Background Histiocytoid Sweet syndrome (HSS) is a rare variant of Sweet syndrome (SS). The nature of histiocytoid cells is still uncertain. Objective We sought to offer a comprehensive overview on ...clinical features of HSS and further information on immunohistochemical phenotype of the infiltrate. Methods The clinical, histologic, and immunohistochemical features of 12 of our patients with HSS and all cases retrieved through a PubMed search were analyzed. Results Lesions consisted of erythematous-violaceous papules and plaques, randomly distributed mostly on the trunk and the limbs. Three patients had myelodysplastic syndrome and 1 had a monoclonal gammopathy. The infiltrate was mainly composed of CD68+ CD163+ myeloperoxidase+ myeloid cell nuclear differentiation antigen+ CD117− CD15− CD34− , a phenotype suggestive of M2-like macrophages. A few mature neutrophils and lymphocytes were also present. Review of all HSS cases showed no sex predominance and no extracutaneous infiltrates; inconstant presence of fever and blood neutrophilia; association with hematologic or solid neoplasms (26%), autoimmune conditions (12%), and infectious diseases (10%); and good response to steroid treatment, with rare relapses or recurrences. Limitations The study includes a limited case series. The pathogenesis of the disease remains to be clarified. Conclusions HSS lesions are infiltrated mostly by M2-like macrophages. The clinical features present more similarities than differences with SS.
The first case was a 46-year-old woman who presented with a 10-year history of recurrent pustular eruption on an erythematous base on the neck, axillae, submammary folds, lip commissures, pubis, and ...scalp Figure 1a and Figure 1b. Histological examination showed hyperplasia with focal parakeratosis, mild exocytosis of neutrophils, spongiform pustules in the epidermis, and a scant, perivascular neutrophilic infiltrate in the superficial and mid dermis. The third case was a 44-year-old Hispanic woman affected by systemic lupus erythematosus (SLE) since the age of 18 years and with a 3-year history of recurrent erosions and nonfollicular pustules involving the intergluteal and submammary folds, axillae, genital area, retroauricular groove, and occipital region of the scalp Figure 1c and d. The upper dermis showed a mixed neutrophilic and lymphocytic infiltrate with perivascular and interstitial distribution Figure 1e. The patient was treated with oral corticosteroids (1 mg/kg/day) and topical mometasone furoate with clinical benefit after 2 weeks and total remission after 10 weeks.
Alopecic and aseptic nodule of the scalp/Pseudocyst of the scalp is a rare but probably underdiagnosed nonscarring alopecia with good prognosis and doxycycline is a safe and effective option ...treatment.
Alopecic and aseptic nodule of the scalp/Pseudocyst of the scalp is a rare but probably underdiagnosed nonscarring alopecia with good prognosis and doxycycline is a safe and effective option treatment.
Secreted Frizzled Receptor Protein 4 (SFRP4) has been shown to be increased in Scleroderma (SSc). To determine its role in immune-driven fibrosis, we analysed SSc and sclerotic Chronic Graft Versus ...Host Disease (sclGVHD) biosamples; skin biopsies (
= 24) from chronic GVHD patients (8 with and 5 without sclGVHD), 8 from SSc and 3 healthy controls (HC) were analysed by immunofluorescence (IF) and SSc patient sera (
= 77) assessed by ELISA. Epithelial cell lines used for in vitro Epithelial-Mesenchymal-Transition (EMT) assays and analysed by Western Blot, RT-PCR and immunofluorescence. SclGVHD skin biopsies resembled pathologic features of SSc. IF of fibrotic skin biopsies indicated the major source of SFRP4 expression were dermal fibroblasts, melanocytes and vimentin positive/caveolin-1 negative cells in the basal layer of the epidermis. In vitro studies showed increased vimentin and SFRP4 expression accompanied with decreased caveolin-1 expression during TGFβ-induced EMT. Additionally, SFRP4 serum concentration correlated with severity of lung and skin fibrosis in SSc. In conclusion, SFRP4 expression is increased during skin fibrosis in two different immune-driven conditions, and during an in vitro EMT model. Its serum levels correlate with skin and lung fibrosis in SSc and may function as biomarker of EMT. Further studies are warranted to elucidate the role of SFRP4 in EMT within the pathogenesis of tissue fibrosis.
