Idiopathic lymphoplasmacellular mucositis of the lips: A case report and review of the literature

Idiopathic lymphoplasmacellular mucositis (ILPM) is a group of disorders histologically characterized by dense infiltrates of lymphocytes and plasma cells in submucosa. These lesions were initially observed on the glans penis and vulva, and later on buccal mucosa, lips, gingiva, tongue, epiglottis, larynx and other mucosal surfaces have also been reported. We have reviewed the previously reported cases of ILPM with lip involvement, outlining the clinical presentation, treatment and outcome of the entity to date. The etiology of ILPM is unknown, and the diagnosis is largely one of exclusion. To date, no consensus or consistently effective treatment for ILPM is available. Herein, we report an 18‐year‐old woman presented with markedly swollen lips with severe erosions, bleeding and yellow to black crusts who was diagnosed to have ILPM by histopathological study. The case occurred on rare lip site with an unusual appearance. Various treatment modalities were tried, but the responses were unsatisfactory and the lesion only responded to long‐term administration of oral steroids.     

Dermatitis artefacta of the breast: a retrospective analysis of 27 patients (1976–2006)

Background Dermatitis artefacta (DA) is defined as all dermatological, self‐inflicted skin lesions, where the patient denies having produced the lesions. Objectives The purpose of this study is to make a single‐centre retrospective clinical review of patients diagnosed as DA of the breast. Materials and methods During a 30‐year period (1976–2006), patients diagnosed as DA of the breast, seen in the Department of Dermatology of the Virgen Macarena Hospital in Seville, were recorded. Clinical and epidemiological features are described. Results A total of 27 women with a mean age of 34.33 years were selected representing 13.43% of the total of DA patients recorded (n = 201) in this period. The most frequent clinical forms were: excoriations (nine patients, 33.33%) and ulcers (nine patients, 33.33%), followed by burns (six patients, 22.22%), blisters (one patient, 3.70%), contact dermatitis (one patient, 3.70%) and haematomas (one patient, 3.70%). Ten of the cases were located exclusively on the breasts, whereas 17 had also other locations such as face in seven cases, arms in five cases, abdomen in five cases and the entire body in two cases. Cutaneous lesions were treated with occlusive bandages using zinc paste or plaster splint when necessary. Conclusion To our knowledge, this is the major series of DA of the breast studied. This complicated psychodermatological condition requires a correct diagnosis, appropriate management and psychiatric assessment.     

Phase 1 studies to assess the safety,tolerability and pharmacokinetics of JTE‐052 (a novel Janus kinase inhibitor) ointment in Japanese healthy volunteers and patients with atopic dermatitis

The purpose of the present two phase 1 studies was to assess the safety, tolerability and pharmacokinetics for topical application of a novel Janus kinase (JAK) inhibitor, JTE‐052, in Japanese healthy adult male volunteers and Japanese adult patients with atopic dermatitis (AD). Additionally, exploratory investigation was performed on the efficacy for disease severity and pruritus score in AD patients. In the QBX1‐1 study, the cutaneous safety of JTE‐052 ointment by a patch test and a photo patch test was assessed in an intra‐individual comparative study using placebo ointment, white petrolatum and non‐application as comparators. The study demonstrated that JTE‐052 ointment would be associated with a low potential for phototoxicity but had no potential for skin irritation or photoallergy. In the QBX1‐2 study, it was revealed that the systemic exposure to JTE‐052 in both healthy volunteers with normal skin and AD patients with inflamed skin was low in application of not only 1% but also 3% JTE‐052 ointment. JTE‐052 ointments of 1% and 3% were generally safe and well tolerated in both populations. In a repeated twice‐daily application for 7 days, the efficacy of JTE‐052 ointment to AD patients was observed with both 1% and 3% ointments in the exploratory investigations evaluated by Eczema Area and Severity Index, Investigator's Global Assessment and Numeric Rating Scale assessments. The mean scores for each assessment declined from the baseline throughout the study. These results suggest that the treatment of JTE‐052 ointment is generally safe and effective in AD patients, although further large confirmatory studies are needed.     

Macrophage migration inhibitory factor (MIF) in the stratum corneum: a marker of the local severity of atopic dermatitis

Different biomarkers are used to evaluate the severity of atopic dermatitis (AD); however, it remains difficult to determine the severity of localized skin lesions. MIF plays an essential role in the pathophysiology of skin inflammation. To establish whether the MIF level in the stratum corneum (SC) serves as a marker of the severity of AD lesions, we examined the SC MIF (scMIF) levels in AD patients. The SC of the cheek, neck and upper arm skin was collected using tape stripping, and the scMIF levels were measured. Consequently, the scMIF levels were found to be significantly higher in the involved skin lesions than the uninvolved areas within the same patient. Moreover, the scMIF levels were significantly correlated with the severity of local skin lesions. These findings suggest that the scMIF level can be used as an effective marker for evaluating the local severity of AD.     

