Background

Anetoderma refers to localized, circumscribed areas of cutaneous atrophy that are often only slightly discolored, and slightly wrinkled. It is caused by loss of elastic tissue fibers in the mid-reticular to papillary dermis.

OUTLINE

Epidemiology
Disease Associations
Pathogenesis
Laboratory/Radiologic/Other Diagnostic Testing
Gross Appearance and Clinical Variants
Histopathological Features and Variants
Special Stains/ Immunohistochemistry/ Electron Microscopy
Differential Diagnosis
Prognosis
Treatment
Commonly Used Terms
Internet Links

EPIDEMIOLOGY	CHARACTERIZATION
SYNONYMS	Anetoderma of Jadassohn-Pellizzari.
INCIDENCE	Rare

 

DISEASE ASSOCIATIONS	CHARACTERIZATION
ANTIPHOSPHOLIPID ANTIBODY SYNDROME/ THROMBOSIS
Anetodermic lupus panniculitis and antiphospholipid antibodies: report of three cases. Marzano A, Vanotti M, Alessi E. Institute of Dermatological Sciences of the University of Milan, Milan, Italy.	: Acta Derm Venereol. 2004;84(5):385-8. Abstract quote   Anetoderma is a rare cutaneous disease characterized by a loss of normal elastic tissue that is presented clinically as localized areas of wrinkled or flaccid skin. This form may be associated with several immunological abnormalities, most notably lupus erythematosus and antiphospholipid antibodies with or without clinical manifestations of the antiphospholipid syndrome. A retrospective study was conducted with the aim of summarizing the clinical characteristics, course and laboratory findings in three women with anetoderma-associated lupus erythematosus panniculitis, an unusual variant of cutaneous lupus erythematosus. The 3 patients (of the 12 patients with lupus erythematosus panniculitis seen by us since 1990) were all at a young age at onset of panniculitis (median, 22 years). None of the patients developed severe systemic involvement up to 9 years (median, 5 years) from onset of the disease. The most noteworthy laboratory finding was the presence of antiphospholipid antibodies. Anetodermic lupus erythematosus panniculitis may be regarded as an uncommon variant of cutaneous lupus erythematosus mainly affecting young females and showing a favourable clinical course, although the patients should be followed and screened for the emergence of antiphospholipid syndrome. Antiphospholipid antibodies could play a role in the elastolytic process, leading to anetoderma.
Anetoderma and its prothrombotic abnormalities. Sparsa A, Piette JC, Wechsler B, Amoura Z, Frances C. Department of Internal Medicine, University Hospital Pitie, 83 Boulevard de l'Hopital, 75651 Paris Cedex 13, Paris, France.	J Am Acad Dermatol. 2003 Dec;49(6):1008-12. Abstract quote   BACKGROUND: Anetoderma is characterized by circumscribed areas of flaccid skin due to the loss of elastic tissue in the dermis. It may be primary or secondary to various dermatoses. The primary form has been reported in association with autoimmune diseases and recently with antiphospholipid antibodies. Its etiology remains unknown. OBJECTIVES: To analyze clinical and laboratory data from a series of patients with anetoderma referred in our university reference center for connective tissue disorders. PATIENTS AND METHODS: All the consecutive patients with histologically confirmed anetoderma followed in our clinic from 1996 to 2001 were enrolled in this study. Laboratory investigations included the screening for prothrombotic abnormalities and classical immunological investigations for systemic lupus erythematosus. Clinical and laboratory data were analyzed retrospectively. RESULTS: Anetoderma was primary in 9 cases and secondary to lupus profundus in 2 cases. Prothrombotic abnormalities were detected in 10 patients (9/9 with primary and 1/2 with secondary anetoderma). Antiphospholipid antibodies were detected in 9 patients. Only 4 patients fulfilled criteria for definite antiphospholipid syndrome which was primary for 3 and associated with systemic lupus erythematosus in the other. CONCLUSION: Patients with anetoderma should be evaluated for the possible presence of a prothrombotic state and warned of its potential risks when present.
Increased anticardiolipin antibodies associated with the development of anetoderma in HIV-1 disease. Military Medical Consortium for the Advancement of Retroviral research (MMCARR). Lindstrom J, Smith KJ, Skelton HG, Redfield R, Alving BM, Wagner KF, Lupton GP. Dermatology Service, Walter Reed Army Medical Center, Washington, District of Columbia, USA.	Int J Dermatol 1995 Jun;34(6):408-15 Abstract quote BACKGROUND AND OBJECTIVE. Anetoderma has been reported in patients with HIV-1 disease. In patients with autoimmune disease, anetoderma has been associated with increased levels of antiphospholipid antibodies (APL) that include anticardiolipin antibodies (ACA) and lupus anticoagulant (LA). This has led to speculation that the autoimmune phenomena seen in HIV-1 disease and the immune dysregulation induced by HIV-1 disease may play a role in the development of these lesions. We have seen both primary and secondary lesions of anetoderma in patients followed for HIV-1 disease. In this study, we wanted to determine whether there was an association in the development of anetoderma and elevated anticardiolipin antibodies (ACA) in HIV-1 patients. METHODS. Quantitative ACA levels were measured in eight HIV-1-infected patients with anetoderma and four HIV-1-infected patients without anetoderma. RESULTS. Anticardiolipin antibodies were moderately elevated in seven of eight patients with lesions and were borderline in the four HIV-1-positive patients without lesions of anetoderma. CONCLUSIONS. There appears to be a correlation between increased ACA and the development of cutaneous lesions of anetoderma in HIV-1 disease. Patterns of immune dysregulation, including APL, may predispose to the development of lesions of anetoderma in HIV-1-positive patients. Although some of the lesions appear to represent primary anetoderma, the majority of our patients develop lesions in areas secondary to well characterized eruptions.
