Erythema Elevatum Diutinum

Abstract

A 57‐year‐old woman presented with symmetrical reddish‐brown plaques on the entire skin, especially the extremities. The lesions differed in size and shape, and their consistency ranged from soft to tight‐elastic, to hard in the older lesions (Fig. 1). The forearms and the dorsae of the hands and feet were covered with confluent plaques; some of these had an isolated nodular lesion on top. The lesions on the arms were papular‐nodular, arranged in rows to form “cords” with “pseudokeloid” appearance (Fig. 2). On the face, around the cheeks, the wings of the nose, and the ears, there were purplish nodular lesions and plaques. The lesions first appeared 14 years ago in the winter as lentile‐sized, slightly reddish papules on both legs. These lesions disappeared spontaneously during the summer and returned the following winter. For 4 years the lesions followed this pattern of appearing and disappearing, leaving residual pigmentation. The patient reported that at each recurrence the lesions were larger and darker. Subsequently, the lesions had become permanent without any improvement during the summer and had also spread to the upper limbs and trunk. For the past 4 years the patient has had arthralgia of the large joints, swelling of the lower extremities, burning and pain in the lesions themselves. These symptoms became more evident in the evening and after exposure to cold. A biopsy specimen of a recent lesion showed a dermal perivascular infiltrate comprised mainly of neutrophilic granulocytes and histiocytes and a few eosinophils. The vessels appeared dilated with swelling of the endothelial cells (Fig. 3). In and around the vessel walls an eosinophilic deposit of fibrinoid material was noted. Electronmicroscopic examination revealed macrophages with secondary lysosomes containing electrondense, osmiophilic, probably lipid material, in the perivascular infiltrate. lnterdigitate cells, juxtaposed with the lymphocytes among the infiltrate cells were also seen (Fig. 4). Direct immunofluorescence (DIF) revealed linear IgA deposits at the dermoepidermal junction. Direct immunofluorescence on healthy perilesional skin after histamine injection1 showed IgA, C3, and fibrinogen at the perivascular level in the superficial dermis and at the dermoepidermal junction with granular appearance. Cutaneous fibrinolytic activity (CFA) evaluated with Todd's autohistographic method1, modified by Lotti et al.2, was reduced by 80% compared to that of healthy subjects. Findings of other laboratory tests are listed in Table 1. Relevant investigations showed a decrease in fibrinogen, positive protein C and RA tests, the presence of cryoglobulin, and an increase in serum |gA.| Circulating immune complexes were detected and glucose‐6‐phosphate‐dehydrogenase (GG‐PD) was slightly low. The tests of clotting function showed some changes due to increased platelet aggregability from exogenous adenosine diphosphate (ADP), a slight reduc‐tion in fibrinogenemia due to the presence of D‐dimers and other products of fibrinogen degradation, suggesting a...

Dermatitis herpetiformis presenting as pseudovasculitis

“…Additionally, DIF showed linear IgA deposits. 10 , 11 Although the morphology and the distribution of our patient's cutaneous findings may be similar to that of EED, the lesions in EED tend to be asymptomatic unlike those in our patient. Further, the histopathologic and DIF findings argue against EED, as no such evidence of leukocytoclasis was found in our patient other than a nonspecific dermal hemorrhage.…”

Erythema elevatum diutinum and plastic surgery Case report

“…Other conditions reported to be associated include hyperimmunoglobulinemia D syndrome, cryoglobulinemia, myelodysplastic syndromes, abnormalities in the clotting mechanism, haematological malignancies, prostatic carcinoma, testicular lymphoma, viral and bacterial infections, rheumatic fever, HIV infection, inflammatory bowel disease, rheumatoid arthritis and peripheral ulcerative keratitis, relapsing polychondritis, Wegener's granulomatosis, and acro-osteolysis [1,9,10,16,18,23,24,37,39,40,41,42].…”

“…The treatment of choice in EED is dapsone [41], which suppresses the lesions in some way by stabilizing the neutrophil lysosomes, thereby breaking the cycle of continuing vascular damage [35]. Other modes of therapy with variable response include clofazimine, chloroquine, niacinamide, tetracycline, steroids, topical betamethazone, flucinolone under occlusion, phenformin, Colchicine and salfapyridine [10,11,13,19,23,34,37,41]. In one case of EED associated with celiac disease, while dapsone did not improve the skin manifestations, the lesions disappeared with a gluten-free diet [31].…”

Erythema elevatum diutinum - a chronic leukocytoclastic vasculitis microscopically indistinguishable from granuloma faciale?

“…For EED, the presence of an underlying disease can also be sought. Previous case reports have identified EED occurring in conjunction with hematological abnormalities, autoimmunity, HIV disease, other infectious diseases and minor impairments such as insect bites or folliculitis 1,2,9,15–25 . However, the role of associated medical problems is controversial.…”