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such hereditary malformations present in an autosomal dominant pattern have also been reported, and such cases are difficult to differentiate from HBT. The bilateral clinical presentation, presence of telangiectasias, absence of previous trauma, and appearance of the lesions in both twins makes HBT a more likely diagnosis in our two patients. We have found only two reports in the literature of HBT in patients with no family history4,5 and none in monochorionic twins.
Federica Liuti, MD Pablo J. Almeida, MD Leopoldo Borrego, MD Department of Dermatology Complejo Hospitalario Universitario Insular Materno-Infantil Las Palmas de Gran Canaria Spain Tarsila Montenegro, MD Department of Pathology Complejo Hospitalario Universitario Insular Materno-Infantil Las Palmas de Gran Canaria Spain Federica Liuti, MD Department of Dermatology Complejo Hospitalario Universitario Insular Materno-Infantil Las Palmas de Gran Canaria Spain E-mail:
[email protected]
Eosinophilic annular erythema: complete clinical response with dapsone
Editor, Eosinophilic annular erythema (EAE) is a rare entity of unknown etiology which presents as annular erythematous plaques with tissue eosinophilia. There is controversy over whether EAE should be classified as a new and distinct entity or as a subtype of Wells syndrome. Responses to treatment are variable, and the best reported outcomes are those that occur in response to hydroxychloroquine. A 29-year-old woman with a history of type 1 diabetes mellitus and chronic renal failure was evaluated for a 12-month history of itching and erythematous, annular, polycyclic plaques on the trunk and proximal extremities (Fig. 1). The patient had been treated with oral cortico-steroids and hydroxychloroquine for two months without response. Biopsy material from skin lesions showed minimal alterations in the epidermis (orthokeratosis and mild spongiosis), and changes in the superficial and deep dermis with superficial perivascular and perifollicular infiltrate composed predominantly of eosinophils and a few lymphocytes. There were no signs of vasculitis, flame figures, dermal mucin, or vacuolar changes (Fig. 2). Based on the clinical and histopathological findings, a diagnosis of EAE was made. In this case, given the patient’s history of a poor response to treatment with hydroxychloroquine and poor metabolic control, we initiated dapsone at a dose of 25 mg/d for three weeks. This achieved the resolution of the lesions, which has been sustained over eight months of follow-up (Fig. 3).
Funding sources: None. Conflicts of interest: None. References 1 Wells RS, Dowling GB. Hereditary benign telangiectasia. Br J Dermatol 1971; 84: 93–94. 2 Requena L, Sangueza OP. Cutaneous vascular anomalies. Part I. Hamartomas, malformations, and dilation of preexisting vessels. J Am Acad Dermatol 1997; 37: 549–552. 3 Brancati F, Valente EM, Tadini G, et al. Autosomal dominant hereditary benign telangiectasia maps to the CMC1 locus for capillary malformation on chromosome 5q14. J Med Genet 2003; 40: 849–853. 4 Nakajima I, Okuyama R, Terui T, et al. The first report of non-hereditary benign telangiectasia. J Eur Acad Dermatol Venereol 2006; 20: 1329–1331. 5 Cai L, Sun QM, Zang DJ, et al. Hereditary benign telangiectasia without family history in China. Chin Med J (Engl) 2011; 124: 795–796. International Journal of Dermatology 2015, 54, e94–e108
Figure 1 Clinical examination shows erythematous annular polycyclic plaques on the trunk in a 29-year-old woman with a history of type 1 diabetes mellitus and chronic renal failure ª 2014 The International Society of Dermatology
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Figure 2 (a) Histopathology shows minimal alterations in the epidermis (orthokeratosis and minor spongiosis) and changes in the superficial dermis and the deep dermis, with perivascular and perifollicular infiltrate. (b) The infiltrate is composed mainly of eosinophils and some lymphocytes; there is no evidence of vasculitis, flame figures, dermal mucin or vacuolar changes. (Hematoxylin and eosin stain; original magnification [a] 940, [b] 9200)
Eosinophilic annular erythema is a disease of unknown origin.1 Some authors consider it a subtype of Wells syndrome.2–4 Clinically, it presents as symmetrical erythematous plaques on the trunk and limbs that may occur in either of two patterns: a figurate/centrifugum-type pattern reminiscent of erythema annulare centrifugum, or an urticarial/annular-type pattern resembling granuloma annulare or erythema multiforme.3 These lesions are usually asymptomatic or mildly pruritic. Histologically, the disease is characterized by vacuolar changes in the basal layer of keratinocytes with eosinophilic infiltrate and mucin in the dermis. Direct immunofluorescence is negative. In longstanding lesions, histological findings are sim-
