Anetoderma Secondary to Mid-dermal Elastolysis Gabriela A. Maloney, BS,* Jane James, MD, PhD,** Michael Welsch, MD,** Marylee Braniecki, MD** *Midwestern University, Downers Grove, IL **Pathology Department, University of Illinois Hospital & Health Sciences System, Chicago, IL Abstract Anetoderma usually presents as circumscribed, 1 cm to 2 cm patches and plaques of flaccid skin secondary to loss of dermal elastic tissue. Lesions often occur in the neck, upper extremities, chest, and back. On histopathology, one sees complete loss of dermal elastin involving the papillary and reticular dermis, with infiltration of plasma cells and histiocytes. A 40-year-old female with no significant medical history presented with multiple round, 1 cm to 2 cm lesions scattered on her upper back and chest. Skin biopsy demonstrated elastic-fiber loss localized to the mid-dermis along with a lymphohistiocytic infiltrate with elastophagocytosis and active inflammatory phase in the papillary and mid-reticular dermis. The histopathological findings were consistent with mid-dermal elastolysis with advancing inflammation, and the clinical features were consistent with anetoderma. The microscopic examination revealed an active inflammatory phase of mid-dermal elastolysis, supporting the postulated theory that MDE may be part of a continuous spectrum with anetoderma. Case Report by lax, wrinkled skin with underlying palpable biopsy and elastic-fiber staining demonstrated A 40-year-old female with no significant medical depression (Figure 1). They were often preceded elastic-fiber loss in the mid-dermis along with history presented with multiple round, 1 cm to by two to six months of local erythema and had a lymphohistiocytic infiltrate with evidence 2 cm lesions scattered throughout the upper increased in number over the past two years. No of elastic-fiber phagocytosis and an active back and chest. The lesions were characterized response was seen after topical steroids. Skin inflammatory background in the papillary and reticular dermis (Figures 2, 3 and 4). This is a case demonstrating the development of anetoderma as seen by the progressive inflammation and elastophagocytosis in the papillary and reticular dermis that developed in the setting of mid- dermal elastolysis (MDE). Figure 1: Multiple round-to-ovoid, wrinkled skin lesions with overlying palpable depressions and surrounding erythema. Figure 2: H&E (10x): Active inflammatory phase with mid-dermal stromal histiocytes with scattered plasma cells. Discussion Mid-dermal elastolysis (MDE) is a rare acquired disorder of elastic-tissue degradation limited to the mid-dermis. It consists of a clear band of mid-dermal elastic-tissue loss as a result of inflammatory destruction of dermal elastotic fibers.1 The elastic-tissue loss occurs as a result of inflammatory destruction of dermal elastic fibers. Remnants of abnormal elastic tissue and granuloma formation may be present, along with evidence of elastophagocytosis.2 Elastic tissue is Figure 3: H&E (20x): Multinucleated Figure 4: Elastic stain (20x): Elastic fibers usually preserved around hair follicles, resulting histiocytes with elastophagocytosis. engulfed by histiocytes. in perifollicular papules on the affected skin. Page 40 ANETODERMA SECONDARY TO MID-DERMAL ELASTOLYSIS The condition was first described in 1977 by anetoderma. Emer et al. described a case of of anetoderma as seen by progressive Shelley and Wood, and since then, there have anetoderma that was instigated by penicillin G to inflammation and elastophagocytosis in the been approximately 80 cases reported in the treat syphilis in an HIV-positive patient without papillary and reticular dermis that developed in literature.3 It has a female predominance and any previous skin complaints.9 the setting of mid-dermal elastolysis (MDE). The presents clinically as diffuse, fine wrinkling on Mid-dermal elastolysis and anetoderma, both histopathological findings were consistent with the neck, arms, and trunk in patients between the mid-dermal elastolysis with active inflammation, 2 disease entities resulting from elastic-fiber ages of 30 and 50 years. degradation, are differentiated histopathologically and the clinical features were consistent with It is classified into three types: type I, or classic by the extent and location of elastic-fiber loss. The anetoderma. The microscopic examination type, with well-demarcated patches with former consists of elastic-tissue loss localized to revealed elastic fiber loss localized to the mid- wrinkling; type II, with perifollicular papular the mid-dermis, and the latter is characterized by dermis along with a lymphohistiocytic infiltrate protrusions; and type III, with reticular/annular elastic-fiber loss in the entire dermis.1 with elastophagocytosis