Annular lichenoid dermatitis of youth: a separate entity or on the spectrum of mycosis fungoides? Case report and review of the literature

Annular lichenoid dermatitis (ALDY) is a rare dermatosis that is most often seen in children and young adults and is characterized by annular patches with raised borders, most frequently on the trunk and the groin. A distinct lichenoid tissue reaction involving the base of the rete, resulting in squared‐off rete ridges, helps to differentiate this from other lichenoid dermatoses and mycosis fungoides (MF). Herein, we report an additional case of this condition in a 7‐year‐boy, whose biopsy exhibited the typical quadrangular rete alteration and also contained distinct aggregates of CD8+ lymphocytes, Langerhans cells and colloid bodies within the involved rete. A literature review with emphasis on the clinical and histopathological differential diagnosis reveals additional clinical features of ALDY to potentially help differentiate this entity from annular presentations of mycosis fungoides.     

Onychogryphosis in elderly persons: an indicator of long-standing poor nursing care? Report of one case and review of the literature

A 92-year-old immobilized white woman under the daily nursing care of a private ambulatory nursing service displayed acquired deformities of the toenails resembling a ram's horn. In light of a rapidly growing elderly population, this case report illustrates the need for close monitoring of the quality of care that nursing services provide to older persons. In addition, it reviews the clinical aspects of onychogryphosis, as well as its pathomechanisms and treatment options.     

Large solitary café au lait spots: a report of 5 cases and review of the literature

The presence of multiple café au lait spots (CALSs) has been well described and associated with several neurocutaneous and genetic syndromes including, most commonly, neurofibromatosis. However, scant literature exists regarding the clinical significance of the large solitary CALS. We describe 5 patients with congenital large solitary CALSs without associated abnormalities. Our cases and review of the literature suggest that large solitary CALSs are uncommon benign lesions that most likely are not associated with any neurocutaneous syndromes or developmental anomalies.     

Bi-clonal, multifocal primary cutaneous marginal zone B-cell lymphoma: report of a case and review of the literature

Bi-clonality is a rare phenomenon seen in approximately 5% of chronic B-cell lymphoproliferative disorders. Both true bi-clonality and somatic hypermutation resulting in intraclonal evolution have been described. We present the case of a 37-year-old female who developed extranodal marginal zone B-cell lymphoma with immunohistochemical studies showing monotypic immunostaining of plasma cells for immunoglobulin lambda light chain on her right arm in 2008. Three years later, she developed a second focus of extranodal marginal zone B-cell lymphoma on her left arm, but immunohistochemical studies demonstrated monotypic immunostaining of plasma cells for immunoglobulin kappa light chain confirmed after repeat analysis. Evaluation for systemic lymphoma with laboratory and imaging studies was negative. Together, the findings were consistent with bi-clonal, multifocal extranodal primary cutaneous marginal zone B-cell lymphoma. We present this case to highlight a rare phenomenon within primary cutaneous marginal zone lymphomas.     

Löffler syndrome caused by extensive cutaneous larva migrans: a case report and review of the literature

In rare cases, cutaneous larva migrans may be complicated by L?ffler syndrome. This syndrome is thought to result from a type I hypersensitivity reaction related to the pulmonary larval migration phase of various parasites. It is characterized by migratory pulmonary eosinophilic infiltrates and peripheral eosinophilia, with malaise, fever, and cough. Our patient was successfully treated with ivermectin, a corticosteroid cream, and inhalation medication in an early phase, which prevented complications. We present the details of this case and review the literature.     

Circumscribed palmo‐plantar hypokeratosis: a disease of desquamation? Immunohistological study of five cases and literature review

Background Circumscribed palmoplantar hypokeratosis (CPH) is a recently recognized, rarely reported dermatosis that shows characteristic clinicopathological features; however, its pathogenesis remains unknown. Objective The aim of this study was to get further insight into the pathogenesis of CPH. Methods An immunohistological study was performed on five cases of CPH to investigate the expression of several epidermal proliferation and differentiation proteins, with emphasis on those involved in corneocyte desquamation [including corneodesmosin, kallikrein 5 and lympho‐epithelial Kazal type inhibitor (LEKTI)]. Results In three of five cases, a decreased expression of LEKTI, corneodesmosin and filaggrin was found, along with an increased expression of kallikrein 5 and keratin 6. The expression of several other antigens (including involucrin, Ki67, p63, CD138/syndecan I, EGF‐R) did not present a consistently different pattern as compared with the unaffected epidermis. Conclusion The immunohistopathologic features of CPH suggest that an altered (accelerated) corneocyte desquamation process could be the main pathological mechanism underlying the development of lesions.     