Incomplete efficacy of 5-aminolevulinic acid (5 ALA) photodynamic therapy in the treatment of widespread extramammary Paget's disease

Photodynamic therapy (PDT) using 5-aminolevulinic acid (5-ALA) is an effective treatment for several conditions such as Bowen's disease, subsets of basal cell carcinomas and actinic keratosis. Surgical resection is the first-choice therapy for extramammary Paget's disease (EMPD), but extensive resection is highly invasive and recurrences are frequent. We report two cases of genital EMPD treated by PDT with partial efficacy. The first patient, a 78-year-old male, suffered from pubic and scrotal Paget's disease for 6 years despite numerous treatments. The second patient, a 78-year-old female, had vulvar involvement for 2 years that was resistant to multiple treatments. The disease was recurrent and chronic with important pruritus and significant impact on the quality of life. Methyl 5-aminolevulinate was applied for 3 h, and irradiation was applied with red light (630 nm) using a total light dose of 37 J/cm(2) for a period of 10 min. The patients were treated every 2 to 4 weeks for a total of at least three treatments. Both patients experienced a partial transient reduction in their symptoms. One patient had a partial transient remission (< 50% reduction of the involved surface), whereas in the other patient, PDT failed to reduce the surface area of the lesions.     

Levels of immunoglobulin E specific to the major food allergen and chemokine (C‐C motif) ligand (CCL)17/thymus and activation regulated chemokine and CCL22/macrophage‐derived chemokine in infantile atopic dermatitis on Ishigaki Island

Atopic dermatitis (AD) is a multifactorial T‐helper (Th)2‐mediated skin disease frequently associated with elevated serum immunoglobulin (Ig)E and food allergy is also a Th2‐ and IgE‐mediated adverse immunological reaction. Our previous study indicated the relation of egg allergy history and disease severity of AD. Thus, the purpose of the study was to investigate the levels of IgE specific to major food allergens (egg, milk, wheat) and Th2 chemokines (chemokine [C‐C motif] ligand [CCL]17/thymus and activation regulated chemokine [TARC] and CCL22/macrophage‐derived chemokine [MDC]) and the relationship between them. A total of 743 nursery school children were enrolled. Dermatologist‐based physical examination and a questionnaire survey were also conducted. Significantly increased levels of disease severity markers (CCL17/TARC and CCL22/MDC) were confirmed in children with AD. The levels of CCL22/MDC in all of the children were markedly high compared with those reported in adults. IgE specific to egg white, ovomucoid, wheat and mite antigen were significantly higher in the AD group than in the non‐AD group. Among them, IgE specific to egg allergens were well associated with disease severity markers, and IgE specific to ovomucoid seemed particularly well correlated with the presence of egg allergy history. In conclusion, the markedly high level of CCL22/MDC in children as compared with those reported in adults may partly explain the AD‐prone nature of children and their spontaneous remission afterwards. Mild but significant correlation of IgE specific to egg allergens and Th2 chemokines may explain correlation of disease severity and comorbidity of egg allergy in our previous study.     

Cystic fibrosis transmembrane conductance regulator is helpful in the distinction of extra-mammary Paget's disease from squamous cell carcinoma in situ (Bowen's disease)

Cystic fibrosis transmembrane conductance regulator (CFTR) represents a cAMP-dependent channel found in normal apocrine glands. The classification and histogenesis of extra-mammary Paget's disease (EMPD) remains controversial, but it is generally accepted that primary EMPD exhibits apocrine differentiation. Therefore, we examined the utility of CFTR in the differential diagnosis of EMPD and squamous cell carcinoma in situ (SCCIS). Twenty-five cases of SCCIS and 14 cases of EMPD were evaluated for immunohistochemical expression of CFTR. Expression was scored as 0 (<5% of cells positive), 1+ (5-75% of cells positive) or 2+ (>75% cells positive). Twenty-three of 25 cases of SCCIS showed no reactivity for CFTR, and the remaining 2 cases showed 1+ staining. Thirteen of 14 cases of EMPD showed 2+ staining, while 1 case showed 1+ staining. We recognize that the pathological appearance along with clinical history and site of occurrence are sufficient to distinguish EMPD and SCCIS in most instances. However, distinction between the two can become more challenging when the location and histopathology are not characteristic. We conclude that when an immunohistochemical panel is diagnostically necessary, the expression of CFTR favors a diagnosis of EMPD over SCCIS.     

Congenital “self‐healing” Langerhans cell histiocytosis (Hashimoto‐Pritzker disease): A report of two cases with the same cutaneous manifestations but different clinical course

Congenital self‐healing Langerhans cell histiocytosis or Hashimoto‐Pritzker disease is a rare condition present at birth or in the neonatal period characterized by small reddish‐brown crusted papulonodular lesions. In most cases these lesions are not accompanied by systemic findings and tend to involute spontaneously within weeks or months, but in other cases there may be extracutaneous involvement and/or recurrence of the disease. This emphasizes that the clinical course is variable and a long‐term follow‐up is mandatory in order to reveal possible systemic involvement. We describe two cases of congenital self‐healing Langerhans cell histiocytosis with widespread and very similar cutaneous manifestations but different clinical course. The first patient had multisystemic disease (with lymph nodes, bones, liver and lungs affected) that required systemic therapy. The second patient had cutaneous and bony lesions that resolved spontaneously. We think that the adjective “self‐healing” is misleading and should be abandoned. We stress the importance of a complete systemic evaluation and the necessity of a long‐term follow‐up.