Mild Perivascular Infiltrate Research Articles

CPC09 New-onset agminated Spitz naevi following hypopigmented pityriasis lichenoides chronica

Abstract A 9-year-old girl presented to dermatology with erythematous scaly patches affecting her legs and arms, and hypo­pigmented patches affecting the trunk. She was otherwise fit and well, with no preceding illness or new medications. Treatment with mometasone 0.1% and an emollient was unsuccessful. Two years later, she had continued to develop new hypopigmented patches, as well as dark clustered naevi on the trunk. An incisional biopsy was taken from a hypopigmented patch of the right abdomen and one of the new naevi from the right hip. Incisional biopsy of the hypo­pigmented patch showed scattered necrotic keratinocytes in the epidermis with mild hydropic degeneration of basal cells and a mild superficial and perivascular lymphohistiocytic infiltrate in the dermis, consistent with pityriasis lichenoides chronica (PLC). Incisional biopsy of an eruptive naevus showed a junctional proliferation of melanocytes with overlying epidermal hyperplasia and hyperkeratosis and transepidermal elimination of melanin. Nests of epithelioid melanocytes were present at the rete tips, with no pagetoid spread and no atypia, consistent with an eruptive Spitz naevus. PLC is a benign lymphoproliferative disorder, typically presenting with erythematous scaly papules that relapse and remit. However, cases of PLC presenting with hypopigmentation alone have also been reported, as in this case, which can be part of a postinflammatory dyspigmentation or active disease (Elbendary A, Abdel-Halim MR, Youssef R et al. Hypopigmented lesions in pityriasis lichenoides chronica patients: are they only post-inflammatory hypopigmentation? Aust J Dermatol 2021; 63:68–73). The clustered pattern of Spitz naevi in this patient is termed agminated Spitz naevus. Eruptive disseminated and agminated Spitz naevi are much rarer than the solitary presentation. Triggers of eruptive Spitz naevi include pregnancy, blistering disorders and topical tacrolimus, and in one case following pityriasis lichenoides et varioliformis acuta (Mazzurco J, Menssen K, Schapiro B et al. Eruptive disseminated Spitz nevi in a 26-year-old African-American woman. Int J Dermatol 2012; 51:1270–1), while agminated Spitz naevi are reported to occur after radiotherapy and sunburn, and to arise in naevus spilus or congenitally hypo- or hyperpigmented patches. No cases of malignancy in agminated or eruptive disseminated Spitz naevi have been reported. In this case, the new onset of agminated Spitz naevi may have been triggered by the inflammatory process of PLC. As far as we can ascertain, there have not been previous cases of this published in the literature. Our patient’s hypopigmented areas have now started to self-resolve and we have planned for ongoing monitoring of the agminated Spitz naevi.

Nailfold Videocapillaroscopic Alterations as Markers of Microangiopathy in COVID-19 Patients.

Nailfold videocapillaroscopic alterations have been described in COVID-19, but their correlations with biomarkers of inflammation, coagulation and endothelial perturbation are still unclear, and no information is available on nailfold histopathology. Nailfold videocapillaroscopy was performed on fifteen patients with COVID-19 in Milan, Italy and the signs of microangiopathy were correlated with plasma biomarkers of inflammation (C reactive protein [CRP], ferritin), coagulation (D-dimer, fibrinogen), endothelial perturbation (Von Willebrand factor [VWF]) and angiogenesis (vascular endothelial growth factor [VEGF]) along with genetic drivers of COVID-19 susceptibility. Histopathological analysis of autoptic nailfold excisions was performed on fifteen patients who died for COVID-19 in New Orleans, United States. All COVID-19 patients studied with videocapillaroscopy showed alterations rarely seen in healthy individuals consistent with microangiopathy, such as hemosiderin deposits (sign of microthrombosis and microhemorrhages) and enlarged loops (sign of endotheliopathy). The number of hemosiderin deposits correlated both with ferritin and CRP levels (r = 0.67, p = 0.008 for both) and the number of enlarged loops significantly correlated with the levels of VWF (r = 0.67, p = 0.006). Ferritin levels were higher in non-O groups, determined by the rs657152 C > A cluster, (median 619, min-max 551-3266 mg/dL) than in the O group (373, 44-581 mg/dL, p = 0.006). Nailfold histology revealed microvascular damage, i.e., mild perivascular lymphocyte and macrophage infiltration and microvascular ectasia in the dermal vessels of all cases, and microthrombi within vessels in five cases. Alterations in nailfold videocapillaroscopy and elevated biomarkers of endothelial perturbation that match histopathologic findings open new perspectives in the possibility of non-invasively demonstrating microangiopathy in COVID-19.

