Spots on the torso and arms


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A 38-year-old African American woman was referred to dermatology because of spots on her torso and arms. The lesions were asymptomatic, but their development, cause, and cosmesis were of concern. No benefit was seen with the use of OTC remedies such as hydroxycortisone 1% cream or vitamin E oil. Her medical history was significant for systemic lupus erythematosus, well controlled on hydroxychloroquine. Physical examination revealed numerous, well-circumscribed, 0.5- to 2-cm, hypopigmented, round-to-oval macules of wrinkled skin on her anterior chest wall, back, abdomen, and bilateral upper arms. Invagination of the lesions was noted with palpation. A punch biopsy was performed.

Histologic findings from a punch biopsy demonstrated a perivascular and periadnexal lymphohistiocytic infiltrate with normal collagen and no microthrombi; special stains revealed a marked loss of elastic fibers within the dermis, making the diagnosis of anetoderma.Anetoderma, an elastolytic disorder characterized...

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Histologic findings from a punch biopsy demonstrated a perivascular and periadnexal lymphohistiocytic infiltrate with normal collagen and no microthrombi; special stains revealed a marked loss of elastic fibers within the dermis, making the diagnosis of anetoderma.

Anetoderma, an elastolytic disorder characterized by focal areas of loose skin, is categorized as primary or secondary based on a preexisting inflammatory process. The term anetoderma is derived from the Greek word “anetos,” meaning “slack.”1 Sometimes anetoderma is referred to as macular atrophy due to its clinical findings.2

Anetoderma presents as well-circumscribed areas of wrinkled skin that feel atrophic on palpation and can either herniate inward or bulge outward as sac-like protrusions.2 Most lesions are discrete, round-to-oval in shape, and range in diameter from 0.5 to 2 cm; however, some lesions can coalesce into larger patches.3 Areas are typically skin-colored, though they may be erythematous or hypopigmented. Lesions are most commonly localized to the trunk, thighs, and upper arms. Involvement of the face and neck is rare, while the scalp, palms, and soles are distinctively spared.6 The number of lesions varies greatly, from fewer than 5 to more than 100.3 Lesions may develop individually, or in crops, and enlarge throughout the course of 2 to 3 weeks; new lesions may continue to develop over years. Once present, they persist indefinitely. Fortunately, most are asymptomatic, although pruritus can occur. 3

The classification of anetoderma as primary or secondary is determined by the absence (primary) or presence (secondary) of a pre-existing inflammatory process. Curiously, at times both types can be seen in one individual and both can occur in the presence of a systemic disease.2 Familial anetoderma has been described but is exceedingly rare. Similarly rare is anetoderma in infancy.

Initially, primary anetoderma was considered to be an idiopathic disorder; however, substantive evidence indicates an association with various immunologic processes, most commonly the presence of antiphospholipid antibodies, with or without the antiphospholipid syndrome.4 A variety of serologic tests may be abnormal in those with anetoderma, including complement levels and lupus anticoagulant, as well as anticardiolipin, anti-nuclear, and anti-beta-2 glycoprotein antibodies, with or without meeting the criteria for a specific connective tissue disease.4 That said, the association between anetoderma and systemic lupus erythematosus (SLE) is well accepted.5-7 However, because other systemic conditions such as syphilis, Sjogren syndrome, and systemic-sclerosis have also been linked to anetoderma, each patient should be thoroughly evaluated.

Secondary anetoderma develops after an inflammatory, infiltrative, or iatrogenic process. Inflammation due to acne, urticarial pigmentosa, syphilis, varicella, lyme disease, leprosy, angular cheilitis, post-hepatitis B vaccination, granuloma annulare, Takayasu arteritis, and juvenile xanthogranuloma may all result in anetoderma. Neoplastic processes such as pilomatrixomas, schwannomas, B-cell lymphomas, mycosis fungoides, hamartomatous congenital melanocytic nevi, myxofibrosarcoma, and dermatofibromas have each been reported in association with anetoderma as well.2,8

Anetoderma may also be iatrogenic, most commonly seen following penicillamine use7 or in association with premature birth.9 Penicillamine-induced anetoderma has been reported in patients with Wilson disease.3 Theoretically, penicillamine may directly alter elastic tissue, or, perhaps, modify lysyl oxidase activity, a copper-dependent enzyme that plays a role in cross-linking elastin.3