Anetoderma associated with antiphospholipid antibodies: case report and review of the literature. Romani J, Perez F, Llobet M, Planaguma M, Pujol RM. Department of Dermatology, Hospital de Palamos, Girona, Spain.	J Eur Acad Dermatol Venereol 2000 Mar;15(2):175-8 Abstract quote The association of primary anetoderma (PA) with antiphospholipid antibodies (APAs; with or without criteria of primary antiphospholipid syndrome) has been observed repeatedly and a possible pathogenic significance of this asssociation has been hypothesized. We report the case of a 21-year-old-woman who developed anetodermic lesions on her upper trunk and arms. The presence of APAs was demonstrated, but no diagnostic criteria of systemic lupus erythematosus were present. A review of the literature disclosed 20 previously reported patients presenting this peculiar association. Thrombotic phenomena, represented by spontaneous abortions or venous thrombosis, are a frequent complication in this setting. Taking these observations into account a systematic search for APAs in patients with PA seems advisable.
AUTOIMMUNE DISORDERS
Primary anetoderma associated with a wide spectrum of autoimmune abnormalities. Hodak E, Shamai-Lubovitz O, David M, Hazaz B, Lahav M, Sandbank M. Department of Dermatology, Beilinson Medical Center, Petah Tiqva, Israel.	J Am Acad Dermatol 1991 Aug;25(2 Pt 2):415-8 Abstract quote Although the underlying pathologic mechanisms of primary anetoderma have not yet been identified, data suggest the participation of an immunologic mechanism in some cases. In a woman with clinical and histopathologic features of primary anetoderma (Jadassohn-Pellizzari type) of 30 years' duration, laboratory investigation disclosed positive antinuclear factor, hypocomplementemia, hypergammaglobulinemia, granular deposits of immunoreactants along the dermoepidermal junction, and fibrillar deposits in the papillary dermis. In addition, she was found to have autoimmune hemolysis and circulating lupus anticoagulant associated with recurrent deep-vein thrombosis and a history of Graves' disease (starting 5 years after onset of primary anetoderma). To our knowledge, none of the latter three autoimmune conditions has been previously associated with primary anetoderma.
CONGENITAL NEVI
Hamartomatous congenital melanocytic nevi showing secondary anetoderma-like changes. Cockayne SE, Gawkrodger DJ. Department of Dermatology, Royal Hallamshire Hospital, Sheffield, United Kingdom.	J Am Acad Dermatol 1998 Nov;39(5 Pt 2):843-5 Abstract quote A 47-year-old man had multiple large congenital melanocytic nevi associated with striking atrophic changes. Biopsy specimens showed a prominent reduction and fragmentation of elastic fibers in the papillary dermis. The anetoderma-like changes may have been secondary to a past inflammatory process but more likely represent a hamartoma.
HIV-1
Anetoderma and human immunodeficiency virus infection. Ruiz-Rodriguez R, Longaker M, Berger TG. Department of Dermatology, San Francisco General Hospital, CA 94110.	Arch Dermatol 1992 May;128(5):661-2 Abstract quote BACKGROUND--Autoimmune disorders such as vitiligo and alopecia areata have been reported in persons infected with the human immunodeficiency virus (HIV). OBSERVATIONS--Three HIV-infected men had anetoderma develop early in the course of their HIV infection. In two patients this preceded other features of HIV disease, and in the third it occurred while the helper T-cell count exceeded 200/mm3. CONCLUSIONS--These findings suggest that anetoderma may be an early manifestation of HIV disease, perhaps autoimmune in its pathogenesis.
JUVENILE XANTHOGRANULOMA
Anetoderma developing in Juvenile Xanthogranuloma. Gamo R, Ortiz-Romero P, Sopena J, Guerra A, Rodriguez-Peralto JL, Iglesias L. From the Department of Dermatology and Pathology, Hospital 12 de Octubre, Madrid, Spain.	Int J Dermatol. 2005 Jun;44(6):503-6. Abstract quote   Anetoderma is characterized by circumscribed oval macules with overlying wrinkled skin that is slightly depressed or bulges outwards. Skin biopsy shows a decrease of elastic dermal fibers. It may not be associated with an underlying disease (primary anetoderma) or may be related to many dermatoses (secondary anetoderma). We report a 7-year-old girl who presented at birth with yellowish brown papules on the upper trunk, neck and head, which within days evolved to yellowish orange papules. A skin biopsy was carried out and the presence of an histiocytic infiltrate with foam cells and Touton cells in the dermis that were CD68+, factor XIII+ and S-100-, confirmed the diagnosis of Juvenile Xanthogranuloma (JXG). After 4 years the lesions began to evolve to asymptomatic oval and round atrophic skin areas. Histopathologic evaluation showed decrease of elastic fibers in the dermis, diagnostic of anetoderma. The mechanisms of anetoderma are unknown. Although many different dermatoses have been associated with anetoderma we have only found two reported cases of anetoderma and JXG.
