Associated Pyoderma Gangrenosum, Erythema Elevatum Diutinum, and Chronic Recurrent Annular Dermatosis: The Neutrophilic Disease Spectrum

Alj, Maha; Eljazouly, Madiha; Chahboun, Fatimazahra; Bouzidi, Abderahmane Al; Chiheb, Soumiya

doi:10.7759/cureus.21005

Cited by 2 publications

(1 citation statement)

References 14 publications

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“…The timely diagnosis and treatment of EED are important, as this condition points to underlying systemic conditions, and older lesions may be less responsive to therapeutic interventions due to the nodular fibrosis characteristic of long-standing EED. However, even with treatment, the recurrence rate of EED is high, with complete resolution of the disease not always possible [ 12 , 13 ]. Treatment of underlying triggering factors, such as systemic disease, may provide the highest chance of success in the prevention of relapse of EED [ 6 ].…”

Section: Discussionmentioning

confidence: 99%

Transient Erythema Elevatum Diutinum Associated With HIV Viremia

Rashdan¹,

Schäfer²,

Lundgren³

et al. 2023

Cureus

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Erythema elevatum diutinum (EED) is a rare cutaneous small vessel vasculitis of unknown etiology. It is thought to be due to immune complex deposition in small vessels, resulting in complement fixation and subsequent inflammation. EED classically presents with asymptomatic, symmetric, red-brown to purple papules, plaques, and nodules overlying extensor surfaces with a lapsing-remitting course that typically resolves within five to 10 years. We discuss the case of a 47-year-old male with HIV and a new history of EED presenting after several days of missed antiretroviral medications and resolved with improved compliance with antiretroviral medications. A 47-year-old male presented with a four-week history of mildly tender violaceous plaques and nodules on the dorsal feet and posterior heels bilaterally. Medical history was significant for HIV that was well-controlled on antiretrovirals although the patient had missed two days of therapy. A punch biopsy of the lesion demonstrated leukocytoclastic vasculitis with dense dermal mixed infiltrate consisting of histiocytes, neutrophils, and eosinophils. Laboratory findings revealed the presence of HIV RNA. Prior to the initiation of Dapsone therapy, the patient’s eruption cleared entirely within a month solely by restarting his antiretroviral therapy, for which he continues to remain disease-free. EED is a rare, chronic leukocytoclastic vasculitis with a poorly understood etiology. Treatment is typically aimed at treating underlying systemic disease, however, treatment of EED with Dapsone is typically first-line.

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Section: Discussionmentioning

confidence: 99%

Transient Erythema Elevatum Diutinum Associated With HIV Viremia

Rashdan¹,

Schäfer²,

Lundgren³

et al. 2023

Cureus

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Zeldzame neutrofiele dermatose met belangrijke hematologische implicaties

Baert,

Bosma,

Goeteyn

et al. 2023

Tijdschr Geneesk

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Rare neutrophilic dermatosis with significant hematologic implications A patient was seen in the dermatology department because of a solitary lesion on the right forearm. This lesion had an annular aspect with a raised, bright erythematous, inflammatory border of confluent papules with an oedematous aspect. Previously, similar lesions had developed, regressing spontaneously after a few weeks. Histological examination showed a marked oedema and a dens dermal predominant neutrophilic infiltrate with leucocytoclasia without vasculitis. Based on the clinical picture, the chronic recurrent course and the histology, the diagnosis of chronic recurrent annular neutrophilic dermatosis (CRAND) was made. Since CRAND belongs to the neutrophilic skin disorders, a comprehensive blood test was performed, showing a monoclonal B cell population. In consultation with a fellow haematologist, additional CT scans of the thorax and abdomen were performed. These showed neither adenopathies nor hepatosplenomegaly, upon which the diagnosis of monoclonal B cell lymphocytosis was made. This condition increases the risk of developing chronic lymphatic leukaemia. This case report thus shows a rare neutrophilic dermatosis that differs from Sweet syndrome given the absence of fever, systemic symptoms or neutrophilia in the blood. This case is specifically relevant because of the additional finding of a monoclonal B cell lymphocytosis, increasing the risk of developing chronic lymphatic leukaemia. This demonstrates the importance of further additional investigation to exclude possible (pre)malignancies when the diagnosis of CRAND is made.

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Associated Pyoderma Gangrenosum, Erythema Elevatum Diutinum, and Chronic Recurrent Annular Dermatosis: The Neutrophilic Disease Spectrum

Cited by 2 publications

References 14 publications

Transient Erythema Elevatum Diutinum Associated With HIV Viremia

Transient Erythema Elevatum Diutinum Associated With HIV Viremia

Zeldzame neutrofiele dermatose met belangrijke hematologische implicaties

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