and active inflammatory patches with wrinkling. phase in the papillary and mid-reticular dermis. It has been speculated that MDE and anetoderma Such areas of active inflammation indicate the Mid-dermal elastolysis has been reported to are within the same spectrum of disorders, as they development of anetoderma in the pre-existing originate from involuting sites of granuloma present similar histopathological configurations MDE background as elastic-fiber degradation annulare that started as a patchy, slightly indurated to different extents, suggesting MDE may evolve spread beyond the mid-dermis to involve the and violaceous eruption involving the neck and into anetoderma secondary to long-standing papillary and reticular dermis. trunk and eventually became atrophic, pale and inflammation.4,10,11 Our patient demonstrated a wrinkled. Urticaria, atopic dermatitis, Sweet’s classic histopathology of MDE with secondary Anetoderma often occurs from existing inflammatory processes that lead to elastic-fiber syndrome, phototoxic dermatitis, and pityriasis anetoderma resulting from an active inflammation 4 thus the overall findings in this case rosea have also been described to precede MDE. and elastophagocytosis, lymphocytes, plasma cells destruction, may support the theory that MDE is part of a Mid-dermal elastolysis can also occur in areas of and histiocytes extending to the papillary and continuous spectrum with anetoderma. preceding erythema, which is consistent with our reticular dermis. patient’s clinical presentation.5 Mid-dermal elastolysis and anetoderma need to References Anetoderma, also known as dermatitis maculosa be differentiated from other connective-tissue 1. Bolognia JL, Jorizzo JL, Schaffer JV, eds. atrophicans, is an elastic-tissue disorder that diseases affecting elastic fibers, including cutis Dermatology. 3rd ed. Philadelphia: Elsevier shows focal loss of elastic fibers in the dermis. laxa, pseudoxanthoma elasticum (PXE), and Saunders; 2012. 1631-35 p. The term “anetoderma” is derived from the PXE-like papillary dermal elastolysis. Post- 2. Gambichler T. Mid-dermal elastolysis revisited. Greek words “anetos,” meaning slack, and traumatic scars, perifollicular elastolysis, papular 1 Arch Dermatol Res. 2010;302:85-93. “derma,” meaning skin. Sac-like tumors that elastorrhexis, pseudoxanthoma elasticum, focal herniate upon palpation were first described dermal hypoplasia (Goltz syndrome), and nevus 3. Shelley WB, Wood MD. Wrinkles due to by Schweninger and Buzzi in 1891, but lipomatosus are other entities that may be included idiopathic loss of mid-dermal elastic tissue. Br J anetoderma was first officially described in 1892 in the differential diagnosis of anetoderma.1 Dermatol. 1977;97:441-5. 7,8 by Jadassohn. It usually presents as multiple, Cutis laxa is an entity with redundant and loose 4. Lai JHC, Murray SJ, Walsh NM. Evolution of circumscribed, 5 mm to 25 mm areas of flaccid skin seen on the eyelids, cheeks, shoulder girdle, granuloma annulare to mid-dermal elastolysis: skin with fine wrinkling that can occur in the abdomen and neck. It presents clinically with report of a case and review of the literature. J neck, upper extremities, chest, and back. The premature aging secondary to loose skin folds Cutan Pathol. 2014;41:462-8. lesions are skin color but can present with a blue- 6 with or without internal organ involvement. In Affected areas of skin can 5. Gambichler T. Mid-dermal elastolysis revisited. white discoloration. this condition, the whole dermis is affected with herniate after palpation (“buttonhole” sign) and Arch Dermatol Res. 2010;302:85-93. 1 diminished and fragmented elastic fibers, and it can show central depressions. 1 can occur in an acquired or hereditary form. 6. Venecie PY, Winkelmann RK, Moore BA. Anetoderma: clinical findings, associations, and Anetoderma is classified as either primary Pseudoxanthoma elasticum (PXE) and PXE- (idiopathic) or secondary. Primary anetoderma long-term follow-up evaluations. Arch Dermatol. like papillary dermal elastolysis present as 1984;120:1032-9. is divided in two types: Jadassohn-Pellizzari type, cobblestoning yellow papules and redundant folds which has preceding inflammatory lesions, and in flexor areas. The former can be associated with 7. Schweninger E, Buzzi F. Multiple benign Schweninger-Buzzi type, which has no preceding ocular and cardiovascular involvement.1 It occurs tumor-like new growths of the skin. International inflammation. It is seen more commonly in in sites of previous scars, axilla, groin, and lateral Atlas Seltener Hautkrankheiten, plate 15. women than men and mostly occurs in individuals 9 neck and consists of clumped and calcified elastic Leipzig: L Voss, 1891. Secondary between 15 and 25 years of age. fibers