Preclinical safety study of local use of the directed ozone gas flow in abdominal surgery

Purpose of the study: to assess the patho-histological changes in the intact pancreas and parietal peritoneum against the background of the directed ozone gas flow.Material and methods. The prospective study was conducted in 54 adult male Wistar rats. In 1st and 2nd groups (n = 24, each) the left pancreatic lobe and parietal peritoneum, respectively, was treated with a 40 mg/L ozone gas stream at a rate of 2 L/min for 1.5 minutes. 1, 3, 30 and 120 days after the first operation 6 animals of each groups were sacrificed and tissue specimens were collected for histological analysis. In the control group (6 sham operated animals), tissue samples were collected 120 days after laparotomy.Results. A day after treatment with ozone gas stream, the pancreatic specimens of 1st group differed from the control in moderate and mild perivascular infiltration and edema. In the period from 3 to 120 days after exposure, the pancreatic specimens of 1st group and control did not differ significantly. Specimens of 2nd group differed from the control degree of edema and infiltration in the first 3 days, the level of proliferation of fibroblast-like cells and fibrosis in 120 days after treatment of the parietal peritoneum with a directed ozone gas stream.Conclusion. Local use of the directed ozone gas flow at a concentration of 40 mg/L at a rate of 2 L/min is accompanied by the same type of patho-histological changes from the intact pancreas and parietal peritoneum in the form of mild to moderate edema, perivascular polymorphic cell infiltration in the early period after exposure. Late parietal peritoneum reaction (moderate events fibroblast-like cell proliferation and fibrosis) requires additional safety studies of intraabdominal use of ozone-oxygen mixture.

Lichen planus with IgA vasculitis: a rare association

<p>Lichen planus is an inflammatory skin disorder which has been shown to be associated with thyroid disorders, hepatitis B, hepatitis C. IgA vasculitis is a form of cutaneous small vessel vasculitis (CSVV) characterized by palpable purpura, abdominal pain and joint pain usually seen in children. We report a case of 45-year-old female patient came with violaceous itchy lesions which were distributed over trunk and extremities of 1 month duration. Complete blood count (CBC) and serum biochemistry tests were normal. Histopathology of violaceous lesions showed changes of lichen planus. Purpuric lesions showed mild perivascular and transmural inflammatory infiltrate, composed mainly of neutrophils and few lymphocytes in the superficial dermis. Direct immunofluorescence was positive for IgA (granular pattern along with vessel walls of small vessels). Based on clinical, histopathological findings, a diagnosis of lichen planus with IgA vasculitis was made. The patient was treated with 30 mg prednisolone, gradually tapered over 6 weeks and anti-histamines. Satisfactory response was observed at follow-up at 6 weeks. Our case highlights the rare association of lichen planus with IgA vasculitis. Thorough evaluation of the patient helped in reaching correct diagnosis. </p>

Congenital Epidermodysplasia Verruciformis in Skin of Color: New Dermoscopic Features.

