- Case report
- Open Access
An unusual presentation of anetoderma: a case report
BMC Dermatology volume 4, Article number: 9 (2004)
Anetoderma is a benign condition with focal loss of dermal elastic tissue resulting in localized areas of flaccid or herniated saclike skin. Currently, anetoderma is classified as either primary (idiopathic), or secondary anetoderma (which is associated with a variety of skin conditions, penicillamine use, or neonatal prematurity). Lesions appear on the upper arms, trunk, and thighs.
We report a 14-year-old boy, which was noticed to have had multiple, white, non-pruritic areas on the acral sites of upper and lower extremities for two years. In physical examination, the patient had normal mental development. Skin lesions consisted of scattered, white to skin-colored papules, less than 1 cm in diameter, and with central protrusion, with distribution on dorsal part of the index finger, forearms, distal portion of thighs and calves. Lesions were detected neither on the trunk nor the proximal areas of extremities. There are no sensory changes associated with the lesions. Otherwise, his general health was good. He did not have any medication consumption history. Family history was negative. Laboratory examinations were within normal limits. Skin biopsy from one of his lesions was done, that confirmed the diagnosis of anetoderma.
In summary, we report a case of anetoderma on unusual sites of the skin. We could not find similar reports of anetoderma developing on distal extremities without involvement of the upper trunk and proximal arms, in the medical literature.
The term anetoderma (anetos = slack) refers to a circumscribed area of slack skin associated with a loss of dermal substance on palpation and a loss of elastic tissue on histological examination .
In the past, cases of primary anetoderma were divided into the Jadassohn-Pellizari type, in which the lesions are preceded by erythema or urticaria, and the Schweninger-Buzzi type, in which there are no preceding inflammatory lesions. This is now of historical interest only, because in the same patient some lesions may be preceded by inflammation and the others may not, and the prognosis and histology are identical in the two types [2, 3].
Anetoderma is a rare disorder that in the most usual form develop on the trunk, thighs and upper arms, less commonly on the neck and face and rarely elsewhere.
The scalp, palms and soles are usually spared. We report a patient with anetoderma whose lesions present on distal extremities consisting of hands and calves.
A 14-year-old boy was noticed to have had multiple white, non pruritic area on his distal extremities for two years. The lesions consisted of whitish papules and depressed areas with central protrusion.
On clinical examination, the otherwise healthy looking patient's general appearance and mental state had lumbar lordosis, laxity in large joints, tibia vara, high-arched palate, and dental misalignment. Skin lesions consisted of scattered, white to skin-colored papules, less than 1 centimeter in diameter, and with central protrusion, that were distributed on the dorsum of fingers (Fig 1), forearms (Fig 2), distal portion of the thighs and on the calves (Fig 3). No lesions on the trunk or proximal areas of extremities were detected. Palms, soles, dorsum of feet and mucosal membranes were spared. No sensory changes associated with the lesions. He did not have any history of medication consumption. Family history was negative.
Laboratory examinations consisting of complete blood count, urinalysis, and blood chemistry including erythrocyte sedimentation rate and liver function tests were within normal limits. Antinuclear antibody was negative. The patient had no risk factor for AIDS or syphilis, so we did not request HIV or VDRL test. Hepatitis B surface antigens were not detected. Immunological assays consisting of IgM, IgA, IgG, IgE, C3, and C4 levels were normal. An induration of 0.5 centimeter in diameter was observed after tuberculin testing. Chest x-ray film was normal. The skin biopsy was done. Haematoxylin and eosin stained section showed faintly eosinophilic separated collagen fibers in the upper and mid-dermis (Fig 6). Verhoeff-vanGieson stained sections showed a marked decrease or in some areas total absence of elastic fibres, in both superficial and mid-dermis. Elastic fibres around the vessels in the affected areas are also fragmented and markedly decreased (Fig 7,8). We tried to treat the patient with liquid nitrogen cryotherapy by means of cotton-tip applicator for two freeze-thaw cycles (freeze time, 10–15 seconds), in 6–8 sessions weekly; which obtained moderate improvement in some early-onset lesions with no frank atrophy (Fig 4 and 5).
Anetoderma, which was first described by Jadassohn in 1892, is characterized by localized areas of loss of substance and elastic tissue with flaccid skin and often leads to a herniation phenomenon . We could not find similar reports (other than anetoderma-like changes on distal extremities secondary to hamartomatous congenital melanocytic naevi)  of anetoderma developing on distal extremities without involvement of the upper trunk and proximal arms, in the medical literature.