LYME DISEASE
Anetoderma and borreliosis: is there a pathogenetic relationship? Hofer T, Goldenberger D, Itin PH.	Eur J Dermatol. 2003 Jul-Aug;13(4):399-401. Abstract quote   A 32-year-old man simultaneously developed anetoderma and acrodermatitis chronica atrophicans on his left arm and showed a positive serology for borreliosis with ELISA and Western Blot tests. In addition, a 45 year-old man is presented with anetoderma without any associated systemic or cutaneous diseases, with B. afzelii confirmed as a singular causality through serology (ELISA, Western Blot) and amplification of B. afzelii-specific DNA from the skin by PCR. These two observations highly suggest that anetoderma can be the result of an infection with B. afzelii. We conclude that in patients with anetoderma a serological investigation for Borreliosis should be performed.
LYMPHOMA, SKIN
Multiple cutaneous immunocytoma with secondary anetoderma: a report of two cases. Child FJ, Woollons A, Price ML, Calonje E, Russell-Jones R. Skin Tumour Unit, St John's Institute of Dermatology, St Thomas' Hospital, London SE1 7EH, U.K.	Br J Dermatol 2000 Jul;143(1):165-70 Abstract quote We describe two men with multiple erythematous dermal nodules which were clinically and histologically consistent with a diagnosis of primary cutaneous immunocytoma. Both patients exhibited the very unusual feature of secondary anetoderma occurring in spontaneously resolving lesions. There is one previous report of anetoderma in association with a plasmacytoma. The pathogenesis remains unknown but release of cytokines such as interleukin-6 may be implicated.
Anetoderma Arising in Cutaneous B-Cell Lymphoproliferative Disease Richard C. Kasper, etal.	Am J Dermatopathol 2001;23:124-132 Abstract quote Anetoderma is circumscribed atrophy of the skin due to a localized deficiency in elastic tissue. It can follow inflammatory skin diseases of several types, and occasionally is present in the skin around neoplasms. There are a few reports of anetoderma in the lesional skin of cutaneous lymphoma. We report on two patients who presented with multiple lesions of anetoderma and who later proved to have low-grade cutaneous B-cell lymphomas. One patient (Patient 1) is a 39-year-old man and the other patient is a 26-year-old woman who is a renal transplant recipient (Patient 2). Some biopsy specimens from the anetodermic skin of Patient 1 appeared to show an urticarial reaction, although plasma cells were present. A large nodule showed lymphoid follicles surrounded by plasmacytoid lymphocytes, with loss of elastic tissue in the adjacent dermis. The plasmacytoid cells stained overwhelmingly for lambda light chain, and staining of the urticarial lesions from this patient also showed a marked majority of lambda positive cells. Immunoglobulin heavy chain gene (IgH) rearrangements showed a dominant clonal pattern in the nodular lesion. We classified the disease in Patient 1 as marginal zone lymphoma and the disease in Patient 2 as a post-transplant lymphoproliferative disorder. Because of the intimate association of anetoderma and cutaneous B-cell lymphoproliferative disorders in these two patients, it seems possible that anetoderma could result from either a local effect of the neoplastic cells or associated inflammatory cells, especially neutrophils as in Case 1. The infiltrates of Case 1 had many interstitial neutrophils and only a few clonal plasmacytoid lymphocytes, indicating that this presentation of B-cell lymphoma can be a diagnostic pitfall. Given these two cases and similar ones in the literature, biopsy of lesional skin in anetoderma should be performed to ensure that lymphomatous infiltrates are not present. Even if plasma cells are sparse, studies to detect clonality are appropriate. Cutaneous B-cell lymphoma can be added to the list of associations of elastolysis and cutaneous lymphoma, which includes granulomatous slack skin (T-cell lymphoma) and cutis laxa (myeloma).
PILOMATRICOMA
Secondary anetoderma involving a pilomatricoma. Shames BS, Nassif A, Bailey CS, Saltzstein SL. Department of Dermatology, State University of New York, Brooklyn.	Am J Dermatopathol 1994 Oct;16(5):557-60 Abstract quote We describe an 11-year-old girl with secondary anetoderma involving a pilomatricoma. She presented with a soft, wrinkled pedunculated lesion overlying a firm subcutaneous mass on her right anterior shoulder. Pathologic examination revealed a pilomatricoma in the subcutaneous tissue, with focal loss of elastic fibers in the overlying dermis. Secondary anetoderma has been reported to involve various infections, inflammatory disorders, and tumors, but the association with pilomatricoma is very rare.
SYSTEMIC LUPUS ERYTHEMATOSUS
