Primary Anetoderma and Acquired Cutis Laxa associated with Glomerulonephritis in a 37-year-old Filipino male: A case report

@#A 37-year-old Filipino man presented with a 9-month history of sagging skin progressing cephalocaudally from the chin and neck to the axillae, side of the trunk, and pelvic area. This was followed by a 2-month history of increasing serum creatinine levels associated with periorbital and bipedal edema, generalized weakness, decreased appetite, vomiting, and headache. Subsequently, skin-colored, non-tender sac-like plaques appeared on the abdomen, inguinal, and intergluteal areas. Histopathology of the latter lesions showed increased spaces between collagen bundles in the dermis. Staining with Verhoeff-van Gieson revealed focal sparse elastic fibers in the papillary dermis compared to that of the reticular dermis consistent with anetoderma. Further work-up revealed normal ANA titer and low serum C3. Kidney biopsy showed IgG deposition in the tubular basement membrane and trace C3 deposition in the glomerular mesangium, giving a diagnosis of rapid progressive glomerulonephritis. On subsequent follow-up, the sac-like plaques became lax and presented as generalized wrinkling of the skin, raising the question whether cutis laxa and anetoderma are occurring in a spectrum instead as distinct entities. Based on the current review of literature, this is the first reported case of primary anetoderma co-occurring with cutis laxa in a patient with glomerulonephritis. Deposition of immunoglobulins along the elastic fibers could have activated the complement system, mediating the destruction of the elastic fibers, resulting to cutis laxa and anetoderma. This case also considers the possibility of anetoderma and type I acquired cutis laxa occurring either in a spectrum or as distinct diseases in a single patient. Further investigations may identify an ultrastructural pattern that can help differentiate the two entities.

Optimal Timing of Surgical Excision in Pediatric Pilomatricoma: Association between Clinicopathological Features and Cosmetic Outcomes

BACKGROUND: The treatment of choice for pilomatricomas is surgical excision; however, data for the optimal timing of treatment and cosmetic outcomes are limited.OBJECTIVE: This study aimed to investigate the optimal timing of treatment in pilomatricomas by considering clinicopathological findings and cosmetic outcomes.METHODS: Seventy-three pilomatricomas patients aged ≤15 years were retrospectively reviewed. Patients were classified into early excision (disease duration ≤12 months, group A) and delayed excision groups (disease duration >12 months, group B). Tumor characteristics, and histopathological features with evolutionary stages were assessed. Cosmetic outcomes were evaluated by the Modified Vancouver Scar Scale (MVSS), 5-point patient satisfaction score, and complication rates.RESULTS: Group A showed better cosmetic outcomes than group B in the MVSS (1.53±1.22 vs. 3.68±1.84), 5-point patient satisfaction score (4.08±0.89 vs. 3.18±1.01), and complication rates (11.8% vs. 36.4%), respectively (p<0.05). Secondary anetoderma, tent sign, calcification, and late regressive stage (evolutionary stage IV) were more common in group B, (p<0.05). Moreover, evolutionary stages showed a positive correlation with mean MVSS (r=0.670, p<0.05).CONCLUSION: Early excision (disease duration ≤12 months) provides superior cosmetic outcomes compared to delayed procedures. Early recognition, diagnosis, and management for pediatric pilomatricomas is important to improve overall cosmetic outcomes.

Anetodermia secundaria a sífilis / Anetoderma secondary to syphilis. Case report and literature review

La anetodermia es una entidad cutánea benigna, rara e infrecuente, cuya característica es la pérdida localizada de fibras elásticas a nivel de la dermis. Suele observarse en pacientes con síndrome antifosfolípidico, lupus eritematoso sistémico, acné y varicela y de manera inusual como manifestación de una sífilis secundaria. Comunicamos el caso de una mujer con secundario o sifilítico que desarrolló anetodermia posterior a este proceso infeccioso.

Anetoderma is a benign, rare and infrequent cutaneous entity whose characteristic is the localized loss of elastic fibers at the level of the dermis. It is usually observed in patients with antiphospholipid syndrome, systemic lupus erythematosus, acne and varicella and an unusual way as a manifestation of secondary syphilis. We report a case of a woman with syphilitic secondary disease who developed an anetoderma after this infectious process.

