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Primary cutaneous malignant perivascular epithelioid cell tumor: Case of a rare tumor with review of the literature.
Cole, DW, Menge, TD, Renati, S, Bresler, SC, Patel, RM, Fullen, DR, Hamp, LM
Journal of cutaneous pathology. 2021;(8):1088-1093
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Abstract
Perivascular epithelioid cell tumors (PEComas) are mesenchymal neoplasms with characteristic epithelioid or spindled cytomorphology that typically grow around blood vessels. These tumors are phenotypically and immunohistochemically distinct, expressing markers of both melanocytic and smooth muscle differentiation. Herein, we describe a case of histopathologically malignant cutaneous PEComa without metastatic spread, with review of the pertinent literature. Telescoping punch biopsy demonstrated an epithelioid neoplasm with marked atypia, hypercellularity, and increased mitotic activity. Immunohistochemical stains for HMB-45, NK1-C3, PGP9.5, MiTF, CD10, and CD68 were positive within tumor cells. In addition, there was diffuse expression of caldesmon and focal cytoplasmic staining for smooth muscle actin on the excision specimen. The patient underwent treatment with surgical excision with adjuvant radiation and surveillance computed tomography (CT). The patient remains free of recurrence or metastatic disease after 10 months of follow-up. To our knowledge, this is only the third reported case of a malignant cutaneous PEComa reported in the literature to date.
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Pseudoatrophoderma colli: distinct entity or just a variant of confluent and reticular papilomatosis of Gougerot-Carteaud.
Sudy, E, Urbina, F, Gubelin, W, Misad, C, Espinoza, A
Dermatology online journal. 2020;(10)
Abstract
Pseudoatrophoderma colli is a rare entity described in the same time period as confluent and reticular papillomatosis of Gougerot-Carteaud and the two conditions have certain similarities. Pseudoatrophoderma colli is clinically characterized by lesions with an atrophic and wrinkled appearance, which are mainly located on the trunk and neck (hence the name colli). Few references exist in the literature and most of them are very old. Histopathological findings are nonspecific, showing mild hyperkeratosis, thinning of the stratum granulosum, and acanthosis and papillomatosis in some areas. In the papillary dermis there is vascular dilatation with a sparse inflammatory lymphohistiocytic perivascular infiltrate. Fragmentation of elastic tissue has been described only in one case. There is no specific treatment, with variable responses to diverse therapies including ultraviolet light, vitamin A, lactic acid and minocycline. We describe in detail two patients with pseudoatrophoderma colli and show histology. The first patient was treated with minocycline 100mg per day for two months and the second patient was treated with lymecycline 600mg per day for three months and 300mg per day for another two months. Both patients demonstrated a good response within the first month of treatment.
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Two new cases of aquagenic wrinkling of the palms and literature review on drug interactions.
Bouwman, K, Menichino, S, Kruithof, I, Aalfs, AS
Dermatology online journal. 2020;(11)
Abstract
Aquagenic wrinkling of the palms (AWP) is a rare, acquired condition of the skin, defined by transient rapidly developing white to translucent papules on palms and/or soles after brief exposure to water. Aquagenic wrinkling of the palms is associated with cystic fibrosis (CF). Therefore, the diagnosis of AWP can be important. Etiopathogenesis of AWP is still unclear. Treatment is often unsatisfactory and can be very challenging. This article contributes to the knowledge of AWP as we describe two new cases of aquagenic wrinkling of the palms: one patient with familial history of CF and one patient with AWP that was presumed to be induced by use of non-steroidal anti-inflammatory drugs. In addition, we present a review of the literature on drug-induced AWP.
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Facial Involvement in Dermatitis Herpetiformis: A Case Report and Review of the Literature.
Cinats, AK, Parsons, LM, Haber, RM
Journal of cutaneous medicine and surgery. 2019;(1):35-37
Abstract
Dermatitis herpetiformis is a cutaneous manifestation of celiac disease that classically presents as a symmetric pruritic vesicular eruption on extensor surfaces. Typical locations include elbows, knees, and buttocks. Facial involvement has been reported rarely. Here, we report a case of a 44-year-old woman with dermatitis herpetiformis presenting as pruritic vesicles on the face that had previously been misdiagnosed as allergic contact dermatitis. Diagnosis was confirmed with direct immunofluorescence demonstrating granular IgA in the papillary dermis. This eruption cleared with topical dapsone 5% gel and a gluten-free diet. We report this case to raise awareness of facial involvement in dermatitis herpetiformis as well as the possibility of topical dapsone as a therapeutic option.
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Paraneoplastic pityriasis rubra pilaris: case report and literature review.
Bar-Ilan, E, Gat, A, Sprecher, E, Zeeli, T
Clinical and experimental dermatology. 2017;(1):54-57
Abstract
Pityriasis rubra pilaris (PRP; MIM 173200) is an uncommon papulosquamous inflammatory dermatosis. Only a few cases of PRP associated with an underlying malignancy have been documented. We investigated a 59-year-old patient presenting with a fulminant form of PRP recalcitrant to systemic retinoid therapy, in whom the skin disease heralded a diagnosis of cholangiocarcinoma. We searched the MEDLINE database to find articles reporting on similar associations of PRP with malignancies. We identified 10 studies linking PRP and malignancies, but an association between PRP and cholangiocarcinoma has not yet been reported.