Anetoderma associated with antiphospholipid syndrome and systemic lupus erythematosus. Bilen N, Bayramgurler D, Sikar A, Ercin C, Yilmaz A. Department of Dermatology, Kocaeli University School of Medicine, Izmit, Turkey.	Lupus. 2003;12(9):714-6. Abstract quote   Anetoderma is an uncommon disorder characterized by the loss of elastic fibres in the dermis histologically and herniation of subcutaneous tissue clinically. Recent studies indicate that immunologic mechanisms may play a role in this process. Here we report a 33-year-old woman with numerous well-circumscribed, asymptomatic skin lesions in whom clinical and histopathologic features were consistent with anetoderma. Additionally, history and investigations revealed antiphospholipid syndrome and systemic lupus erythematosus. It has been speculated that immune deposits in the dermis or within the capillary walls may lead to ischaemia and subsequent degeneration of the elastic fibres.
Anetoderma in a systemic lupus erythematosus patient with anti-PCNA and antiphospholipid antibodies. Alvarez-Cuesta CC, Raya-Aguado C, Fernandez-Rippe ML, Sanchez TS, Perez-Oliva N. Department of Dermatology, Hospital Central de Asturias, University of Oviedo, Spain.	Dermatology 2001;203(4):348-50 Abstract quote Anetoderma is a rare elastolytic disorder included within the group of cutaneous atrophies. Its pathogenesis is not yet clearly established, but immunological mechanisms could play an important role in dermal elastolysis. It has been associated with different autoantibodies and autoimmune disorders. We present a case of anetoderma in a systemic lupus erythematosus patient with anti-proliferating-cell-nuclear-antigen and antiphospholipid antibodies, highlighting the peculiarities of such an association.
Anetoderma in systemic lupus erythematosus: relationship to antiphospholipid antibodies. Montilla C, Alarcon-Segovia D. Department of Immunology and Rheumatology, Instituto Nacional de la Nutricion Salvador Zubiran, Mexico City, Mexico.	Lupus 2000;9(7):545-7 Abstract quote Anetoderma is an elastolytic disorder where multiple patches of slack skin are formed. Twelve patients with anetoderma associated with systemic lupus erythematous have been described, all in the dermatological literature. Recently, a role for antiphospholipid antibodies has been proposed with microthromboses as its pathogenic mechanism. We present herein a 20-year-old female patient who developed anetoderma soon after sun exposure. She was found to have a false positive VDRL and gradually developed other manifestations of SLE, including interstitial cystitis. She has had repeatedly positive antiphospholipid antibodies. Although there are patients who may have a primary form, diagnosis of anetoderma should trigger a search for SLE and/or antiphospholipid antibodies.
TAKAYASU'S ARTERITIS
Postgranulomatous anetoderma associated with Takayasu's arteritis in a child. Taieb A, Dufillot D, Pellegrin-Carloz B, Calabet A, Clementy J, Guillard JM, Maleville J.	Arch Dermatol 1987 Jun;123(6):796-800 Abstract quote Takayasu's arteritis (TA) is a rare chronic inflammatory arteriopathy affecting mainly the aorta and its branches. Many skin manifestations have been reported in association with this disease. Pyoderma gangrenosum and subcutaneous inflammatory lesions of the leg are the most frequent. We studied a boy with TA in whom a papular rash of the trunk preceded the onset of vascular symptoms by many years. Histologically, the lesions were superficial and consisted of middermal noncaseating tuberculoid granulomas, which progressed to atrophy and anetoderma because of elastic network disruption. Granulomas were also found in synovial tissue but not in a temporal artery biopsy specimen, which showed only intimal hyperplasia. Our observations suggest that vascular and skin lesions with elastic tissue may both result from a common granulomatous hypersensitivity process.
XANTHOMA
Primary papular xanthoma of children: a clinicopathologic, immunohistopathologic and ultrastructural study. Chen CG, Chen CL, Liu HN. Department of Dermatology, National Yang-Ming University and Veteran General Hospital, Taipei, Taiwan, R.O.C.	Am J Dermatopathol 1997 Dec;19(6):596-601 Abstract quote Papular xanthoma (PX) is a very rare skin disorder. We describe a typical case of PX in a 13-month-old Chinese boy who presented with numerous yellow-red papulonodules, 2-8 mm in diameter, mainly on the face, both upper extremities, and abdomen of 10 months duration. Histologic studies showed a diffuse monomorphous infiltrate of foamy cells in the upper dermis. The foamy cells stained positively with oil red O and CD68. The periodic acid Schiff (PAS) stain, S-100 protein, CD1a, CD56, lysozyme, alpha1-antitrypsin, and factor XIIIa were all negative in the foamy cells. The electron microscopic (EM) studies revealed the morphologic features of macrophages with electron-dense, membrane-limited lipid vacuoles in the cytoplasm. After 14 months, neither spontaneous regression nor anetoderma-like scars were noted. Our immunohistochemical and ultrastructural studies support the notion that the origin of the foamy cells is the macrophage rather than the factor XIIIa (+) dermal dendrocyte. There was no associated or underlying disease in this case. We suggest the term primary PX for cases such as this one.