Sir, Epidermodysplasia verruciformis (EDV) is a rare inherited disorder associated with infection of human papillomavirus (HPV). Only a few authors have described the dermoscopic features in EDV.[123] We hereby describe newer dermoscopic features in a rare case of congenital EDV in an Indian family. A 24-year-old female presented to us with multiple white to reddish-brown coloured flat lesions on the trunk, and upper and lower limbs since birth. Her two children, that is, a six year old girl and four year old boy also had similar lesions. However, there was no similar history among the parents or siblings of the patient. Cutaneous examination revealed multiple well-defined erythematous to hypopigmented macules resembling pityriasis versicolor (PV) along with multiple well-defined, verrucous dark-brown coloured plaques of size approximately 1 × 1 cm2 on chest, abdomen, back, and all extremities [Figure 1a and b]. Other dermatological and systemic examination were normal. Complete blood count, serum biochemistry, and urine analysis were normal. Human immunodeficiency virus (HIV), Hepatitis B antigen (HbsAg), and anti-Hepatitis C antibodies (anti-HCV) serology were non-reactive.Figure 1: (a and b) Multiple well-defined hypopigmented (pityriasis versicolor-like) and dark brown keratotic lesionsDermoscopy was done using Dermlite DL-4 under 10× polarized mode. It revealed hypopigmented to erythematous background with red dots, white scaling, and slight crusting in hypopigmented/PV-like macules [Figure 2a]. Margins of most of the lesions had a characteristic continuous scaling [Figure 2a and b]. Verrucous plaques showed brownish-black areas admixed with hypopigmented areas [Figure 2c]. Histopathological examination of skin biopsy showed basket-weave hyperkeratosis, parakeratosis, and mild acanthosis of epidermis. Keratinocytes in the superficial layers were enlarged and swollen with abundant basophilic cytoplasm containing numerous round keratohyaline granules did not mention about vacuolation? Upper dermis showed mild perivascular lymphoplasmacytic infiltrate [Figure 2d]. A final diagnosis of EDV was made. The patient was started on oral isotretinoin.Figure 2: (a) Hypopigmented (pityriasis versicolor-like) lesions: Showing hypopigmented to erythematous background with red dots (orange arrow) and lesional white scales (green arrow) and continuous marginal scaling (black arrows). (b) Continuous marginal scaling (black arrows), brownish-black areas admixed with hypopigmented areas (pink arrows).(c) Admixture of hypopigmented areas with brownish-black areas (red circle). (Dermlite DL-4;10× polarised mode) (d) Basket weave hyperkeratosis and parakeratosis and enlarged keratinocytes with swollen abundant basophilic cytoplasm containing keratohyaline granules (H&E 40x)EDV is characterised by an impairment of cell-mediated immunity which results in disseminated skin eruptions by HPV clinically presenting with lesions resembling PV, verruca plana, and seborrheic keratosis (SK). There are very few reports of EDV, wherein dermoscopic features have been described.[123] Dermoscopy of lesions in EDV has shown demarcated hypopigmented to erythematous background with scaling, unfocused dotted vessels, and diluted hair pigment. SK-like lesions have demonstrated comedo-like openings with abrupt border.[12] Similar findings were seen in our patient except in any lesion, we did not observe dilution of hair pigment, comedo-like opening, or milia-like cysts. Additionally, we observed an admixture of brownish and hypopigmented background with brown globules and a characteristic marginal continuous scaling. These newer features observed in our patient might be due to different skin type and stages of the lesions than the previous reports. A similarity in dermoscopy of different lesions of EDV has been attributed to common etiology.[1] On dermoscopy, the admixture of different areas might also be due to the common etiology and it could be showing different stages of the EDV, which are not yet seen clinically. More such case reports and long-term follow-ups are needed to understand definitive dermoscopic features which would assist in the early diagnosis of malignant transformation. We hereby report this case due to its rarity and paucity of literature regarding its dermoscopic features. Declaration of patient consent The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed. Financial support and sponsorship Nil. Conflicts of interest There are no conflicts of interest. Acknowledgements Renuka Verma, M.D. Pathology, Assistant Professor, Department of Pathology, Pt. BD Sharma Postgraduate Institute of Medical Sciences, Rohtak, Haryana, India (For histopathology pictures).

Severe hand–foot skin reaction and erythema multiforme-like lesions due to sorafenib

A 55-year-old female patient with a history of metastatic renal cell carcinoma presented to us with erythematous, painful, mildly itchy lesions on both hands, arms and feet with impaired function....

Chronic Eosinophillic Leukemia presenting as Peripheral Neuropathy

Chronic Eosinophilic Leukemia (CEL) is a rare form of chronic myeloproliferative disorder of unknown etiology with no data on its true incidence. The disease has a wide variety of manifestations. Literature search has not shown peripheral neuropathy as the only presentation of CEL. Our case is probably the first such report case. Here we are reporting a 45 year old male patient who presented with progressive weakness of upper and lower limbs for 6 months and weight loss for 2 months. Neurological examination revealed findings consistent with bilateral sensorimotor polyneuropathy later confirmed by nerve conduction studies. Complete blood count revealed total wbc count – 51, 870/mm3, eosinophil – 62.1% (32,315/mm3). Peripheral Blood film revealed eosinophilic leucocytosis. Superficial peroneal nerve biopsy showed mild perivascular infiltration with inflammatory cells. No granuloma or malignancy was seen. Bone Marrow examination showed hyperactive granulopoiesis with predominance of eosinophil series with progressive maturation along with presence of myeolcytes, hypersegmented eosinophils and giant eosinophils. Blast cell was around 7%. Patient was treated with imatinib and prednisolone which showed excellent response.Bangladesh Crit Care J March 2018; 6(1): 47-49

Eruptive Facial Postinflammatory Lentigo: Clinical and Dermatoscopic Features.

