a result of the immunosuppressive therapy or drug side effects. Precancerous or cancerous lesions were not observed on the lips or the oral mucosa of our RTRs. This finding is in direct contrast with those of previous studies, yet this can be related to the limited sample size of this study regarding these lesions.

Novel intraoral phenotypes in hyperimmunoglobulin-E syndrome.

AIM: Hyperimmunoglobulin-E syndrome (HIES) is a primary immunodeficiency characterized by eczema, recurrent skin and lung infections with pneumatocoele formation, and extremely elevated serum immunoglobulin-E. The precise immunologic defect and genetic etiology remain unknown. Non-immunologic findings include characteristic facial features (prominent forehead, fleshy nasal tip, and increased interalar distance); skeletal involvement (pathological fractures, scoliosis, and craniosynostosis); and retention of primary teeth. This study aims to characterize intraoral soft tissue findings in HIES patients. METHODS: Sixty HIES patients (4-54 years, 27 males, 33 females) received intraoral and radiographic evaluations. Chronological dental development was also assessed. RESULTS: Lesions of the hard palate and dorsal tongue were found in 55% and 60% of patients, respectively. Palatal lesions ranged from a generalized surface keratosis to a midline sagittal fibrotic bridge. Tongue lesions consisted of multiple fissures and a midline cleft. On the lip and buccal mucosa, keratotic plaques and/or surface fissures were found in 8% and 23% of patients, respectively. Manifested in 76.7% of patients, the intraoral lesions were significantly more prevalent than the characteristic facial traits (P=0.0013). CONCLUSIONS: Alterations in oral mucosa and gingiva were present in the majority of HIES patients. These novel intraoral findings may facilitate the diagnosis of HIES.

Development of lichen planus and psoriasis on lesions of vitiligo vulgaris.

This paper reports a unique case of coexistence of vitiligo vulgaris, lichen planus and psoriasis vulgaris in a 53-year-old man. Five years after the onset of vitiligo, lichen planus developed on his lower lip. Another 4 years after the onset of lichen planus, he also exhibited psoriasis on his upper arms and trunk. Both the lichen planus and psoriasis occurred on lesions of the preceding vitiligo vulgaris. We discuss potential mechanisms for association of these three dermatoses, including Koebner phenomenon and photodamage.

Chronic disseminated histoplasmosis in an apparently immuno-competent Belgian patient.

A 77 year-old Belgian man was admitted for weight loss and elevated liver enzymes. He used to live in Central Africa until 1986 and denied any travel outside Belgium during the last decade. Physical examination was unremarkable except for a lip ulceration. Blood tests showed increases of liver enzymes and CEA. In the search of an abdominal cancer, CT showed a normal liver but enlarged surrenal glands, while colonoscopy disclosed multiple mucosal lesions. Colic and lip biopsies identified Histoplasma capsulatum var capsulatum. HIV serology was negative. Lymphocyte count was normal but lymphocyte function was depressed. The patient dramatically improved under oral treatment with Itraconazole 400 mg daily for six months. This case indicates that chronic disseminated Histoplasmosis can reactivate in a healthy and apparently non-immunocompromised person living in Europe.

Endodontic treatment in cases of allergic reaction to rubber dam.

The prevalence and severity of latex allergies have rapidly increased recently. This article presents two cases of patients with rubber latex allergy. The patient in case A was unaware of her sensitivity to latex and presented symptoms of contact dermatitis-stomatitis during endodontic treatment. The patient in case B reported latex allergy before the initiation of the treatment and a different approach was followed. Certain aspects of latex allergy related to the endodontic treatment are discussed. Moreover, a protocol is proposed for treatment of patients with latex hypersensitivity with safety.

[T and B clonal populations in actinic prurigo, a photodermatosis]. / Poblaciones clonales de células T y B en prurigo actínico, una fotodermatosis.

Actinic prurigo (AP) is a chronic photodermatosis in which genetic and immunological factors has been implicated in the pathogenesis of the disease. This work was designed to investigate the existence of clonal populations of T and B lymphocytes in lesions of the labial mucosa and conjunctiva of patients with actinic prurigo. Genomic DNA of three patients with actinic prurigo and controls were analyzed in Southern blots using DNA molecular probes for the b subunit of the T-cell receptor for antigen (TCR beta) and for the heavy chain of immunoglobulin genes (lg-JH). Clonal rearrangements of T-cell receptor genes were detected in biopsy samples taken from the labial mucosa of two patients and of immunoglobulin genes in DNA extracted from the conjunctiva of a different patient. The presence of distinct clonal T or B lymphocyte populations in patients with actinic prurigo indicates that the immune system may play a relevant role in the pathogenesis of the disease.

Autoimmune progesterone dermatitis and stomatitis.

Autoimmune progesterone dermatitis is a rare clinical condition associated with variable cutaneous and mucosal eruptions such as urticaria, erythema multiforme, and eczema. Exacerbation is influenced by hormonal changes of the menstrual cycle. The patient described in this report had recurrent cyclic lesions on the skin, oral mucosa, and lips that appeared just before regular menstruation and persisted until a few days after. During each cycle, the eruptions appeared at the previously affected sites, mimicking the clinical feature of a fixed drug eruption. This rare phenomenon is attributed to an autoimmune reaction to female sex hormones. The condition failed to respond to therapy with prednisone, but improved with the use of an antiestrogen drug, tamoxifen. This medication suppresses ovulation and the post-ovulation rise in endogenous progesterone levels.

Identification of human papillomavirus types in oral verruca vulgaris.

Eleven oral verruca vulgaris specimens were examined for the presence of papillomavirus structural antigens by reaction with antibody to type-common antigens and detection by the avidin-biotin-peroxidase complex method. The specimens were also examined by in situ hybridization with biotin-labelled human papillomavirus (HPV) DNA to determine the specific HPV types present in the lesions. Six of the 11 specimens were positive for papillomavirus structural antigens. Of these 6, 5 hybridized to the HPV Type 2 (HPV2) probe and one to the HPV4 probe.