 

PATHOGENESIS	CHARACTERIZATION
BACKGROUND	Increased elastases, decreased elastin production, and decreased elastase inhibitors are postulated Elastases most likely produced by neutrophils and macrophages in cases associated with inflammatory skin diseases, and macrophages Some evidence for a role for antibodies against elastic tissue
	Br J Dermatol 1997;137:517–25. Increased levels of elastases (progelatinase A and B) as well as increased activation of progelatinase A when compared with explants of normal skin Localized imbalance of elastolytic enzymes and their inhibitors
ELASTIC FIBER DEFECT
Anetoderma: biochemical and ultrastructural demonstration of an elastin defect in the skin of three patients. Oikarinen AI, Palatsi R, Adomian GE, Oikarinen H, Clark JG, Uitto J.	J Am Acad Dermatol 1984 Jul;11(1):64-72 Abstract quote Three patients with localized cutaneous lesions characteristic of anetoderma were studied. Clinically, the onset of the disease was between the ages of 17 and 25, and numerous flaccid, saclike skin lesions developed over several subsequent years. Histologically, the lesions were characterized by paucity and fragmentation of the elastic fibers. Electron microscopy demonstrated that the elastic fibers, both in papillary and deep reticular dermis in the lesional skin, were fragmented and irregular in appearance. The concentration of elastin, determined by a radioimmunoassay of desmosine, an elastin-specific cross-link compound, was markedly reduced in the lesions, as compared with unaffected skin from the same patients or with normal skin from unrelated control subjects. In contrast, the concentrations of hydroxyproline, an index of collagen, or deoxyribonucleic acid (DNA), a measure of cellularity, were not changed in the lesions. Thus, the results indicate that in the three patients studied, the elastic fibers are defective and reduced in quantity. These observations suggest that the deficiency of elastin in the dermis may lead to development of the cutaneous lesions of anetoderma.
METALLO-PROTEINASES
Anetoderma: an altered balance between metalloproteinases and tissue inhibitors of metalloproteinases. Ghomrasseni S, Dridi M, Gogly B, Bonnefoix M, Vabres P, Venencie PY, Pellat B, Godeau G. Laboratoire de physiopathologie des tissus non mineralises, Universite Rene Descartes-Paris V, UFR d'Odontologie, 1 Rue Maurice Arnoux, 92120 Montrouge, France.	Am J Dermatopathol 2002 Apr;24(2):118-29 Abstract quote The amount of elastic fibers from lesional and healthy skin areas of five patients with anetoderma was determined by automated image analysis. Dermal elastic fibers were almost completely absent in anetodermic skin and preelastic fibers were undetectable or extremely rare. Organ cultures were performed using explants from affected and unaffected skin areas of the same patient. We identified and quantified proteases in the culture media of explants: MMP-1 (collagenase 1), MMP-2 and MMP-9 (gelatinases A and B), MMP-3 (stromelysin 1), MMP-7 (matrilysin 1), and tissue inhibitors of metalloproteinases, TIMP-1 and TIMP-2. The data were compared with those of two healthy donors. For the five samples of anetodermic skin, MMP-1 levels were significantly higher compared with the uninvolved cultures and the two healthy samples. A significant increase of TIMP-1 expression was also observed in the affected cultures. We demonstrated a significant increase in the production of gelatinase A in lesional skin when compared with nonlesional skin and healthy donor samples. We found no significant production of TIMP-2 in the five samples of anetodermic skin compared with the samples from the two healthy donors. There was a significant decrease in TIMP-2 expression in the five nonlesional samples compared with the control samples. These data are in favor of an altered balance in anetodermic patients between MMP-2 and TIMP-2. Levels of MMP-9, MMP-3, and MMP-7 were significantly higher in the culture-conditioned media of the anetodermic skin samples than the nonlesional skin cultures. Because MMP-3, MMP-7, MMP-9 are known to degrade elastin, and MMP-3 can activate the latent forms of MMP-7 and MMP-9, we propose that these metalloproteinases also participate in the degradation of elastic fibers in anetodermic skin.
PHAGOCYTOSIS
Primary anetoderma: phagocytosis of elastic fibres by macrophages. Zaki I, Scerri L, Nelson H. Department of Dermatology, University Hospital, Queen's Medical Centre, Nottingham, UK.	Clin Exp Dermatol 1994 Sep;19(5):388-90 Abstract quote We report a 22-year-old female with a 2-year history of increasingly numerous well-circumscribed, asymptomatic skin lesions. The clinical and histological features were consistent with anetoderma and investigations failed to reveal any associated underlying disorders. Electron microscopy of abnormal skin showed phagocytosis of elastic fibres by macrophages. Although the aetiology of primary anetoderma remains unknown, this finding suggests that phagocytic destruction of elastic fibres plays a major role in its pathogenesis.