The face has not been considered a common site of fixed drug eruption, and the authors lack dermatoscopic studies of this condition on the subject. The authors sought to characterize clinical and dermatoscopic features of 8 cases of an eruptive facial postinflammatory lentigo. The authors conducted a retrospective review of 8 cases with similar clinical and dermatoscopic findings seen from 2 medical centers in 2 countries during 2010-2014. A total of 8 patients (2 males and 6 females) with ages that ranged from 34 to 62 years (mean: 48) presented an abrupt onset of a single facial brown-pink macule, generally asymmetrical, with an average size of 1.9 cm. after ingestion of a nonsteroidal antiinflammatory drugs that lasted for several months. Dermatoscopy mainly showed a pseudonetwork or uniform areas of brown pigmentation, brown or blue-gray dots, red dots and/or telangiectatic vessels. In the epidermis, histopathology showed a mild hydropic degeneration and focal melanin hyperpigmentation. Melanin can be found freely in the dermis or laden in macrophages along with a mild perivascular mononuclear infiltrate. The authors describe eruptive facial postinflammatory lentigo as a new variant of a fixed drug eruption on the face.

The many masks of cutaneous Lyme disease.

Early cutaneous Lyme disease, erythema migrans, may show different histopathologic patterns. The intent of this case series is to raise awareness of these findings to prevent misdiagnosis and keep this entity in the differential. Erythema migrans develops after a tick bite and subsequent infection with the spirochete, Borrelia burgdorferi. It most commonly manifests as a solitary, annular lesion with a bull's-eye appearance. Classic histopathologic findings include superficial and deep perivascular and interstitial lymphocytic infiltrates mixed with plasma cells and eosinophils. We identified and reviewed eight cases of early erythema migrans. Each patient had confirmed B. burgdorferi IgM seropositivity and IgG seronegativity. Histopathologic evaluation of these biopsies reveals a diversity of patterns. Seven of eight cases show sparse to mild perivascular and interstitial mixed infiltrate of variable amount of lymphocytes, eosinophils, neutrophils and plasma cells, with only one case showing a dense inflammatory infiltrate. Epidermal changes such as spongiosis and interface change are seen in some cases. Additionally, perineural lymphocytic infiltrate is seen in one case, periadnexal infiltrate in four cases and pigment incontinence in one case. Based on variable histopathologic findings, it is important to consider erythema migrans in the differential diagnosis for prompt diagnosis and treatment.

Dysplastic Nevus with Eruptive Melanocytic Lesions That Developed during Nilotinib Therapy for Chronic Myeloid Leukemia.