ilar to those observed in Wells syndrome, with flame figures and degranulation of eosinophils.4 Most reports in the literature associate the use of hydroxychloroquine with an improved clinical response.1–6 There are no reports of the use of dapsone. Wells syndrome, however, has been successfully treated with dapsone on several occasions.7–9 To our knowledge, this is the first case of EAE in which a complete clinical response was achieved following treatment with dapsone. We consider this to support the suggestion that this disease is part of the spectrum of conditions seen in Wells syndrome. Acknowledgment Sergio Gonzalez, MD, Professor, Department of Pathology, Pontificia Universidad Catolica de Chile, is thanked for facilitating the production of histological images. Juan Manriquez, MD Daniela Berroeta-Mauriziano, MD Romina Andino-Navarrete, MD Cristi an Vera-Kellet, MD Department of Dermatology Pontificia Universidad Catolica de Chile Marcoleta 350 Santiago Chile E-mail:
[email protected]
Conflicts of interest: None. References Figure 3 After 3 weeks of treatment with dapsone at 25 mg/d,
the patient shows only post-inflammatory hyperpigmentation ª 2014 The International Society of Dermatology
1 Prajapati V, Cheung-Lee M, Schloss E, et al. Spontaneously resolving eosinophilic annular erythema. J Am Acad Dermatol 2012; 67: e75–e77. International Journal of Dermatology 2015, 54, e94–e108
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2 Mebazaa A, Kenani N, Ghariani N, et al. Eosinophilic annular erythema responsive to chloroquin. Eur J Dermatol 2009; 19: 84–85. 3 Rongioletti F, Fausti V, Kempf W, et al. Eosinophilic annular erythema: an expression of the clinical and pathological polymorphism of Wells syndrome. J Am Acad Dermatol 2011; 65: e135–e137. 4 El-Khalawany M, Al-Mutairi N, Sultan M, et al. Eosinophilic annular erythema is a peculiar subtype in the spectrum of Wells syndrome: a multicentre longterm follow-up study. J Eur Acad Dermatol Venereol 2013; 27: 973–979. 5 Howes R, Girgis L, Kossard S. Eosinophilic annular erythema: a subset of Wells syndrome or a distinct entity? Australas J Dermatol 2008; 49: 159–163. 6 Sempau L, Larralde M, Luna PC, et al. Eosinophilic annular erythema. Dermatol Online J 2012; 18: 8. 7 Marks R. Eosinophilic cellulitis – a response to treatment with dapsone: case report. Australas J Dermatol 1980; 21: 10–12. 8 Zhu YI, Stiller MJ. Dapsone and sulfones in dermatology: overview and update. J Am Acad Dermatol 2001; 45: 420–434. 9 Moon S-H, Shin M-K. Bullous eosinophilic cellulitis in a child treated with dapsone. Pediatr Dermatol 2013; 30: e46–e47.
Localized lymphomatoid papulosis
A 63-year-old woman presented with a 2-month history of multiple asymptomatic cutaneous lesions on the abdomen. She was otherwise healthy and was not taking any medication. Dermatological examination revealed a crop of 2–4 mm, erythematous papules confined to the left side of the abdomen; some lesions were covered with crusts (Fig. 1). No other cutaneous or mucosal lesions were noted. There was no lymphadenopathy or hepatosplenomegaly. Routine laboratory studies were normal. A skin biopsy showed a dermal infiltrate containing anaplastic lymphoid cells with pleomorphic vesicular nuclei and abundant cytoplasm (Fig. 2). These cells are mixed with neutrophils, eosinophils, lymphocytes, and histiocytes. Immunohistochemical staining was performed using EnVision+ method (DAKO, Glostrup, Denmark). The atypical lymphoid cells were immunoreactive for CD45, CD4, and CD30 (Fig. 2) but negative for CD15, CD20, CD8, perforin, granzyme B, and T-cell receptor-gamma and -betaF1. A T-cell receptor gene rearrangement was not detected. A diagnosis of localized lymphomatoid papulosis (LyP) type A was made. Treatment with topical clobetasol led to a complete resolution of skin lesions. However, new lesions continued to appear, limited to the affected area, which healed spontaneously. After 18 months of follow-up, no recurrence or associated disease have been detected. International Journal of Dermatology 2015, 54, e94–e108
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Figure 1 (a) A crop of erythematous papules confined to the left abdomen. (b) Close-up view of the lesions.
LyP is currently classified within the spectrum of CD30+ lymphoproliferative disorders according to the WHO-EORTC classification for cutaneous lymphomas.1 Clinically, it consists of red–brown papules and/or nodules that may develop necrosis and crusting and heal spontaneously, sometimes with scarring.1,2 The individual lesions resolve within weeks or months, while the disease may recur for decades. Histopathologically, LyP has traditionally been classified into three subtypes: type A (histiocytic), type B (mycosis fungoides-like), and type C (anaplastic large cell lymphoma-like).1–3 Recently, a further variant simulating histologically an aggressive epidermotropic CD8-positive T-cell lymphoma has been described, which was named LyP type D.3 More recently, an angioinvasive CD30+ CD8+ variant of LyP that simulates aggressive angiocentric T-cell lymphomas has been reported (LyP type E).4 The lesions of LyP usually occur in a generalized distribution, with multiple lesions over the trunk and extremities. However, in a small subset of patients, the eruption is limited to a single anatomic region (localized or regional LyP).5 To date,