 

GROSS APPEARANCE/ CLINICAL VARIANTS	CHARACTERIZATION
General
Non-inflammatory (primary) Schweninger-Buzzi type
Inflammatory (secondary) Jadassohn-Pellizari type	The best known antecedents are inflammatory skin diseases, such as lupus erythematosus, granuloma annulare, syphilis, and varicella Occasional tumors, the best described being pilomatricomas, are also associated
VARIANTS
PEDIATRIC
Primary Anetoderma in children: report of two cases and literature review. Karrer S, Szeimies RM, Stolz W, Landthaler M. Department of Dermatology, University of Regensburg, Germany.	Pediatr Dermatol 1996 Sep-Oct;13(5):382-5 Related Articles, Books, LinkOut Primary Anetoderma in children: report of two cases and literature review. Karrer S, Szeimies RM, Stolz W, Landthaler M. Department of Dermatology, University of Regensburg, Germany. Two boys, age 7 and 9 years, with the diagnosis of primary anetoderma are presented. In one patient a growing number of indolent lesions developed for one year on the neck. The other boy complained of single lesions appearing over four months on the arms, feet, and chest wall. Individual lesions measured up to 1 cm in diameter and showed a palpable herniation phenomenon and wrinkled surface. The lesions did not have an inflammatory onset. Histologically, in both patients the diagnosis of anetoderma was verified by loss of elastic fibers and a lympho-histiocytic infiltration in the middermis. Administration of oral penicillin for three weeks did not result in marked improvement, and atrophic macules continued to appear in the younger boy. Although no autoimmunologic abnormalities or other associated diseases have arisen in our patients, long-term follow-up is mandatory to detect autoimmune disorders that are reported to occur in the course of the disease.
PREMATURE INFANTS
Spontaneous atrophic patches in extremely premature infants. Anetoderma of prematurity. Prizant TL, Lucky AW, Frieden IJ, Burton PS, Suarez SM. Department of Dermatology, University of Pittsburgh Medical School, Pa, USA.	Arch Dermatol 1996 Jun;132(6):671-4 Abstract quote BACKGROUND: Anetoderma, characterized clinically by macular depressions or outpouchings of skin, is associated with loss of dermal elastic tissue as noted on histopathologic findings. We report on 9 extremely premature infants who developed patches of anetoderma during their course in the neonatal intensive care unit. OBSERVATIONS: All 9 patients were born between the ages of 24 and 29 weeks of gestation and had numerous complications associated with prematurity. Eight of the 9 infants were noted to have developed anetoderma on the trunk and proximal extremities while in the neonatal intensive care unit. The locations of the lesions on the skin were not explained by previous trauma, although many areas corresponded with placement of monitoring leads or with adhesive for a monitoring device. Reduction or absence of elastic tissue supported the diagnosis of anetoderma in 4 of 5 biopsy specimens. CONCLUSION: We report a previously unrecognized type of anetoderma associated with extreme prematurity. The exact cause is uncertain, although reactions to cutaneous monitoring leads or adhesives is suspected.
Anetoderma of prematurity in association with electrocardiographic electrodes. Colditz PB, Dunster KR, Joy GJ, Robertson IM. Perinatal Research Centre, Royal Women's Hospital, University of Queensland, Brisbane, Australia.	J Am Acad Dermatol 1999 Sep;41(3 Pt 1):479-81 Abstract quote Anetoderma in premature infants is an uncommon lesion that may be associated with the use of various types of monitoring leads. In 2 infants multiple papules of anetoderma occurred on the forehead in association with the use of gel electrocardiographic electrodes. It is postulated that the cause of these papules was a local hypoxemia caused by pressure from the electrodes. Growth-restricted infants may be particularly predisposed to iatrogenic anetoderma.

