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Anti-Phospholipid Antibodies: Clinical Complications Reported in Medical Literature
Published in E. Nigel Harris, Thomas Exner, Graham R. V. Hughes, Ronald A. Asherson, Phospholipid-Binding Antibodies, 2020
Degos’ Disease: This rare multisystem vasculopathy, is characterized by widespread thrombosis particularly of the skin, gastrointestinal tract and central nervous system and may result in death because of the effects of cerebral or bowel infarcts. A patient with Degos’ Disease who demonstrated typical painless papules and who also had high titers of anticardiolipin (aCL) antibodies and “lupus anticoagulant” activity was documented by Englert et al.21 and its relationship with aPL antibodies commented on by Burton in a recent review.22
Emergencies in systemic lupus erythematosus
Published in Biju Vasudevan, Rajesh Verma, Dermatological Emergencies, 2019
Vishalakshi Viswanath, Rashmi Modak
Vasculopathy presenting as Raynaud phenomenon, livedo reticularis, livedoid vasculopathy (Figure 40.8), ulcerated purpuric plaques, ecchymosis, digital gangrene (Figure 40.9), and thrombophlebitis may be seen as the clinical presentation of SLE in the ED. Raynaud phenomenon with impending gangrene is common in SLE patients in the presence of periungual telangiectasias, ice-pick or pitted scarring of pulps of fingers, and high ANA, anti-RNP, and nucleolar antibodies [6]. Livedo reticularis is seen as mottled bluish-red discoloration, blanching on pressure, distributed over buttocks, legs, and the outer aspect of the arms. In patients with SLE and APS, the appearance of livedo reticularis may herald central nervous system (CNS) involvement [6]. The patient may present to the ED with atrophie-blanche-like lesions, including painful, stellate white scars seen over the lower extremities and Degos disease-like lesions (white atrophic macules with peripheral erythema) [6]. Erythromelalgia, which is characterized by burning pain and erythema of hands and feet, increasing on dependence and exposure to heat, may be seen due to microvascular arteriovenous shunting in SLE patients with or without APS. Purpura fulminans may also be an atypical manifestation in SLE along with APS [16].
Hyperpigmented dermatoses
Published in Aimilios Lallas, Enzo Errichetti, Dimitrios Ioannides, Dermoscopy in General Dermatology, 2018
Enzo Errichetti, Aimilios Lallas
Few studies have reported the dermoscopy aspects of Dowling–Degos disease.6,10,11 Dermoscopy of hyperpigmented macules reveals the presence of irregular brownish projections sometimes delimiting small, roundish, hypopigmented areas10–12 (Figures 7.5B and 7.6B); these findings histopathologically correlate with an antler-like branching of rete ridges with basal hyperpigmentation.10 In papular lesions, dermoscopic examination shows a star-like brown pattern, which has been related to follicular plugging, on a reddish-brown background.11 The dermoscopy of hypopigmented lesions reveals an accentuation of the normal reticular pattern around the hypopigmented center, which histopathologically correlate with epidermal atrophy and lack of pigment.10
An update on the pathogenesis of hidradenitis suppurativa: implications for therapy
Published in Expert Review of Clinical Immunology, 2018
Deborah Negus, Christine Ahn, William Huang
HS has also been described in association with several clinical syndromes. The first disease that was described with HS as part of the syndrome is the follicular occlusion triad, which includes HS, acne, and dissecting cellulitis. With the presence of pilonidal cysts, this disease has been expanded to the follicular occlusion tetrad. Several other syndromes featuring HS have been described, including pyogenic arthritis, pyoderma gangrenosum (PG), and acne (PAPA), PG, acne, and suppurative hidradenitis (PASH), pyogenic arthritis plus the features of PASH (PAPASH), and psoriatic arthritis plus the features of PASH (PsAPASH) [24]. In addition, HS can be seen as a minor feature in other syndromes, including Bazex-Dupré-Christol syndrome, Down’s syndrome, SAPHO syndrome, and Dowling–Degos disease [4].