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CLINICAL REPORT Advances in dermatology and venereology ActaDV Acta Dermato-Venereologica ActaDV
Annular Eruptive Pseudoangiomatosis and Adenovirus Infection: A Novel Clinical Variant of Paraviral Exanthems and a Novel Virus Association
Antonio CHUH 1, Rüdiger PANZER 2, Ann-Christine ROSENTHAL 2, Ehrhardt PROKSCH 2, Werner KEMPF 3, Vijay ZAWAR 4, Helmuth FICKENSCHER 5 and Regina FÖLSTER-HOLST 2
1
Jockey Club School of Public Health and Primary Care, The Chinese University of Hong Kong and the Prince of Wales Hospital, Shatin, Hong Kong, 2 Dermatologie, Venerologie und Allergologie, and 5 Institut für Infektionsmedizin, Christian-Albrechts-Universität zu Kiel und Universitätsklinikum Schleswig-Holstein, Campus Kiel, Kiel, Germany, 3 Histologische Diagnostik, and Department of Dermatology, University Hospital Zürich, Zürich, Switzerland, and 4 Department of Dermatology, Godavari Foundation Medical College and Research Center, DUPMCJ, Nashik, India
Eruptive pseudoangiomatosis is a distinct exanthem thought to be caused by viruses. The usual rash configu ration is erythematous papules and macules. An association with echovirus infection has been reported. We present here one adult and one child with this exan them, supported by clinical, histopathological, and immunohistochemical findings. Both patients presented with prodromal symptoms, widespread angioma-like macules in annular configuration, blanchable telangiectasia, followed by spontaneous remission in 6 – 8 weeks. Lesional histopathology of the adult patient revealed dilated dermal blood vessels and lymphohistiocytic infiltrates predominated by CD4 + lymphocytes with a 5:1 ratio of CD4: CD8 lymphocytes. No B cells or CD56 + natural killer cells were found. Serology of both patients revealed evidence of active infections by adenoviruses, and a range of other viruses were excluded. We believe that these 2 patients manifested annular eruptive pseudoangio matosis, a novel variant of the rash with a probable adenovirus association that has not yet been reported.
Key words: annular rash; cell-medicated immunity; eruptive hypomelanosis; Gianotti – Crosti syndrome; pityriasis rosea; viral infection.
Accepted Oct 3, 2016; Epub ahead of print Oct 4, 2016 Acta Derm Venereol 2017; 97: 354 – 357.
Corr: Antonio Chuh, Shops 5 and 6, The Imperial Terrace, 356 Queen’ s Road West, G / F, Hong Kong. E-mail: antonio. chuh @ yahoo. com. hk
Eruptive pseudoangiomatosis( EP) was first described by Cherry et al. in 1969( 1). In 1993, Prose et al.( 2) described 3 children with angioma-like papules during viral illnesses. As the skin lesions appeared suddenly,“ like an explosion”, it qualifies as eruptive. As the macroscopic appearance is akin to cherry angiomas, with the histopathological changes not incorporating vascular proliferation, it qualifies as pseudoangiomatosis. EP was thus termed as such by Prose et al. in 1993( 2), some 24 years after the initial description of this exanthem( 1).
Owing to its clinical course, with spontaneous remission, infectious causes are suspected. EP is one of several loosely categorized skin diseases known as paraviral exanthems, denoting that the disease is suspected to be related to infectious, usually viral, causes, but without an established single microbe-disease aetiology( 3, 4).
The inclusion of diseases in this category is debated, but is generally taken to be pityriasis rosea, pityriasis lichenoides, Gianotti – Crosti syndrome, asymmetric periflexural exanthem / unilateral laterothoracic exanthem, papular-purpuric gloves and socks syndrome, EP, and eruptive hypomelanosis( 3 – 5).
In this family of diseases, EP is probably one of the least common. We present here reports of one adult and one child with eruptions for which the clinical features and laboratory findings strongly substantiated a diagnosis of EP, and suggest a novel aetiology and a novel morphological variant of this exanthema.
CASE REPORTS
Patient 1. A 19-year-old male presented with a 2-day history of a mildly pruritic skin rash, characterized by annular erythema with dark-red plaques. Some of the annular lesions were closed, while others were openended( arcs). The lesions were identifiable as blanchable telangiectasia on the trunk, arms and face( Fig. 1a – c). The patient had diarrhoea, which started one week before onset of the rash.
A lesional skin biopsy was performed for histopathological and immunohistochemical analyses( Fig. 1d). Beneath an epidermis with focally increased basal pigmentation, there were slightly ectatic capillary vessels with prominent hobnail-like endothelia, and perivascular lympho-histiocytic infiltrates with admixture of melanophages. Immunohistochemical staining showed predominantly CD4 + lymphocytes with a ratio of CD4: CD8 lymphocytes of approximately 5:1. There was no admixture of B cells, CD56 + natural killer cells, or natural killer T cells. In addition, CD123 + plasmacytoid dendritic cells were absent.
Serological investigations on the active serum revealed elevated titres of IgG and IgA against the adenoviruses( IgG: 33.6 U / ml, reference: < 14 U / ml; IgA: 29.8 U / doi: 10.2340 / 00015555-2541 Acta Derm Venereol 2017; 97: 354 – 357
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