Dear Editor: Eruptive melanocytic lesions (EMLs) have been associated with immunosuppressive conditions, immunodeficiency, and blistering diseases. Melanocytic lesions that suddenly develop after the administration of immunosuppressants indicate that the immune system may play an important role in limiting normal melanocyte proliferation1. The development of EML is probably mediated by the immunosuppressive action of drugs2. Biological drugs such as infliximab, alefacept, etanercept, and sorafenib have also been reported to be associated with EML1,3,4. We report a case of EML and a dysplastic nevus that developed in a patient with chronic myeloid leukemia (CML) after using nilotinib. A 39-year-old female patient was found to have CML after a routine health evaluation and began to take 400 mg nilotinib twice a day from 2008; she was still taking the medication at the time of the study. After several months, she gradually developed pigmented lesions on her face and trunk. During the next 4 years, the number of pigmented lesions increased, appearing throughout the whole body, including areas that are not exposed to sunlight. On examination, we found ≥100 pigmented lesions with diameters of 2~3 mm. In addition, 4 years after the initiation of therapy, she noticed a 4-mm dark papule on her right thigh, with itching. Dermoscopic examination of the papule on the thigh showed structureless pigmentation with asymmetric and irregular and fuzzy borders, and several blackish globules were found on one side of the periphery (Fig. 1A, B). Histopathological examination of the 3-mm, benign melanocytic lesion showed slightly elongated, clubbed rete ridges and intermittently distributed single melanocytes at the tip and side of the rete ridges without signs of dysplasia (Fig. 2A). In contrast, the atypical pigmented lesion showed elongated, fused rete ridges and contiguously distributed single melanocytes and a few small nests at the dermal-epidermal junction. In addition, eosinophilic fibroplasias and mild perivascular infiltrate were observed. The lesional melanocytes were observed to have moderately enlarged nuclei and slightly hyperchromatic nucleoli (Fig. 2B~D). Therefore, we established a diagnosis of dysplastic nevus with EML that developed during nilotinib therapy. We removed the dysplastic nevus through punch biopsy and the multiple melanocytic lesions with a 755-mm alexandrite laser. Fig. 1 Dermoscopic picture showing structureless pigmentation with asymmetric and irregular and fuzzy borders, and several blackish globules. Fig. 2 (A) Histopathological picture of the benign melanocytic lesion showing slightly elongated, clubbed rete ridges and intermittently distributed single melanocytes at the tip and side of the rete ridges. Lesional melanocytes did not show dysplasia (H&E, ... Nilotinib is a second-generation tyrosine kinase inhibitor with excellent efficacy for CML5. The effects of nilotinib as a KIT inhibitor were also reported to show a durable response in patients with metastatic melanoma harboring the KIT mutation5. However, there have been no clinical or laboratory reports of nilotinib affecting the proliferation of melanocytes or tumor development from a melanocyte origin. Nevertheless, the development of EML after nilotinib use may possibly be caused by interference with the functions that control the proliferation of melanocytes through similar mechanisms as those of sorafenib-3 or tumor necrosis factor-α inhibitor-induced EML1,4. Because the development of eruptive pigmented lesions has been related to immunosuppression, potentially diminished immune surveillance may allow melanocyte growth factors to the promote development of pigmented lesions3. To our knowledge, this is the first reported case of a dysplastic nevus with EML that developed during nilotinib therapy in a middle-aged woman with CML.

Clinical response to ustekinumab in familial pityriasis rubra pilaris caused by a novel mutation inCARD14

Funding sources: none. Conflicts of interest: none declared. Dear Editor, Pityriasis rubra pilaris (PRP) manifests with erythematous scaly plaques, typically involving the scalp, trunk and limbs, associated with a yellowish diffuse palmoplantar keratoderma (PPK).1 Familial PRP is a very rare entity, which is inherited as an autosomal dominant trait.1 Recently, gain‐of‐function mutations in CARD14, which encodes the caspase recruitment domain family member 14, were identified as the cause of familial PRP and found to be associated with increased expression of CARD14 in the epidermis of affected patients.2 CARD14 is a known activator of the noncanonical nuclear factor‐kappa B (NF‐κB) pathway, leading to the activation of the inflammatory response.3 Herein, we describe the beneficial effect of ustekinumab in a case of familial PRP caused by a novel mutation in CARD14 that was refractory to multiple other therapeutic approaches. A 57‐year‐old man was referred for investigation of near‐erythroderma. The patient had experienced erythematous scaly plaques over most parts of the body since childhood. His father, a younger sister and his daughter were similarly affected (Fig. 1a). Physical examination revealed a diffuse and symmetric rash, involving the trunk and limbs, consisting of large scaly plaques and distinct islands of sparing. PPK and follicular accentuation were also noted (Fig. 1b,c). The patient was obese. Initially, psoriasis was diagnosed, but the clinical findings were, upon reconsideration during subsequent visits, deemed more consistent with PRP. Histopathology was not conclusive, showing epithelial hyperplasia with alternating para‐ and orthohyperkeratosis, and a mild lymphohistiocytic perivascular infiltrate (not shown).

Itchy erythroderma in a neoplastic patient—mind the mite!

A 75 year-old Caucasianman presentedwith an itchy erythrodermic dermatosis showing scaling in the arms and in the gluteal region (Fig. 1A, upper panel). Hewasundergoing amultidrug therapywith ciprofloxacin and salmeterol for a BPCO exacerbation, timolol for a glaucoma, citalopram and lormetazepam for anxious-depressive symptoms, and aspirin for stroke prevention. He had been surgically treated 4 years before for a prostatic adenocarcinoma (pT2c N0 at staging) and was recently diagnosed with a superficially invasive transitional carcinoma of the bladder. Due to the worsening erythroderma, he underwent a skin biopsy with a clinical suspect of erythrodermic psoriasis or adverse drug reaction. On histology, the skin showed only a mild perivascular inflammatory infiltrate of the dermis. The patient was then treated with methylprednisolone without any improvement. Therefore, a second histological examination was performed with three punch biopsies on the scaly sites. On histology, many mite parts and eggs were identified in the stratum corneum on a spongiotic epidermal background (Fig. 1B, lower panel). In the dermis, a mild perivascular inflammatory infiltrate was present.