This rare disorder occurs mainly in women aged 20–40 years, but is occasionally reported in younger and older patients of both sexes. It is perhaps more frequent in central Europe than elsewhere, which suggests a possible relationship to chronic atrophic acrodermatitis (due to Borrelia species) in some cases. In the most usual form, crops of round or oval, pink macules 0.5–1 centimeter in diameter develop on trunk, thighs and upper arms, less commonly on the neck and face and rarely elsewhere. The scalp, palms and soles are usually spared. Each macule extends for a week or two to reach the size of 2–3 centimeter . Sometimes there are larger plaques of erythema, and nodules have also been reported as primary lesion . The number of lesions varies widely, from less than five to one hundred or more . The lesions remain unchanged throughout life, and new lesions often continue to develop for many years. If the lesions coalesce, they form large atrophic areas, which are indistinguishable from acquired cutis laxa . They may become confluent, to cover large areas, especially at the roots of the limbs and on the neck .
Although infrequently reported, anetoderma may occur in families, and patient must be examined for associated systemic abnormalities for thorough assessment of their skin disorders. In familial anetoderma, there were associated ocular, gastrointestinal or orthopedic anomalies in the affected patients or in any other family members, but causes without them have been reported . Although isolated and perhaps coincidental, these abnormalities could be related to the same process that produces the lesions of anetoderma .
Primary anetoderma can be inherited, but it has also been described in association with prematurity, lupus erythematosus, antiphospholipid syndrome, and with decreased serum levels of alpha-1-antitrypsin [6, 7, 9, 10] and . Secondary anetoderma develops over other dermatoses are shown in Table 1.
The differential diagnosis of anetoderma includes other focal dermal atrophies and miscellaneous diseases that must be differentiated from the skin herniation phenomenon of anetoderma , are shown in Table 2.
Atrophoderma of Pasini and Pierini is a major source of confusion both etymologically and clinically. Patients have larger lesions with a sharp peripheral border dropping into a depression with no outpouching. On biopsy, elastin is normal, while collagen may be thickened, but this finding is difficult to quantify . Perifollicular atrophoderma is most prominent on the dorsa of the hands and often is associated with multiple basal cell carcinomas and hair abnormalities in the Bazex syndrome . Perifollicular atrophy also has been described in extreme forms of keratosis pilaris, in which large keratin plugs may produce a dilated patulous follicle. This condition usually found on the cheeks of young children. Both of these lesions mimic perifollicular anetoderma but lack elastin changes . In focal dermal hypoplasia thinning or absence of dermis, rather than changes in elastin fibres, accounts for the proximity of the subcutis to the epidermis . Cutis laxa, postinflammatory elastolysis , and mid-dermal elastolysis  share with anetoderma the property of cryptogenic loss of elastic fibres.
Elastase-producing strains of staphylococcus epidermidis have been held responsible for perifollicular macular atrophy. Anetoderma has also been reported in 5 patients with false-positive syphilis serology, 3 of who also fulfilled the criteria for the antiphospholipid syndrome . Its pathogenesis is not yet clearly established, but immunological mechanisms could play an important role in dermal elastolysis . The association of primary anetoderma with decreased levels of alpha-1-antitrypsin may be of significance: Alpha-1-antitrypsin inhibits neutrophil elastase and its reduction may cause increased elastic activity and elastin breakdown. Phagocytosis of elastic fibres by macrophages has been found in primary anetoderma . No antibodies have been demonstrated against elastic fibres .
Venencie et al. , suggested that the degradation of elastic fibres in patients with anetoderma is caused by enhanced expression of progelatinases A and B and production of the activated form of gelatinase A, and that the lack of control of these enzymes by tissue inhibitors of metalloproteinases is probably a key factor in the development and duration of anetodermic lesions.
Ghomrasseni et al. , demonstrated that for the five samples of anetodermic skin, matrix metalloproteinase-1 (MMP-1) levels were significantly higher compared with the uninvolver cultures and the healthy samples. A significant increase of tissue inhibitors of metalloproteinase (TIMP-1) expression was also observed in the affected cultures of explants. The study demonstrated a significant increase in the production of gelatinase A (MMP-2), and no significant production of TIMP-2 in lesional skin compared with the samples from the two healthy donors.
Penicillamine-induced anetoderma has also been reported [3, 34] and . Penicillin and the antifibrinolytic drug ε-aminocaproic acid have been advocated , but Venencie et al.  studied 16 patients and found that no treatment was beneficial once the atrophy had developed. However, the wrinkled skin appearance in our patient had been present for 2 years, and his lesions did not show any signs of inflammation or pre-existing conditions like melanocytic naevi.
