Pseudoxanthoma Elasticum–Like Papillary Dermal Elastolysis

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4 Sahin MT, Turel A, Gunduz K, Kandiloglu AR, Ozturkcan S. Malignant eccrine poroma in an unusual location. J Eur Acad Dermatol Venereol 2002; 16: 631–633. 5 Nakanishi Y, Matsuno Y, Shimoda T et al. Eccrine porocarcinoma with melanocyte colonization. Br J Dermatol 1998; 138: 519–521. 6 Lan CC, Yu HS, Wu CS, Tsai KB, Wen CH, Chen GS. Pigmented eccrine poroma with enhanced endothelin-1 expression: implications for mechanism of hyperpigmentation. Br J Dermatol 2005; 152: 1070–1072. 7 Ohata U, Hara H, Suzuki H. Pigmented eccrine poroma occurring on the scalp: derivation of melanocytes in the tumor. Am J Dermatopathol 2006; 28: 138–141. 8 Lee HJ, Jeong SH, Seo EJ, Ha SJ, Kim JW. Melanocyte colonization associated with malignant transformation of eccrine poroma. Br J Dermatol 1999; 141: 582–583.

fig. 1 A 62-year-old woman with eruption on the neck characterized by yellowish non-follicular papules.

DOI: 10.1111/j.1468-3083.2007.02317.x Letter totothe XXX Letter LETTERS the TOEditor THE Editor EDITOR

Pseudoxanthoma elasticum-like papillary dermal elastolysis with solar elastosis Editor Pseudoxanthoma elasticum-like papillary dermal elastolysis (PXE-PDE) is a rare, acquired condition characterized by non-follicular yellowish papules coalescing into plaques resembling PXE; it is generally localized on the sides of the neck and subclavicular regions in elderly persons and by loss or decrease of elastic fibres in the papillary dermis.1 Nine cases of PXE-PDE have been reported since 1992.2,3 Despite the preferential occurrence on sun-exposed areas of the skin, the disease has been suggested to be a disorder related to intrinsic ageing based on the histological and ultrastructural similarities characterized by loss of papillary dermal elastic fibres.4,5 No prior case has presented with evidence of solar elastosis. In this report, we present a case of PXE-PDE with solar elastosis. This case shows that PXEPDE may present with actinic damaged skin. A 62-year-old Korean woman presented with asymptomatic skin lesions on the lateral sides of her neck over 1 year. Physical examination revealed many yellow, palpable flat papules on the neck (fig. 1). These lesions were not confluent or follicular. There were no similar lesions on other regions of the body. The patient had no abnormal ophthalmologic and cardiovascular findings. The past medical and family histories were unremarkable. Laboratory tests and the chest X-ray were within normal range. A skin biopsy was fixed in 10% formalin and embedded in paraffin. Paraffin-embedded sections were each stained 368

by haematoxylin and eosin (H&E), Verhoff-van Gieson, and von Kossa stains. Histological examination with H&E stain showed mild perivascular infiltrate of lymphocytes in the superficial dermis and basophilic degeneration in the subpapillary layer. There were no thickened collagen bundles in the papillary and reticular dermis. With Verhoffvan Gieson staining, complete loss of elastic fibres was found in the papillary layer, and focal elastotic changes were seen in the subpapillary to mid-dermal layer (fig. 2). The biopsy specimen was negative for the von Kossa stain. The patient was diagnosed as having PXE-PDE and recommended to have continued observation. Papillary dermal elastolysis of PXE-PDE has been described as an age-related fibro-elastolytic syndrome, characterized by white fibrous papulosis of the neck, temporary wrinkles, and, in some cases, with noninflammatory mid-dermal elastolysis.4 Although late onset, microscopic, and ultrastructural features suggest intrinsic ageing, the exact underlying mechanism remains unknown. Electron microscopic examination of PXE-PDE has confirmed the absence of elastic fibres in the papillary dermis, the presence of immature elastic fibres in the upper reticular dermis and fibroblasts with prominent rough endoplasmic reticulum, as well as numerous dilatations with elongated dendritic processes.4 These elastogenic changes have been interpreted as representing a process of repair of elastolytic damage and as an independent process from solar elastosis. It has been suggested that the elastotic changes observed might be more reflective of the PXE-like clinical appearance rather than elastolytic changes.1 Immunohistochemical studies of PXE-PDE, with anti-elastin and anti-fibrillin-1 antibodies, have shown a decrease in both fibrillin-1 and elastin in affected areas; however, in aged normal-appearing skin, only a decrease in fibrillin-1 is shown.6 Moreover, the loss or accumulation