 

HISTOLOGICAL TYPES	CHARACTERIZATION
General	Sparse infiltrates of lymphocytes, plasma cells, and histiocytic giant cells are evident in routine sections Elastic stain reveals a profound loss of fibers in both the mid-reticular dermis and sometimes the papillary dermis A few fine fibers often remain around small vessels
Histopathologic findings in anetoderma Venencie PY, Winkelmann RK.	Arch Dermatol 1984 Aug;120(8):1040-4 Abstract quote We reviewed the histopathologic findings in 34 biopsy specimens from 15 patients with anetoderma. Focal loss of normal elastic fibers, necessary for the diagnosis of anetoderma, was found in all 15 cases. The persistence of fine, irregular, or twisted elastic fibers is common. A perivascular infiltrate composed of lymphocytes was found in all specimens. Plasma cells were observed in specimens from six patients, and histiocytes with some granuloma formation were found in specimens from six patients. Variations in the intensity of inflammation or in the loss of elastic tissue were not related to the clinical findings, course, or associated diseases. All anetoderma lesions appeared to have an inflammatory pathogenesis.

 

SPECIAL STAINS/ IMMUNO-HISTOCHEMISTRY	CHARACTERIZATION
DIRECT IMMUNO-FLUORESCENCE
An immunofluorescence study of primary anetoderma. Bergman R, Friedman-Birnbaum R, Hazaz B, Cohen E, Munichor M, Lichtig C. Department of Dermatology, Rambam Medical Center, Petah Tiqva, Israel.	Clin Exp Dermatol 1990 Mar;15(2):124-30 Abstract quote Primary anetoderma (PA) has occasionally been described in association with lupus erythematosus (LE). The present study was performed to elucidate a possible causal link between PA and LE by the use of direct and indirect immunofluorescence (IF) methods. Two patients with PA were studied. Biopsy specimens were obtained from early inflammatory and atrophic anetoderma lesions and from the exposed and unexposed uninvolved skin of each patient. The pattern of immune deposits observed in one patient was indistinguishable from that which is often seen in systemic LE, and in the other patient from that which may be observed in chronic cutaneous LE. The direct IF study also showed fibrillar immune deposits in the dermis that resembled elastic fibres morphologically. The indirect IF study, however, failed to demonstrate anti-elastic fibre antibodies in the patients' sera. The results of this study and a review of the literature suggest that some cases of PA have direct IF findings similar to those of either chronic cutaneous or systemic LE. However, these findings, along with the serological findings, are insufficient to establish a diagnosis of LE in most of these PA cases.
ELECTRON MICROSCOPY
Ultrastructural findings in the skin lesions of patients with anetoderma. Venencie PY, Winkelmann RK, Moore BA.	Acta Derm Venereol 1984;64(2):112-20 Abstract quote Eight biopsy specimens from the skin lesions of five patients with anetoderma were studied for their ultrastructural findings. In all of them, normal elastic fibers were absent and a few very thin, irregular elastic fibers with a more or less complete loss of the amorphous substance and a relative conservation of the microfibrils were observed. The collagen fibers were normal. Inflammation composed of macrophages and lymphocytes, with some plasma cells, was a prominent finding. It is suggested that anetoderma and acquired cutis laxa are part of the same spectrum of elastolytic disease.