Idiopathic eruptive macular pigmentation

Idiopathic eruptive macular pigmentation (IEMP) is an uncommon, rather under-reported disease entity of the pediatric age characterized by asymptomatic, brownish hyperpigmented macules involving the neck and trunk with no preceding inflammation or exposure to drug. Here we report this case to increase awareness of this entity among dermatologists, dermatopathologists and pediatricians. A 11-year-old healthy boy presented with brownish-gray to dark, discrete, asymptomatic macules on the face, trunk, neck and limbs of insidious onset. Histopathologic examination showed melanophages, mild perivascular lymphohistiocytic infiltrate in the papillary dermis and papillomatosis. The natural course of the disease is spontaneous remission without treatment, which was so even in our patient. IEMP is a benign entity with an excellent prognosis as it exhibits spontaneous resolution. It falls into the differential diagnoses of asymptomatic hyperpigmentatory disorders in pediatric population. Awareness of the entity leads to avoidance of unnecessary aggressive damaging treatment.

Sudden Infant Death in an 8-Month-Old Baby With Dengue Virus Infection

Sudden infant death due to dengue virus infection is very rare. An 8-month-old male infant was found unresponsive during a nap in his nursery school. We emphasize the usefulness of dengue viral antigens as a postmortem diagnostic technique to demonstrate the presence of virus in human tissue specimens by immunohistochemistry and Western blotting.

Erythema Multiforme after Radiotherapy with 5-fluorouracil Chemotherapy in a Rectal Cancer Patient

Erythema Multiforme after Radiotherapy with 5-fluorouracil Chemotherapy in a Rectal Cancer Patient

Hyperimmunoglobulinemia D Syndrome Variant — Seek and You Shall Find

80% neutrophils, elevated C-reactive protein (CRP) 222 mg/dl (nl 0–6), positive antinuclear antibody with nucleolar pattern, positive c-antineu trophil cytoplasmic autoantibodies and IgG anticardiolipin antibody 20 IU (normal to 10). Urinalysis and complement concentrations were normal. Viral and bacterial serologies were negative, with cultures sterile. Computed tomography of neck, lungs, and abdomen revealed only the cer vical lymphadenopathy and hepatosplenomegaly. A skin biopsy of the rash showed mild perivascular mononuclear infiltrate of the superficial dermis, with no evidence of vasculitis. The patient was treated with diclofenac, with gradual resolution of the signs — including fever, rash, and lym phadenopathy — within 7 days. Review of his past medical records at the hospital revealed 8 admissions since the age of 19 years with episodes of abdominal pain, rash, diarrhea, pleuritis, and lymphadenopathy. A previous lymph node biopsy at age 19 was consistent with a reactive pattern. A bone marrow biopsy was normal, and a previous skin biopsy showed leukocyto clastic vasculitis. In assessing him for various periodic syndromes, his immunoglobulin levels were measured and elevated IgD, 150 mg/ml (nor mal range 0–8 mg/ml), and IgA, 7.54 mg/ml (normal range 0.9–3.2 mg/ml) were noted. Gene mutation analysis of MVK revealed a single V377I muta tion in exon 11; MEFV and TNF receptor mutations were absent. Urine mevalonic acid was undetectable during the attack.

Fibroblastic rheumatism: fibromatosis rather than non‐Langerhans cell histiocytosis