Anetodermia primaria asociada a lupus eritematoso sistémico. A propósito de un caso / Associated primary anemia to systemic lupus erythematosus About a case

La anetodermia es un trastorno elastolítico infrecuente, caracterizado clínicamente por áreas de piel laxa y pérdida o disminución de las fibras elásticas en la histología. Este hallazgo sin enfermedad cutánea previa es conocido como anetodermia primaria y se suele asociar a enfermedades autoinmunes; dentro de éstas el lupus eritematoso sistémico (LES), con anticuerpos antifosfolipídicos, y el síndrome antifosfolipídico (SAF) son las más frecuentes. Presentamos una paciente con anetodermia primaria, LES y anticuerpos antifosfolipídicos positivos sin clínica de SAF (AU)

Anetoderma is an infrequent elastolytic disorder, clinically characterized by areas with lack of the skin and decreased elastic fibers at histology. This finding without previous skin disease is known as primary anetoderma and is often associated with autoimmune diseases such as systemic lupus erythematosus (SLE), with antiphospholipid antibodies, and antiphospholipid syndrome (APS). A female patient with primary anetoderma, SLE and positive antiphospholipid antibodies without clinical APS is reported (AU)

Elastosis perforante serpiginosa y pseudo-pseudoxantoma elástico secundarias y tratamiento con D- penicilamina en paciente con enfermedad de Wilson / Elastosis perforans serpiginosa and pseudo-pseudoxanthomaelasticum secondary to D-penicillamine in a patient with Wilson’s disease

La D-penicilamina es la opción terapéutica más utilizada en la enfermedad de Wilson, rara enfermedad genética, autosómica recesiva, en la cual existe una alteración en el metabolismo del cobre que se deposita en los tejidos (hígado, encéfalo y córnea). Presenta numerosos efectos adversos, la mayoría cutáneos, que se observan cuando la droga es utilizada en altas dosis y por largo tiempo; entre ellos se encuentran las dermatosis degenerativas, que incluyen elastosis perforante serpiginosa, cutis laxa, anetodermia y pseudo-pseudoxantoma elástico (también llamado pseudoxantoma elástico símil o pseudoxantoma elástico like). Se presenta una paciente de 29 años con antecedentes de enfermedad de Wilson asociada a elastosis perforante serpiginosa y pseudo-pseudoxantoma elástico, ambas secundarias al tratamiento con D-penicilamina.

Penicillamineis the most commonly used therapeutic option in Wilson's disease.This is a rare, genetic, autosomal recessive diseasein which there is an alteration inthe metabolism of copper that is deposited in the tissues (liver, brain and cornea).It has numerous adverse effects, most of them affecting skin, but they are onlyobserved when the drug is used in high doses and for a long time, such as perforatingelastosis serpiginosa, cutis laxa, anetodermia and pseudo-pseudoxantomaelasticum (also called elasticum pseudoxantoma simil or elasticum pseudoxantomalike). We present the case of a29 year-old woman with a history of Wilson's diseaseand two concomitant degenerative dermatoses: elastosis perforans serpiginosa andpseudo pseudoxanthoma elasticum, both of them, secondary to treatment with Dpenicillamine.

Anetoderma primaria: revisión de la literatura a propósito de un caso / Primary anetoderma: a case report and literature review

La Anetoderma es una enfermedad elastolítica rara que se caracteriza por la pérdida focal de tejido elástico en la dermis. La patogénesis de esta enfermedad es desconocida y ha sido descrita en asociación con enfermedades autoinmunes, como lupus eritematoso y síndrome antifosfolípidos, y con enfermedades infecciosas, principalmente VIH, sífilis y borreliosis. Su diagnóstico diferencial es amplio e incluye otras enfermedades elastolíticas, liquen escleroatrófico extragenital y morfea, entre otros. Se reporta el caso de una paciente que consultó por un cuadro de larga data de múltiples parches blanquecinos atróficos, cuya biopsia cutánea fue compatible con Anetoderma. Se realiza una revisión de la literatura dada la necesidad de un adecuado estudio y seguimiento de estos pacientes en busca de una pesquisa temprana de enfermedades autoinmunes asociadas.

Anetoderma is a rare elastolytic disease characterized by focal loss of elastic tissue in the dermis. Pathogenesis is unknown and has been described in association with autoimmune diseases such as lupus erythematosus and antiphospholipid syndrome, and infectious diseases, especially HIV, syphilis and Lyme disease. Differential diagnosis is broad and includes other elastolytic disease, extragenital lichen sclerosus and morphea. We report the case of a patient with long-standing history of multiple atrophic white patches on the trunk and extremities, with diagnostic skin biopsy of Anetoderma. We conducted a literature review given the importance of good study and monitoring of these patients for an early investigation of associated autoimmune diseases.