© 2007 The Authors JEADV 2008, 22, 363– 404 Journal compilation © 2007 European Academy of Dermatology and Venereology

Letters to the Editor

2 Rongioletti F, Rebora A. Pseudoxanthoma elasticum-like papillary dermal elastolysis. J Am Acad Dermatol 1992; 26: 648–650. 3 Akagi A, Tajima S, Kawada A, Ishibashi A. Coexistence of pseudoxanthoma elasticum-like papillary dermal elastolysis and linear focal dermal elastosis. J Am Acad Dermatol 2002; 47 Suppl 2: S189–S192. 4 Rongioletti F, Rebora A. Fibroelastolytic patterns of intrinsic skin aging: pseudoxanthoma elasticum-like papillary dermal elastolysis and white fibrous papulosis of the neck. Dermatology 1995; 191: 19–24. 5 Balus L, Amantea A, Donati P, Fazio M, Giuliano M, Bellocci M. Fibroelastolytic papulosis of the neck a report of 20 cases. Br J Dermatol 1997; 137: 461–466. 6 Ohnishi Y, Tajima S, Ishibashi A, . Inazumi. , Sasaki H, Sakamoto A. Pseudoxanthoma elasticum-like papillary dermal elastolysis: report of four Japanese cases and an immunohistochemical study of elastin and fibrillin-1. Br J Dermatol 1998; 139: 141–144. DOI: 10.1111/j.1468-3083.2007.02318.x Letter totothe XXX Letter LETTERS the TOEditor THE Editor EDITOR

Male genital oedema – allergy and angio-oedema in the differential diagnosis fig. 2 Histochemical staining of the skin lesion. The complete loss of elastic fibres is seen in the papillary layer and focal elastotic changes in the subpapillary to mid-dermal layer are seen (Verhoff-van Gieson staining, ×40).

of microfibrillar components in PXE-PDE lesions has been described.3 Thus, it is suggested that a defect in elastogenesis may contribute to the pathogenesis of PXE-PDE. Our patient represents the first case of PXE-PDE with actinic damage. Although intrinsic ageing has been thought to be the primary pathogenesis of PXE-PDE, we cannot exclude the role of extrinsic factors. HS Lee, HJ Song, WK Hong, JH Shin,* GS Choi Department of Dermatology, Inha University School of Medicine, Incheon, South Korea, *Corresponding author 7-206 shinhung-dong-3-ka, Jung-ku, Incheon 400-711, South Korea, tel. +82 32 890 2238; fax +82 32 890 2236; E-mail: [email protected]

References 1 Tajima S, Ohnishi A, Akagi A, Sasaki T. Elastotic change in the subpapillary and mid-dermal layers in pseudoxanthoma elasticum-like papillary dermal elastolysis. Br J Dermatol 2000; 142: 586–588.

Editor We read the recent contribution by Weinberger et al. on a diagnostic algorithm for male genital oedema with great interest.1 As mentioned by the authors, swelling of the prepuce, penile shaft, and/or scrotum may have different causes.1 Nevertheless, we missed several disorders, which may elicit a swelling of the male genitals alone or in combination with other regions of the body. Angio-oedema [synonymous angioneurotic oedema or Quincke’s oedema; (fig. 1)] describes swelling of the deeper dermis, subcutaneous, or submucosal tissues. The disorder may be elicited by immunological (e.g. IgE) or non-immunological mechanisms (e.g. chemical or physical stimuli). This type of genital lesion is usually not associated with pruritus but rather with pain and/or burning. An angio-oedema may last as long as 48 to 72 h.2,3 In hereditary angio-oedema, an autosomal-dominant disorder, a deficiency of C1 inhibitor is detected. The patients suffer from recurrent episodes of subcutaneous angio-oedema as well as attacks of laryngeal and gastrointestinal involvement with cramps.2,3 Contact urticaria is characterized by typical localized weal and flare response with accompanying symptoms such as burning, itching, or stinging 30 to 60 min after contact with the causative agent, sometimes giving rise to

© 2007 The Authors JEADV 2008, 22, 363– 404 Journal compilation © 2007 European Academy of Dermatology and Venereology


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