Iatrogenic anetoderma of prematurity develops in infants, born between 24 to 32 weeks of gestation, within areas of trauma due to monitoring leads or electrodes or where adhesive tape was placed during their time in the intensive care units.10 Although most neonatal cases of anetoderma are iatrogenic, rarely will anetoderma present in a premature newborn who has not had perinatal monitoring; most congenital cases are associated with very low birth weight (<5th centile).9

The differential diagnosis of localized areas of loose skin is extensive. Conditions to be considered include a variety of other elastic tissue disorders such as cutis laxa, mid-dermal elastolysis, post-inflammatory elastolysis, papular acne scars, acrodermatitis chronica atrophicans, Atrophoderma of Pasini and Pierini, corticosteroid-induced atrophy, lichen sclerosus, discoid lupus erythematous, perifollicular atrophoderma, perifollicular macular atrophy, neurofibromas, acrodermatitis chronica atrophicans, granulomatous slack skin (a variant of mycosis fungoides), and acrodermatitis chronica atrophicans.2

It is important to use the entire constellation of history, physical examination, and histologic findings to make the diagnosis of anetoderma. Lesion morphology and distribution are uniquely distinct from those of cutis laxa, mid-dermal elastolysis, and postinflammatory elastolysis, which tend to be more diffuse and widespread, compared with the well-circumscribed lesions of anetoderma.2 When in doubt, histologic examination with special stains will demonstrate the classic loss of elastic fibers of anetoderma.11

The exact pathogenesis of anetoderma remains speculative. Proposed mechanisms include alterations in elastolytic enzyme activity, tissue ischemia, and phagocytosis of elastic fibers, as well as a variety of autoimmune and inflammatory processes.12 Jeong et al postulated that microthrombosis within dermal vessels induced local ischemia and elastic tissue degeneration in lupus-associated anetoderma, especially when associated with antiphospholipid antibodies.5 In the rare cases of familial or congenital anetoderma, theories revolve around the possibility of decreased production of elastic tissue, or increased destruction due to an altered balance of matrix metalloproteases.9

There is no approved treatment for anetoderma; lesions are generally everlasting.2 Reassurance should be provided that anetoderma is a benign, and usually asymptomatic, condition.3 When identified, causes of secondary anetoderma should be appropriately treated to reduce the risk of new lesions.8

Anecdotal reports suggest that topical corticosteroids and vitamin E, as well as oral penicillin G, niacin, aspirin, phenytoin, dapsone may be somewhat helpful.

Although not FDA approved, or currently standard of care, surgical techniques such as cryotherapy7 or laser therapy may show some benefit.13,14 Complete surgical excision will result in lesion removal, but the cosmetic result may not be a good alternative.

Complete evaluation for our patient showed no additional laboratory abnormalities, specifically, no antiphospholipid antibodies. It was determined that her systemic lupus erythematosus was the associated systemic condition. She opted not to pursue any other treatment option for the isolated lesions.

Margaret Barton is a fourth-year medical student, and Julia Nunley is a professor of medicine at the Virginia Commonwealth University School of Medicine in Richmond, Va.


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  3. Venencie PY, Winkelmann RK, Moore BA. Anetoderma. Clinical findings, associations, and long-term follow-up evaluations. Arch Dermatol. 1984;120:1032-1039.
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  10. Maffeis L, Pugni L, Pietrasanta C, et al. Iatrogenic anetoderma of prematurity: a case report and review of the literature. Case Rep Dermatol Med. 2014;2014:781493.
  11. Venencie PY, Winkelmann RK. Monoclonal antibody studies in the skin lesions of patients with anetoderma. Arch Dermatol. 1985;121:747-749.
  12. Ishida Y, Koizumi N, Shinkai H, Miyachi Y, Utani, A. Primary anetoderma: a case report and its modified classification. J Dermatol. 2005;32:982-986.
  13. Cho S, Jung JY, Lee JH. Treatment of anetoderma occurring after resolution of Stevens-Johnson syndrome using an ablative 10,600-nm carbon dioxide fractional laser. Dermatol Surg. 2012;38:677-679.
  14. Lee SM, Kim YJ, Chang SE. Pinhole carbon dioxide laser treatment of secondary anetoderma associated with juvenile xanthogranuloma. Dermatol Surg. 2012;38:1741-1743.
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