Is cocamidopropyl betaine a contact allergen? Analysis of network data and short review of the literature

Background. There is no general agreement on whether cocamidopropyl betaine (CAPB) is a skin sensitizer. Objective. To examine the evidence for CAPB being a (non‐)sensitizer. Methods. This was a retrospective analysis of data on patch testing with CAPB 1% aqua collected by the Information Network of Departments of Dermatology from 1996 to 2009, with a focus on the patch test reaction profile, and demographic and clinical features of CAPB positives, supplemented by a literature review. Results. Eighty‐three thousand eight hundred and sixty‐four patients were patch tested with CAPB 1% aqua, yielding 2.16% [95% confidence interval (CI) 2.06–2.26%] positive (2.03% + and 0.13% + + /+ + + ) and 4.6% non‐allergic reactions. Thus, the reaction index was—0.368 and the positivity ratio was 94.2%. Reproducibility on synchronous patch testing (n = 6534) was poor [Cohen's kappa: 0.29 (95% CI 0.25–0.32)] and results upon retesting (n = 1157) were almost non‐reproducible [kappa: 0.12 (95% CI 0.05–0.19]. Multifactorial logistic regression analysis revealed an increased risk associated with being male and aged ≥40 years, with atopic dermatitis, with scalp dermatitis, with being a hairdresser, and with a 48‐hr patch test application. When only + + or + + + reactions were used as a conservative outcome, only the elevated risk in males and in patients with atopic dermatitis remained significant. Conclusion. The vast majority of positive reactions to CAPB are presumably false positive. Allergic reactions are very rare. This would support the notion of CAPB being ‘not a significant skin sensitizer’, in line with current classification systems.     

Unilesional follicular mycosis fungoides: report of two cases with progression to tumor stage and review of the literature

Mycosis fungoides (MF) is the most common type of cutaneous lymphoma and has protean clinicopathological manifestations. Follicular or folliculotropic MF (FMF) is a rare variant, which histopathologically is characterized by pronounced folliculotropism of neoplastic T cells, with or without follicular mucinosis, and clinically by an impaired prognosis compared to classic MF. In contrast, unilesional MF is a very rare variant with an excellent prognosis, with a single case of large-cell transformation reported to date. The combination of folliculotropic and unilesional MF is very unusual, with only two cases reported to date. Here we report two patients with unilesional folliculotropic MF with progression to tumor stage in both patients. To the best of our knowledge, this is the first report on the disease evolution with large-cell transformation and progression of unilesional FMF. Complete remission was achieved by local radiation therapy in both patients. The differential diagnoses, classification and implications for the treatment of unilesional FMF as well as the pertinent literature are discussed.     

Is tuberculin skin testing reliable during anti-tumor necrosis factor-alfa therapy? A case report and review of the literature

The increased risk to patients on anti-tumor necrosis factor-alfa therapy of developing active tuberculosis supports screening of these patients for latent tuberculosis infection. The current practice of determining the presence of latent tuberculosis infection primarily, and often entirely, depends on the use of tuberculin skin testing (TST). We report a patient with psoriasis on long-term etanercept therapy who had a negative TST result and a positive interferon-gamma release assay result. Similar cases have also been found through a review of the literature. These findings suggest that TST might be unreliable during long-term anti-tumor necrosis factor-alfa therapy. QuantiFeron-TB Gold testing may be a more appropriate primary test in patients with risk factors for false-negative TST results.     

Eosinophilic fasciitis 30 years after - what do we really know? Report of 11 patients and review of the literature