Fibroblastic rheumatism is a unique fibro-proliferative disease affecting the skin and joints. It is characterized by distinctive clinical and histological features related to benign spindle-shaped cells proliferation. Pediatric reports are scarce in the literature. We describe here a new case in a 10-year-old boy and discuss the potential origin of the cell proliferation. Clinical findings, radiology, microscopic examination and outcome are reviewed. Histopathology and immunochemistry studies were performed on skin biospies using CD68, CD163, desmin, factor XIIIa, CD34, smooth muscle actin, PS100, epithelial membrane antigen, and calponin. Histological sections disclosed a rather circumscribed nonencapsulated nodular infiltrate, invading the dermis and the upper subcutaneous tissue, consisted of a proliferation of spindle or stellate-shaped cells and thickened collagen fibers. Orcein staining showed disappearance of the elastic network. Aponeurosis and muscle were normal. A mild perivascular lymphohistiocytic infiltrate was noted. Calponin-staining was less strongly expressed as SMA, and some of them but not all were CD68 positive, as well. On the other hand, all were CD34, CD163, FXIIIa, PS100, EMA and desmin-negative. The true origin of these cells remains unclear. Some authors have speculated a histiocytic origin. However, immuno-chemical staining in our case failed to confirm this hypothesis and instead supported a fibroblastic/myofibroblastic origin. Given the clinical course and the histological and immunohistochemical results, we suggest that FR should be added to the group of fibromatoses.

T helper17 Cells Are Sufficient But Not Necessary to Induce Acute Graft-Versus-Host Disease

T helper (Th)1 cells were considered responsible for the induction of graft-versus-host disease (GVHD), but recently the concept has been challenged. Th17 cells play a critical role in mediating autoimmune diseases, but their role in the pathogenesis of GVHD remains unclear. Herein we compare the ability of in vitro generated Th1 and Th17 cells from C57BL/6 mice to induce GVHD in lethally irradiated BALB/c recipients. Allogeneic Th17 cells had superior expansion and infiltration capabilities in GVHD target organs, which correlated with their increased pathogenicity when compared with naïve or Th1 controls. Th17 cells caused no pathology in the syngeneic recipients, indicating that antigen-activation was required for their pathogenicity. Polarized Th17 cells could not maintain their phenotype in vivo as they produced a significant amount of interferon (IFN)-gamma after being transplanted into allogeneic recipients; however, IFN-gamma was not required for Th17 cell-induced GVHD. Further, we evaluated the pathogenesis of Th17 cells in GVHD by using polyclonal nonprimed CD4T cells in a clinically relevant allogeneic bone marrow transplantation (BMT) setting. We found that disruption of Th17-differentiation alone by targeting RORgammat (Th17-specific transcription factor) had no significant effect on GVHD development. We conclude that Th17 cells are sufficient but not necessary to induce GVHD.

Cutaneous Reactive Angiomatosis Associated With Chronic Lymphoid Leukemia

Cutaneous reactive angiomatosis is an unusual benign vascular disorder of the skin usually associated to systemic diseases. It is characterized by lobular or diffuse proliferation of small blood vessels with hyperplasia of endothelial cells, pericytes, and sometimes histiocytes. We report a 59-year-old man with asymptomatic erythematous-violaceous patches on back, palms, and elbows for 9 months. Laboratory examination revealed changes consistent with B-chronic lymphocytic leukemia. Cutaneous biopsy showed a predominantly lobular small blood vessel proliferation in dermis with pericytic hyperplasia and mild perivascular lymphoplasmacytic infiltrate. Spontaneous involution of lesions occurred after 6 months. A second biopsy performed at the beginning of clinical involution showed a less prominent vascular component with perivascular giant cells with coexpression of CD68 and CD 31. To our knowledge, this is the first case of cutaneous reactive angiomatosis with documented histopathological findings of clinical involution.

Sudden onset weakness in an immunocompromised patient

Toxoplasmosis is an infection caused by the intracellular parasite Toxoplasma gondii. Human infection usually occurs via an oral or transplacental route. In adults, most infections are subclinical, but severe infection can occur in patients who are immunocompromised, particularly those with AIDS or a history of transplantation. In our patient, a steroid-induced immunosuppression was likely to blame for her susceptibility to the pathogen. Imaging with a non-contrast CT scan often depicts an isodense lesion with surrounding edema and mass effect. On MRI, T2-weighted FLAIR images reveal a hyperintense signal consistent with the underlying mass and edema. On T1-weighted MRI the lesion is often hypo/isodense to gray matter with ring enhancement following the administration of contrast. Diffusion-weighted images often show restricted diffusion (Fig. 1). Histology demonstrated an area of necrosis with scattered cysts (arrow) containing basophilic bradyzoites consistent with toxoplasma micro-organisms (Fig. 2). In our patient, a mild perivascular lymphoplasmacytic infiltrate with scattered cells positive for Epstein-Barr virus (EBV) was present raising the possibility of an early EBV-associated lymphoproliferative disorder. The primary treatment consists of a regimen of pyrimethamine and sulfadiazine.