Eruptive Anetoderma in a Patient with Systemic Lupus Erythematosus

Anetoderma is a rare cutaneous disorder characterized by a loss of normal elastic tissue that presents clinically as atrophic patches located mainly on the upper trunk. Recent studies suggest immunological mechanisms may play a role in this process. Furthermore, a secondary form of macular atrophy occurs in the course of infectious diseases (e.g. syphilis and tuberculosis) and autoimmune disease (e.g. systemic lupus erythematosus [SLE]). Here, we report the case of a 20-year-old woman previously diagnosed with SLE, who presented with numerous well-circumscribed atrophic macules on the face and upper trunk. Histopathological examination showed decreased elastic tissues in the reticular dermis and mononuclear cells adhering to elastic fibers, consistent with anetoderma. Thus, the eruptive anetoderma localized widely on the face and upper trunk may have been caused by an autoimmune response of SLE.

Anetoderma Developing in Generalized Granuloma Annulare in an Infant

No abstract available.

Secondary Anetoderma Developed in Juvenile Xanthogranuloma / 대한피부과학회지

Anetoderma is characterized by a loss of normal elastic tissue, and is clinically presented as atrophic patches located mainly on the upper trunk. It may be primary or secondary, occurring in the course of various dermatoses. The most common diseases among them are acne and varicella, but there are several reports about other skin diseases causing anetoderma. A 16-year-old girl visited our clinic for the evaluation of asymptomatic atrophic macules on the head, upper trunk and lower extremities. She had a 10-month history of yellowish brown papules located on the same area fifteen years ago. Histopathologic findings at that time showed histiocytic infiltration with foam cells and Touton giant cells in the dermis, which were consistent with juvenile xanthogranuloma (JXG). Skin biopsy was carried out at an atrophic macule; histologic findings revealed a decrease in dermal elastic fibers. All these findings were compatible with anetoderma. To our knowledge, this case is the first report of anetoderma developed in JXG in Korea.

A Case of Pityriasis Versicolor Atrophicans / 대한의진균학회지

We report a case of pityriasis versicolor atrophicans in a 20-year-old woman who presented with chronic multiple brownish atrophic macules and patches on chest and back. The lesions had developed six years ago and the patient had used topical medication including steroid ointments for the last two years prior to visiting our clinic. KOH examination was positive, so skin biopsy was performed and the diagnosis of pityriasis versicolor atrophicans was made. Since our patient had applied topical steroids for some time, it was difficult to rule out steroid-induced atrophy in a common tinea versicolor. Also, differential diagnosis was performed with respect to other skin diseases manifesting with cutaneous atrophy including anetoderma.

Penicillamine-induced Elastosis Perforans Serpiginosa and Cutis Laxa in a Patient with Wilson's Disease

Elastosis perforans serpiginosa (EPS) is a rare reactive perforating dermatosis that is characterized by the transepidermal elimination of abnormal elastic fibers. Penicillamine, which is one of the clear triggers for EPS, is a heavy metal chelator that is primarily used for disorders such as cystinuria and Wilson's disease. It may cause alterations in the dermal elastic tissue such as pseudo-pseudoxanthoma elasticum, acquired cutis laxa, EPS and anetoderma. Herein we present a case of cutis laxa and EPS in a 34-year-old man who was previously on a long-term, high-dose of penicillamine for Wilson's disease. The combination of EPS and cutis laxa induced by penicillamine has rarely been reported and we report the first such case in Korea.

A Case of Dermatofibrosarcoma Protuberans Presenting as an Anetoderma-like Lesion with Nodular Lesions / 대한피부과학회지

Atrophic dermatofibrosarcoma protuberans (DFSP) is an uncommon clinical variant, which clinically mimics other atrophic dermatological conditions. Given the typical 'protuberant' morphology of DFSP, such atrophic lesions may be difficult to diagnose clinically. We report a 44-year-old man who presented with an 8-month history of an asymptomatic, depressed, anetoderma-like plaque on the right subclavicular area. He also presented with a 1-year history of a 1.5x1.5 cm, asymptomatic, firm, erythematous nodule above an atrophic lesion. Biopsy specimens were taken from each lesion. These specimens, along with histochemical staining for CD34, established the diagnosis of dermatofibrosarcoma protuberans.