BACKGROUND: Eosinophilic fasciitis (EF) is a rare fibrosing disorder associated with peripheral eosinophilia and scleroderma-like induration of the distal extremities which affects substantially quality of life. Although the disease has been described 30 years ago, the etiology and pathomechanisms are still obscure, and consensus for therapy is lacking. Numerous case reports of patients with EF exist but series are scarce. PATIENTS AND METHODS: Eleven patients with EF from the Department of Dermatology, Kantonsspital Aarau, the University Hospital Basel and the Outpatient Clinic of Dermatology, Triemli Hospital Zurich, Switzerland, were retrospectively studied. RESULTS: In 4 patients the initial diagnosis was not recognized by the referring nondermatologists. The median age was 55 years, excluding the youngest patient ever diagnosed with EF (age = 1 year). All patients showed an induration of the skin, which led to painful contractures in the joints in 3 cases. All but 2 patients demonstrated edema. A slight predominance of the upper extremities was observed. Sclerodactyly was noticed in 1 patient. Three patients reported an initial trauma at the affected site. Two patients were tested positive for borreliosis. One patient subsequently developed aplastic anemia and Hashimoto thyroiditis. Visceral or extracutaneous involvement was absent. Eight patients had a full or partial recovery under corticosteroids whereas in 2, improvement could be achieved only with cyclosporine, azathioprine or cyclophosphamide. CONCLUSIONS: The diagnosis of EF can be established by clinical, laboratory and histological findings. In general, corticosteroids are highly efficacious in EF and only a minority of patients need other immunosuppressive or cytostatic drugs.     

Aplasia cutis congenita of the scalp- what are the steps to be followed? Case report and review of the literature

Aplasia cutis congenita is a rare malformation characterized by localized congenitalabsence of the skin. It rarely occurs on the trunk and limbs, and can occur inisolation or as part of a heterogeneous group of syndromes. We report a case of a4-day-old boy with a 5.6-cm- diameter tumor, with a central crust, non-indurate andno inflammatory rim; localized on the scalp and a small, atrophic hairless scarappeared 6 months later (approximately 5cm in length) at the site of the previoustumor.     

Temperature and humidity affect the incidence of hand,foot, and mouth disease: a systematic review of the literature – a report from the International Society of Dermatology Climate Change Committee

Hand, foot, and mouth disease (HFMD) is an enterovirus-mediated condition that predominantly affects children under 5 years of age. The tendency for outbreaks to peak in warmer summer months suggests a relationship between HFMD and weather patterns. We reviewed the English-language literature for articles describing a relationship between meteorological variables and HFMD. Seventy-two studies meeting criteria were identified. A positive, statistically significant relationship was identified between HFMD cases and both temperature (61 of 67 studies, or 91.0%, reported a positive relationship) [CI 81.8–95.8%, P = 0.0001] and relative humidity (41 of 54 studies, or 75.9%) [CI 63.1–85.4%, P = 0.0001]. No significant relationship was identified between HFMD and precipitation, wind speed, and/or sunshine. Most countries reported a single peak of disease each year (most commonly early Summer), but subtropical and tropical climate zones were significantly more likely to experience a bimodal distribution of cases throughout the year (two peaks a year; most commonly late spring/early summer, with a smaller peak in autumn). The rising global incidence of HFMD, particularly in Pacific Asia, may be related to climate change. Weather forecasting might be used effectively in the future to indicate the risk of HFMD outbreaks and the need for targeted public health interventions.     

Acrochordons are not a component of the Birt-Hogg-Dubé syndrome: does this syndrome exist? Case reports and review of the literature.

Multiple fibrofolliculomas, trichodiscomas, and acrochordons compose the triad of cutaneous lesions characterizing the Birt-Hogg-Dubé syndrome, inherited in an autosomal dominant fashion. We report a case of a family who had the triad of tumors of the Birt-Hogg-Dubé syndrome. Two members were observed clinically and histologically. Biopsies of the facial papules disclosed features of the fibrofolliculoma/trichodiscoma spectrum. Lesions that were clinically acrochordon-like proved to correspond to the same histopathologic spectrum. The characterization of the nature of the acrochordon-like lesions in our patients and the review of the literature allow us to question if acrochordons (skin tags) should be maintained as a component of this association. In light of our conclusion that fibrofolliculoma, trichodiscoma, and the acrochordon-like lesions are histologic variations of a single lesion, we further question whether the term "syndrome" is valid.     

Progressive extensive osteoma cutis associated with dysmorphic features: a new syndrome? Case report and review of the literature

Osteoma cutis, also called cutaneous ossification, refers to the rare occurrence of bone in the skin. It may be primary, occurring in normal skin, or secondary, occurring in disrupted skin tissue. A 42-year-old white woman presented with long-standing progressive primary osteoma cutis involving her head and neck, trunk and extremities. She had craniofacial dysmorphism with mid-face hypoplasia, including saddle nose deformity, mild to moderate generalized joint hypermobility, extensive paravertebral ossification, and disc space calcification. The differential diagnosis for this entity is presented. This phenotype may be a previously undescribed syndrome.