Papules and vesicles on the ears of a young boy

Clinicopathologic challenge Papules and vesicles on the ears of a young boy ~ oz1, MD, Cristina Lo  pez-Sa nchez2, MD, Eduardo Rozas-Mun 2, MD and Juan F. Mir-Bonafe 4, MD Jaime Piquero-Casals3, MD, Esther Roe 1 Department of Dermatology, Hospital San Pablo, Coquimbo, Chile, 2Department of Dermatology, Hospital Sant Pau, Barcelona, Spain, 3Department of gica Dermatology, Clınica Dermatolo Multidisciplinar Dermik, Barcelona, Spain, and 4Department of Dermatology, Hospital tzer, Palma de Mallorca, Spain Son Lla What is your diagnosis? Correspondence ~oz, MD Eduardo Rozas-Mun Department of Dermatology Hospital San Pablo Avenida Videla S/N 1780000 –Coquimbo Chile E-mail: [email protected] Conflict of interest: None. Funding source: None. doi: 10.1111/ijd.15336 Case presentation similar, but less severe and self-limited, eruption 1 year ago. Parents denied the use of any topical or systemic photosensi- An 11-year-old Caucasian boy presented with a slightly pruritic tive medication. Results of laboratory investigations, including a rash involving his ears and face that appeared 1 day after play- serum chemistry panel, liver function tests, complete blood cell ing soccer outdoors. Physical examination revealed erythema- count, and erythrocyte sedimentation rate, revealed no abnor- tous papules and vesicles involving the helix of his ears malities. Serologic tests were negative for herpes virus 1 infec- bilaterally. Additionally, erythematous confluent papules and small vesicles were located on the lips, cheeks, neck, periauric- tion, and antinuclear and anti-Ro antibodies. A 4 mm punch biopsy was taken from one of the lesions (Fig. 2). ular area, and dorsum of the arms (Fig. 1). The patient had a Figure 1 (a) Vesicles and crusts on the lips. Erythema on the neck. (b) Erythematous plaques and small vesicles on the neck and auricular and periauricular area. (c) Erythema and vesicles on the left helix ª 2020 the International Society of Dermatology Figure 2 (a) Biopsy specimen showing spongiosis and intraepidermal vesiculation with marked papillary dermal edema. A mild perivascular infiltrate of lymphocytes without atypia was seen in the upper dermis. (c) High power image showing intraepidermal vesiculation and papillary dermal edema. (Hematoxylin and eosin, (a) 94; (b) 910; (c) 920) 1 International Journal of Dermatology 2020 2 Clinicopathologic challenge ~ oz et al. Rozas-Mun Papules and vesicles on ears of a young boy Diagnosis atinocytes. Acute lesions of subacute cutaneous lupus erythematosus are less pruritic and also present with interface Juvenile spring eruption. dermatitis. Hydroa vacciniforme characteristically evolves with severe scarring and histologically shows extensive reticular degeneration and epidermal necrosis, focally atypical lympho- Discussion cytes, and some degree of angiocentricity. In situ hybridization Juvenile spring eruption is a rare variant of polymorphic light for EBV is positive. The exact pathogenesis remains unknown, eruption characterized by a pruritic eruption on the ears of chil- but genetic susceptibility and environmental factors seem to 1 dren during spring or early summer. Patients are usually young boys, with short hair, presenting with recurrent episodes of ery- play an important role. Significant associations include uncovered and large ears, and sunny and cool weather.5 thematous and edematous papules and vesicles, located on the Provocative photo testing is usually negative, but the positive light-exposed areas of the ears. In rare cases, involvement of other light-exposed areas typical of polymorphic light eruption, reaction to repetitive doses of ultraviolet A (UVA), applied to the such as the face and dorsum of the arms and hands, have also eruption may represent a localized hypersensitivity reaction to 1 been noted. Skin lesions usually appear within hours or some- affected skin in one case, has suggested that juvenile spring UV radiation.3 times days after exposure to sunny but cold weather. The erup- Most cases do not require any treatment, since lesions heal tion lasts one to several weeks and then resolves with minimal or no scarring. Outbreaks have been described in children’s spontaneously in a few days.4 However, symptomatic improvement can be obtained with oral and topical antihistamines and summer camps and military soldiers performing outside exer- corticosteroids.1 Additionally, preventive measures such as sun cises in sunny days.2,3 Histologically, fully developed cutaneous lesions are characterized by marked edema of the papillary der- avoidance and sunscreens are recommended to prevent recurrences.4 mis that may be associated with subepidermal or intraepidermal vesicle formation. A mild-to-dense superficial and sometimes deep perivascular lymphohistiocytic infiltrate is also seen. Papillary dermal erythrocyte extravasation and features secondary to scratching, such as hypergranulosis, hyperkeratosis, and acanthosis, are also frequent.2,4 Differential diagnosis includes disorders such as impetigo, herpes simplex viral infection, erythema multiforme, subacute cutaneous lupus erythematosus, and hydroa vacciniforme. Impetigo and herpes simplex infection are usually present unilaterally. Additionally, impetigo characteristically has golden yellow crusts, and herpes simplex infection is usually more painful. In case of doubt, ancillary testing such as cultures or polymerase chain reaction (PCR) can be helpful. Erythema multiforme may affect the ears and may also rarely be sun induced.5 However, histological examination will reveal an interface dermatitis with a variable number of necrotic ker- International Journal of Dermatology 2020 References 1 Berth-Jones J, Norris PG, Graham-Brown RA, et al. Juvenile spring eruption of the ears: a probable variant of polymorphic light eruption. Br J Dermatol 1991; 124: 375–378. 2 Requena L, Alegre V, Hasson A. Spring eruption of the ears. Int J Dermatol 1990; 29: 284–286. 3 Stratigos AJ, Antoniou C, Papadakis P, et al. Juvenile spring eruption: clinicopathologic features and phototesting results in 4 cases. J Am Acad Dermatol 2004; 50: 57–60. 4 Lava SAG, Simonetti GD, Ragazzi M, et al. Juvenile spring eruption: an outbreak report and systematic review of the literature. Br J Dermatol 2013; 168: 1066–1072. 5 Wolf P, Soyer HP, Fink-Puches R, et al. Recurrent post-herpetic erythema multiforme mimicking polymorphic light and juvenile spring eruption: report of two cases in young boys. Br J Dermatol 1994; 131: 364–367. ª 2020 the International Society of Dermatology