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6.
An unusual presentation of eruptive xanthoma: A case report and literature review.
Kashif, M, Kumar, H, Khaja, M
Medicine. 2016;(37):e4866
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Abstract
BACKGROUND Eruptive xanthomas are benign skin lesions caused by localized deposition of lipids in the dermis. The lesions are generally caused by elevated levels of serum triglycerides that leak through the capillaries and are phagocytosed by macrophages in the dermis. Clinical manifestation varies from asymptomatic skin lesions to intense pruritus and tenderness. METHODS We present a case of a middle-aged man admitted with diabetic ketoacidosis secondary to noncompliance with insulin. He was found to have skin lesions as multiple crusted papules on the extremities. Further evaluation revealed elevated serum triglycerides. A diagnosis of eruptive xanthomas was made on skin biopsy, and after starting treatment with lipid lowering agents his cutaneous lesions gradually subsided. CONCLUSION Appearance of eruptive xanthomas can signify the onset of serious complications. Prompt recognition of such skin manifestations is warranted to prevent development of fatal medical condition like coronary artery disease and pancreatitis.
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Calcinosis cutis presenting in the context of long-term therapy for chronic myeloid leukemia: a case report and review of the literature.
Altman, I, Lee, IH, Burns, MR, Rondelli, D, Ennis, WJ
Wounds : a compendium of clinical research and practice. 2015;(2):20-5
Abstract
Calcinosis cutis is a poorly understood process in which calcium salts deposit in the skin and subcutaneous tissues. Due to its multifactorial pathogenesis, several subtypes and potential etiologies have been described. Presented here is a case of bilateral pretibial calcinosis cutis in a patient on long-term tyrosine kinase inhibitor therapy for chronic myeloid leukemia. The patient initially presented with a right tibial ulceration treated with multiple surgical debridements, antibiotics, and negative pressure wound therapy. The wound was ultimately closed with a split-thickness skin graft. Relevant literature is examined and several possible mechanisms are discussed.
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Fixed drug eruption to quinine: a case report and review of the literature.
Genest, G, Thomson, DM
The journal of allergy and clinical immunology. In practice. 2014;(4):469-70
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9.
Multiple lesions of cutaneous focal mucinosis in a teenager.
Hernandez, C, Fowler, A, Gandhi, MY
Skinmed. 2007;(6):301-3
Abstract
A 19-year-old Hispanic woman presented to our clinic with a chief complaint of multiple pink papules on her face (Figure 1). The lesions were asymptomatic, had appeared several years before her consultation, and had remained stable in size. Similar lesions on her trunk and extremities had spontaneously resolved before presentation. She had no significant medical history and was not on any medications. Cutaneous examination was remarkable for a few scattered, well-demarcated, slightly raised pink papules and plaques with no scale, ranging from 0.5 to 1 cm in diameter. The lesions were distributed on the right cheek, right temple, left lower eyelid, and neck area. The rest of the cutaneous examination was unremarkable. A punch biopsy was performed from a lesion near the left premandibular area. Hematoxylin and eosin staining of the biopsy specimen revealed superficial perivascular dermatitis with a slight increase in the number of fibroblasts (Figure 2). Colloidal iron staining was diffusely positive with replacement of collagen with mucinous material in the dermal layer (Figure 3). Laboratory values for a complete blood cell count, metabolic panel, serum protein electrophoresis, urine protein electrophoresis, thyroid-stimulating hormone, and antinuclear antibody were within normal limits. The patient was diagnosed with cutaneous focal mucinosis. Because of cosmetic concerns, the patient declined surgical excision and intralesional corticosteroid injections. A 4-month trial of topical tacrolimus 0.1% ointment was ineffective.
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10.
Immunohistochemical investigations and introduction of new therapeutic strategies in scleromyxoedema: case report.
Breuckmann, F, Freitag, M, Rotterdam, S, Stuecker, M, Altmeyer, P, Kreuter, A
BMC dermatology. 2004;(1):12
Abstract
BACKGROUND Scleromyxoedema is a rare chronic skin disease of obscure origin, which may often be associated with severe internal co-morbidity. Even though different casuistic treatment modalities have been described, to date, curing still seems to be impossible. CASE PRESENTATION We report a 44-year-old Caucasian female presenting with remarkable circumscribed, erythematous to skin-coloured, indurated skin eruptions at the forehead, arms, shoulders, legs and the gluteal region. Routine histology and Alcian blue labelling confirmed a massive deposition of acid mucopolysaccharides. Immunohistochemical investigations revealed proliferating fibroblasts and a discrete lymphocytic infiltration as well as increased dermal expression of MIB-1+ and anti-mastcell-tryptase+ cells. Bone marrow biopsies confirmed a monoclonal gammopathy of undetermined significance without morphological characteristics of plasmocytoma; immunofixation unveiled the presence of IgG-kappa paraproteins. CONCLUSIONS Taking all data into account, our patient exhibited a complex form of lichen mxyoedematosus, which could most likely be linked a variant of scleromyxoedema. Experimental treatment with methotrexate resulted in a stabilisation of clinical symptoms but no improvement after five months of therapy. A subsequent therapeutic attempt by the use of medium-dose ultraviolet A1 cold-light photomonotherapy led to a further stabilisation of clinical symptoms, but could not induce a sustained amelioration of skin condition.