Pilomatricoma is a relatively common benign cutaneous adnexal tumor and a well-recognized entity, while its pigmented variant is far less common and less reported. Its estimated frequency ranges from ...11 to 24%, according to a limited number of published case series. This article describes the case of a 42-year-old man presenting a firm subcutaneous nodule of the periareolar region. Histopathologic examination revealed a cystic lesion composed of matrical and supramatrical cells accompanied by a foreign body granulomatous cell reaction. Interestingly, a hyperpigmented area with numerous hyperplastic melanocytes and few mitoses was detectable. In order to assess the cell lineage of the mitotically active component in the hyperpigmented area, double immunohistochemistry with Ki67/Mart1 and p63/SOX10 was performed. Pigmented pilomatricoma is an underrecognized, underreported variant, and double immunohistochemistry stain is an effective tool in providing the correct interpretation of the proliferative activity in the different cellular populations.
Drug-induced scleroderma has been rarely reported, mostly with the features of diffuse scleroderma or acrosclerosis, and exceptionally with the characteristics of morphea. We report the case of an ...adult white woman, enrolled in a double-blind, placebo-controlled, multicentric trial evaluating the efficacy and safety of the cathepsin K inhibitor balicatib for osteoporosis. Typical morphea lesions developed on the patient's trunk 9 months after the beginning of therapy. Lesions completely resolved after drug withdrawal and a single brief course of systemic steroids. No recurrence occurred in a 2-year follow-up. Fifteen cases of drug-induced morphea could be retrieved from the literature. Drug withdrawal determined complete remission in only a few patients. Different drug classes have been implicated. Some of these, including balicatib, alter directly connective tissue metabolism.
The role of complement system in the pathogenesis of systemic sclerosis (SSc) has been debated during the last decade but an evident implication in this disease has never been found. We carried out ...an explorative study on SSc patients to evaluate the expression of soluble and local C5b-9 complement complex and its relation with a complement regulator, the Membrane Cofactor Protein (MCP, CD46) on skin vascular bed as target distinctive of SSc disease. We also analyzed two polymorphic variants in the complement activation gene cluster involving the MCP region.
C5b-9 plasma levels of SSc patients and healthy subjects were analyzed by ELISA assay. Archival skin biopsies of SSc patients and controls were subjected to immunofluorescence analysis to detect C5b-9 and MCP on vascular endothelial cells. The expression of MCP was validated by immunoblot analysis with specific antibody. Polymorphic variants in the MCP gene promoter were tested by a quantitative PCR technique-based allelic discrimination method.
Even though circulating levels of C5b-9 did not differ between SSc and controls, C5b-9 deposition was detected in skin biopsies of SSc patients but not in healthy subjects. MCP was significantly lower in skin vessels of SSc patients than in healthy controls and was associated with the over-expression of two polymorphic variants in the MCP gene promoter, which has been related to more aggressive phenotypes in other immune-mediated diseases.
Our results firsty document the local complement activation with an abnormal expression of MCP in skin vessels of SSc patients, suggesting that a subset of SSc patients might be exposed to more severe organ complications and clinical evolution due to abnormal local complement activation.
Acute urticaria is self-limiting, and a cause can be identified in many patients. Chronic urticaria is a long lasting disease, and patients are commonly examined for an autoimmune origin and for ...associated diseases. Although the diagnosis of urticaria is straightforward in most patients, it may pose some difficulties at times and it may require a careful differential diagnosis with a number of conditions. Urticarial syndromes comprise both cutaneous and systemic disorders. Part I of this two-part series focuses on the clinical and histologic features that characterize common urticaria and on the cutaneous diseases that may manifest with urticarial lesions and must be considered in the differential diagnosis. Learning objectives After completing the learning activity, participants should be able to distinguish between the typical wheals of urticaria and urticarial lesions suggesting other diagnoses and to assess patients with urticarial lesions in order to exclude or confirm other cutaneous diseases.
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