In summary, we report a case of anetoderma with lesions on unusual sites. We did not find similar reports of acral anetoderma in the medical literature.
According to this paper liquid nitrogen cryotherapy has moderate efficacy in the treatment of some of the early anetoderma lesions, without frank atrophy.
Burton JL, Lovell CR: Anetoderma. In: Textbook of Dermatology (Edited by: Champion RH, Burton JL, Burns DA, Breathnach SM). Blackwell Science Ltd London 1998, 2012–4.
Venencie PY, Winkelmann RK: Histopathologic findings in anetoderma. Arch Dermatol 1984, 120: 1040–4. 10.1001/archderm.120.8.1040
Venencie PY, Winkelmann RK, Moore BA: Anetoderma: clinical findings, associations, and long term follow-up evaluations. Arch Dermatol 1984, 120: 1032–9. 10.1001/archderm.120.8.1032
Cockayne SE, Gawkrodger DJ: Hamartomatous congenital melanocytic nevi showing secondary anetoderma-like changes. J Am Dermatol 1998, 39: 843–5.
Indianer L: Anetoderma of Jadassohn. Arch Dermatol 1970, 102: 697–8.
Stephansson EA, Niemi KM: Antiphospholipid antibodies and anetoderma: are they associated? Dermatology 1995, 191: 204–9.
Hodak E, Shamai-Lubovitz O, David M, Hazaz B, Katzenelson-Weissman V, Lahav M, et al.: Immunological abnormalities associated with primary anetoderma. Arch Dermatol 1992, 128: 799–803. 10.1001/archderm.128.6.799
Friedman SJ, Venencie PY, Bradley RR, Winkelmann RK: Familial anetoderma. J Am Acad Dermatol 1987, 16: 341–5.
Peterman A, Scheel M, Pandya AG: Hereditary anetoderma. J Am Acad Dermatol 1996, 35: 999–1000. 10.1016/S0190-9622(96)90134-6
Prizant TL, Lucky AW, Frieden IJ, Burton PS, Sanrez SM: Spontaneous atrophic patches in extremely premature infants. Arch Dermatol 1996, 132: 671–4. 10.1001/archderm.132.6.671
Disdier P, Harle JR, Andrac L, Verrot D, Bolla G, San Marco M, et al.: Primary anetoderma associated with the antiphospholipid syndrome. J Am Acad Dermatol 1994, 30: 133–4.
Goltz Rw, Burgdorf WHC: Anetoderma (macular atrophy) and acrodermatitis chronica atrophicans. In: Dermatology in General Medicine 2 Edition (Edited by: Fitzpatrick TB, Eisen AZ, Wolff K). New York, McGraw-Hill Book Co 1979, 696–700.
Viksnins P, Berlin A: Follicular atrophoderma and basal cell carcinomas. Arch Dermatol 1977, 113: 948. 10.1001/archderm.113.7.948
Lewis PG, Hood AF, Barnett NK, et al.: Postinflammatory elastolysis and cutis laxa. J Am Acad Dermatol 1990, 22: 40–48.
Rudolph RI: Mid-dermal elastolysis. J Am Acad Dermatol 1990, 22: 203–206.
Stanton O: Anetoderma. In: Cutaneous Medicine and Surgery (Edited by: Arndt KA, Robinson JK, LeBoit PE). Philadelphia, WB Saunders Co 1996, 910–12.
Thivolet J, Cambazard F, Souteyrand P, et al.: Les mastocytoses ae'volution ane'todermique (revue de la litte'rature). Ann Dermatol Venereal 1981, 108: 259–66.
Kelly SE, Humpherys F, Aldridje RD: The phenomenon of anetoderma occurring over pilomatriamas. J Am Acad Dermatol 1993, 28: 511.
Jones CC, Tschen JA: Anetodermic cutaneous changes overlying pilomatricomas. J Am Acad Dermatol 1991, 25: 1072–6.
Hirschel-Scholz , Salmon D, Merot Y, Saurat JH: Anetodermic prurigo nodularis (with pautrior's neuroma) responsive to arotinoid acid. J Am Acad Dermatol 1991, 25: 437–42.
Jubert C, Cosnes A, Wechsler J, Andre P, Revuz J, Bagot M: Anetoderma may reveal cutaneous plasmacytoma and benign cutaneous lymphoid hyperplasia. Arch Dermatol 1995, 131: 365–6. 10.1001/archderm.131.3.365
Tatnall R, Rycrof TR: Pityriasis versicolor with cutaneous atrophy. Clin Exp Dermatol 1985, 10: 258–61.