 

DIFFERENTIAL DIAGNOSIS	KEY DIFFERENTIATING FEATURES
ATROPHERMA ELASTOLYTICA DISCRETA
Atrophoderma elastolytica discreta. Carrington PR, Altick JA, Sanusi ID. Department of Dermatology, Louisiana State University School of Medicine, Shreveport, USA.	Am J Dermatopathol 1996 Apr;18(2):212-7 Abstract quote Atrophoderma elastolytica discreta is the clinicopathologic name for a unique entity herein first described in a patient with cutaneous lesions simulating atrophoderma of Pasini and Pierini but coupled with histopathologic changes of anetoderma. The clinical and histological findings seen here have not been previously seen in the many variants of anetoderma, and, as such, they are sui generis evidence of a new entity in clinical dermatology.
ELASTIC FIBERS
Morphometric analysis of elastic skin fibres from patients with: cutis laxa, anetoderma, pseudoxanthoma elasticum, and Buschke-Ollendorff and Williams-Beuren syndromes. Ghomrasseni S, Dridi M, Bonnefoix M, Septier D, Gogly G, Pellat B, Godeau G. Laboratoire de physiopathologie des tissus non-mineralises, Universite Rene Descartes-Paris V, UFR d'Odontolgie, Montrouge, France.	J Eur Acad Dermatol Venereol 2001 Jul;15(4):305-11 Abstract quote Computed morphometric analysis of elastic skin fibres in patients with cutis laxa, anetoderma, Williams-Beuren syndrome, pseudoxanthoma elasticum (PXE), and Buschke-Ollendorff syndrome, all clinically ascertained, was performed and compared with data obtained from healthy individuals of the same age. The diameters, area fractions (AA%) and volume fractions (VV%) occupied by pre-elastic fibres and dermal elastic fibres were determined. Irrespective of age the diameter of dermal elastic fibres followed a Gaussian distribution for all groups studied. These diameters were taken into consideration for VV% determinations. Compared with data from skin of healthy subjects of similar age range, VV% of pre-elastic fibres was significantly decreased in patients with cutis laxa, anetoderma, Williams-Beuren syndrome, and PXE and undetectable in Buschke-Ollendorff patients. VV% of dermal elastic fibres was four- to fivefold increased in Buschke-Ollendorff syndrome, two- to threefold increased in PXE skin, four- to fivefold decreased in cutis laxa and anetoderma skin and about twofold decreased in Williams-Beuren skin. The diameter of oxytalan fibres was decreased in anetoderma and Williams-Beuren syndrome while oxytalan fibre diameter was unchanged in PXE and cutis laxa. The diameter of dermal elastic fibres was increased in PXE and Buschke-Ollendorff syndrome, but was decreased in anetoderma and Williams-Beuren syndrome and unchanged in cutis laxa. We demonstrated that cutis laxa, anetoderma, Williams-Beuren syndrome, PXE, and Buschke-Ollendorff syndrome could be easily differentiated by morphometric analysis of elastic skin fibres. Thus we propose that morphometric analyses together with skin biopsies are a valuable tool for distinguishing between inherited and/or acquired skin diseases known to display alterations of elastic fibres.

 

PROGNOSIS AND TREATMENT

CHARACTERIZATION

Anetoderma. Clinical findings, associations, and long-term follow-up evaluations. Venencie PY, Winkelmann RK, Moore BA.

Arch Dermatol 1984 Aug;120(8):1032-9 Abstract quote In 16 patients with anetoderma, a clinicohistologic entity related to a local dermal defect of elastic tissue, old lesions did not heal, and new lesions often continued to form for many years, despite various forms of treatment. Systemic lupus erythematosus, which occurred in one patient, must be ruled out in patients with anetoderma. Discoid lupus erythematosus occurred later in one patient. Other associated findings, noted in one patient each, included cataract, congenital hip dislocation, congenital fusion of the vertebrae at C2-3, diverticulum of the midesophagus, Addison's disease (before the onset of anetoderma), and mitral valve prolapse. Our results and a review of the literature indicate that patients with anetoderma must be examined for associated eye, bone, heart, pulmonary, digestive tract, and endocrine abnormalities for a better assessment of their skin disorders.

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