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Erythema Elevatum Diutinum Appearing Within Old Scar Tissue

2021; Lippincott Williams & Wilkins; Volume: 43; Issue: 10 Linguagem: Inglês

10.1097/dad.0000000000002042

1533-0311

Rita Bouceiro-Mendes, Pedro Garrido, Maria Sanches, Heinz Kutzner, Luís Soares‐de‐Almeida,

Hidradenitis Suppurativa and Treatments

To the Editor: Erythema elevatum diutinum (EED) is a rare chronic cutaneous leukocytoclastic vasculitis (LCV) belonging to the group of neutrophilic dermatoses. Although it can be an idiopathic vasculitis, in some cases it occurs in the context of an underlying systemic disease. As such, these patients must be carefully evaluated to exclude associated pathology. EED usually presents as symmetrical distributed red–brown nodules, papules, or plaques commonly affecting extensor joint surfaces of the limbs.1–4 Atypical presentations have been described.2,4–6 To the best of our knowledge, EED presenting within areas of scar tissue has never been reported, and this finding may bring some light into the pathophysiology of this rare and misunderstood disease. A 30-year-old Brazilian man with a 20-year history of hypertrophic burn scars on the neck, trunk, and limbs (secondary to an accident with boiling water) was seen in our outpatient department with a 3-day history of burning sensation and erythema on his old scars. The patient also reported an episode of sore throat 4 days before the onset of the skin lesions, which had spontaneously resolved. Physical examination revealed tender red plaques presenting exclusively on the burn scars (Fig. 1). The patient was otherwise fit and well. Laboratory investigations were all within the normal range except for a borderline antistreptolysin O titer (200 U/mL, N < 200) and a slightly elevated angiotensin-converting enzyme (54 IU/L, N < 52). Notably, full-blood count, complement, immunoglobulins, electrophoresis, and autoimmune profile were within the normal range. HIV and hepatitis B and C serology results were negative. Chest radiography was unremarkable. Biopsy specimens revealed an acute stage of LCV with dilated postcapillary venules in all layers of the dermis surrounded by a dense perivascular neutrophilic infiltrate (positive immunostaining for Myeloid Cell Nuclear Differentiation Antigen (MNDA) and myeloperoxidase) with lots of perivascular extravasated erythrocytes (glycophorin A–positive staining) and conspicuous karyorrhexis in a fibrotic dermal stroma (Fig. 2). These findings were consistent with the diagnosis of an acute stage of EED within old scar tissue. The patient was treated with betamethasone dipropionate cream 0.05% twice a day and oral prednisolone (0.5 mg/kg/d) for 1 week with complete clinical resolution. He remains well at the 6-month follow-up.FIGURE 1.: Erythema elevatum diutinum, physical examination: Tender red plaques presenting exclusively within burn scars with the involvement of the trunk, upper limbs (A), and lower limbs (B and C).FIGURE 2.: Erythema elevatum diutinum, histopathological image: Acute stage of LCV with dilated postcapillary venules in all layers of the dermis surrounded by a dense perivascular neutrophilic infiltrate, with lots of perivascular extravasated erythrocytes and conspicuous karyorrhexis in a fibrotic dermal stroma (A, C). Verhoeff stain highlighting the loss of elastic fibers in the scar tissue (B). Perivascular extravasated erythrocytes showing glycophorin A–positive staining (D). Dense perivascular neutrophilic infiltrate showing positive immunostaining for MNDA (E) and myeloperoxidase (F).EED was first described by Hutchinson in 1878. It affects both men and women and can develop at any age, with no racial predilection.3,7 Lesions are often asymptomatic, but some patients experience pruritus, pain, and/or arthralgias. EED is benign in nature, with spontaneous resolution often occurring after 5–10 years.2,7 However, it may be associated with systemic diseases, most commonly hematologic disorders (particularly Immunoglobulin A monoclonal gammopathy), autoimmune disorders, and infections (streptococcal infection, HIV, viral hepatitis, and tuberculosis).1–4 Screening for underlying diseases is mandatory. Its physiopathology is still not clearly defined, but many of the conditions associated with EED are immunogenic and are believed to produce immune dysregulation with the deposition of immune complexes in small blood vessel walls.1,3 In this clinical case, EED lesions have developed exclusively within old scar tissue, possibly making this a recall phenomenon, such as bullous pemphigoid in areas of radiodermatitis. Although this is an isolated and not previously described clinical case, considering this observation, we may speculate that EED had a predilection to develop within areas of old scarred tissue. EED is clinically characterized by symmetrical distribution of erythematous violaceous papules and nodules, isolated or confluent, with hardened consistency over extensor surfaces of the extremities.1,7 However, it has many clinical and pathological mimics, and rare presentations have been described including variants with pustular,5 vesiculobullous, hemorrhagic, ulcerative, and annular-type lesions.8 Cases presenting as diffuse neuropathy6 or with major palmoplantar involvement4 have also been reported. For this reason, a skin biopsy is required to establish the diagnosis. The histological features correlate with the stage of the disease. Early lesions demonstrate classical findings of LCV in the upper and mid dermis with neutrophilic perivascular infiltrates and dermal fibrin deposits, endothelial expansion, and leukocytoclasis. Papillary dermal edema may also appear and is clinically related with pseudovesiculation. Late stages of EED characteristically present with indurated nodules consisting of sclerotic fibrous tracts in a lamellar array–amid these, there is conspicuous LCV. The fibrosis is most likely a result of chronic dermal injury.1–3,5,7,9,10 Neutrophilic dermatoses, fibrous proliferations (such as sclerotic fibroma, fibroid nodules of Borrelia, and dermatofibroma), and granuloma faciale share histological similarities to EED but the former lesions lack vasculitis. In granuloma faciale, there is sparing of the adventitial dermis by the infiltrate that is characterized by a predominance of eosinophils. Other differential diagnosis of late stage EED includes granuloma annulare and Kaposi sarcoma.1,2,5–7,9,10 In our clinical case, we observed typical features of pandermal acute LCV within preexisting scar tissue, rather than EED-induced fibrosclerosis, representing the acute stage of EED. Treatment options are limited but dapsone is considered the first-line treatment for EED.1,7 Contrasting results were observed with prednisolone monotherapy.1,2 Our patient had a complete clinical response to oral prednisolone and topical corticosteroids, considered second-line treatment without any secondary side effects. Other therapies include nicotinamide, tetracyclines, chloroquine, nonsteroidal anti-inflammatory drugs, and colchicine.1,7 Surgical excision of isolated EED nodules has been reported, but it should be reserved for patients who do not respond to the standard medical therapy.1,4 Regardless of the treatment, the recurrence rate of EED is high.2,7 When an underlying disease is identified, directed therapy toward it is often the best management.2 Although our clinical follow-up time is small because EED is a chronic disease with fluctuating severity and with possible underlying systemic diseases, the patient needs to maintain a close follow-up. In conclusion, EED shows a heterogeneous clinical and pathological presentation. Recognizing these distinct phenotypes will help clinicians to make a correct clinicopathological diagnosis. Once the diagnosis of EED is established, an investigation of coexisting diseases must be conducted. EED presenting exclusively within scar tissue has not been previously reported, and as such, it may be considered a recall phenomenon. Awareness of this presentation is useful to avoid a misdiagnosis and may help to elucidate the physiopathology of this rare and enigmatic disease.