Gebauer KA, Navaratnam TE, Holgate C: Pruritic pigmented papules posing permanent problems. Urticaria pigmentosa (UP) with secondary anetoderma. arch Dermatol 1992, 128: 1071. 10.1001/archderm.128.1.107
Olive MP, Spielvogel RL: Anetoderma: anetoderma, the primary type. Arch Dermatol 1993, 129: 106–7-109–10.
Özkan Ş, Fetil E, Izler F, Pabucçuoğlu U, Yalçin N, Guneş AT: Anetoderma secondary to generalized granuloma annulare. J Am Acad Dermatol 2000, 42: 335–8. 10.1016/S0190-9622(00)90106-3
Daoud MS, Dicken CH: Anetoderma after hepatitis B immunization in two siblings. J Am Acad Dermatol 1997, 36: 341–5.
Herrero-Gonzalez JE, Herrero-Mateu C: Primary anetoderma associated with primary Sjogren's syndrome. Lupus 2002, 11: 124–6. 10.1191/0961203302lu139cr
Shames BS, Nassif A, Bailey CS, Seltzstein SL: Secondary anetoderma involving a pilomatricomas. Am J Dermatopathol 1994, 16: 557–60.
Stephansson EA, Niemi K-M: Antiphospholipid antibodies and anetoderma: are they associated? Dermatology 1995, 191: 204–9.
Ryll-Nardzewski C: Remarques sur le lupus érythémate profound et sur l'anetodermie érythématoide. Ann Dermatol Syphiligr 1960, 87: 627–36.
Schnitzler L, Sayag J: Pseudotumoral lupus anetoderma and infantile chorea. Ann Dermatol Vénéréol 1988, 115: 679–85.
De Bracco MM, Bianchi CA, Bianchi O, et al.: Hereditary complement (C2) deficiency with discoid lupus erythematosus and idiopathic anetoderma. Int J Dermatol 1979, 18: 713–15.
Page EH, Assaad M: atrophic dermatofibroma and dermatofibrosarcoma protuberans. J Am Acad Dermatol 1987, 17: 947–50.
Davis W: Wilson's disease and Penicillamine-induced anetoderma. Arch Dermatol 1977, 113: 976–7.
Alvarez-Cuesta CC, Raya-Aguado C, Fernandez-Rippe ML, Sanchez TS, Perez-Olive N: Anetoderma in a systemic lupus erythematosus patient with anti-PCNA and antiphospholipid antibodies. Dermatology 2001, 203: 348–50. 10.1159/000051789
Zaki I, Scerri L, Nelson H: Primary anetoderma: phagocytosis of elastic fibers by macrophages. Clin Exp Dermatol 1994, 19: 388–90.
Bechelli LM: Anetoderma in leprosy. Dermatologica 1967, 135: 329.
Venencie PY, Bonnefoy A, Gogly B, et al.: Increased expression of gelatinases A and B by slin explants from patients with anetoderma. Br J Dermatol 1997, 137: 517–25.
Ghomrasseni S, Dridi M, Gogly B, et al.: Anetoderma: an altered between metalloproteinases and tissue inhibitors of metalloproteinases. Am J Dermatopathol 2002, 24: 118–29. 10.1097/00000372-200204000-00003
Prizant TL, Lucky AW, Frieden IJ, et al.: Spontaneous atrophic patches in extremely premature infants: Anetoderma of prematurity. Arch Dermatol 1996, 132: 671–4. 10.1001/archderm.132.6.671
Reiss F, Linn E: The therapeutic effect of a ε-aminocaproic acid on anetoderma of Jadassohn. Dermatologica 1973, 146: 357–60.
Ruiz-Rodriguez R, Longaker M, Berger TG: Anetoderma and human immunodeficiency virus infection. Arch Dermatol 1992, 128: 661–62. 10.1001/archderm.128.5.661
The pre-publication history for this paper can be accessed here:http://www.biomedcentral.com/1471-5945/4/9/prepub
We would like to thank Dr. N. Shokrpour for editing the manuscript. Also we thank patient's family for permission us to publish the patient's details and photograph.
Authors’ original submitted files for images
Below are the links to the authors’ original submitted files for images.
About this article
Cite this article
Aghaei, S., Sodaifi, M., Aslani, F.S. et al. An unusual presentation of anetoderma: a case report. BMC Dermatol 4, 9 (2004). https://doi.org/10.1186/1471-5945-4-9