A Case of Multiple Pilomatricoma Combined with and without Secondary Anetoderma / 대한피부과학회지

Pilomatricoma, also known as the calcifying epithelioma of Malherbe, is a benign adnexal neoplasm that arises from primitive cells of the hair matrix. It usually appears as a firm, slowly growing dermal or subcutaneous nodule, almost solitary but occasionally multiple. Anetoderma is characterized clinically by discrete, atrophic skin lesions and pathologically by the focal loss of elastic fibers in the dermis. Anetodermic cutaneous changes in the skin overlying a pilomatricoma have been rarely described in the literature. Herein, we report 17-year-old girl who presented with two skin lesions, one of which was a painful nodule with bag-like, wrinkled surface on the left posterior shoulder and the other was asymptomatic deep seated nodule with skin colored surface on the left upper back. The histopathologic examination showed typical findings of pilomatricoma, and anetoderma was associated with tender nodule on the shoulder.

Four Cases of Secondary Anetoderma due to Pilomatricoma / 대한피부과학회지

Anetoderma is characterized by atrophic patches located mainly on the upper trunk. Secondary anetoderma occurs in the courses of various diseases, such as syphilis, lupus erythematosus, and leprosy. Among them secondary anetoderma due to pilomatricoma is rare and has a characteristic skin manifestation such as scrotal skin or round striae. Herein we report four cases of pilomatricomas with secondary anetodermic cutaneous changes. The overlying skin appeared variously as a pinkish, wrinkled, bag-like bulged, or scar-like atrophic surface. However the histopathologic examination showed commonly a prominent decrease of elastic fibers in the dermis overlying the pilomatricoma mass.

A Case of Primary Anetoderma / 대한피부과학회지

Anetoderma is a rare cutaneous disorder characterized by circumscribed loss of skin elasticity. This has been historically divided into inflammatory(Jadassohn-Pellizzari) and non-inflammatory(Schw eninger-Buzzi) type. We herein, report a case of Schweninger-Buzzi type anetoderma occurring on a 6 year-old girl. The lesions did not have an inflammatory onset. Histologically, the focal loss of elastic fibers was seen on the lesion.

Anetoderma Secondary Due to Actinic Granuloma / 대한피부과학회지

We report a case of actinic granuloma in a 39 year-old woman who was presented with multiple anetoderma on non-sun exposed skin such as the neck, upper arms, elbows and calfs. The anetodermic presentation of actinic granuloma has never been reported and the common pathogenesis involving reduction of elastic fibers link the two disorders. Anetoderma secondary due to actinic granuloma should to be included in the list of secondary anetoderma and may suggest one of the possible pathogenesis of anetoderma.

Actinic Granuloma:One of the Causes of Secondary Anetoderma

Actinic granuloma (O'Brien) is an annular inflammatory reaction that develops in skin after long-term sun-exposure. Anetoderma is characterized by discrete skin lesions that appear to be loose and wrinkled. The primary type arises in clinically normal skin, and the secondary type appears in lesions of other disorders. We describe a case of anetoderma that developed on the neck, both arms and calves, and showed histopathological findings of actinic granuloma.

A Case of Anetoderma Overlying Pilomatricoma / 대한피부과학회지

Anetoderma is loose and wrinkled skin lesions that show the characteristic histopathological feature of focal loss of elastic fibers in the dermis. The primary type of anetoderma arises in clinically normal skin and the secondary type replaces the lesions of associated disorders including various infections, infiammatory diseases and tumors. However, anetoderma due to pilomatricoma is very rare. Our patient, a 21-year-old female, presented with a soft and wrinkled skin overlying a firm, pedunculated tumor on her left upper arm. The histopathological examination showed anetodermic cutaneous changes which were associated with the underlying pilomatricoma.

Secondary Anetoderma due to Pilomatricoma

We report a case of secondary anetoderma due to pilomatricoma. A 15-year-old girl presented with a soft, 1.5 × 1.5cm in size, atrophic, pinkish and bulged-out lesion overlying the palpable subcutaneous mass on the left upper arm for several months. Histologic examination revealed the reduced number of dermal elastic fibers overlying a typical pilomatricoma.