Anetoderma after disseminated Mpox.

Anetoderma or macular atrophy is a rare skin condition of unclear pathogenesis, often associated with autoimmune diseases and skin damage from various infections. Human immunodeficiency virus (HIV), syphilis, and poxviruses have been implicated in the development of anetoderma. A 37-year-old male patient with HIV and recent unprotected sexual encounters presented with more than 400 skin lesions, consistent with Mpox. Symptomatic treatment for Mpox resulted in acute symptom resolution. However, 8 months later he developed papular anetoderma lesions in areas previously affected by Mpox. Biopsy confirmed the loss of elastic fibers in the affected skin areas, leading to the diagnosis of Mpox-induced anetoderma. This report presents a unique case of anetoderma following Mpox in an HIV-positive patient.

Use of dermal fat grafts for treating anetoderma with lipoatrophy following involution of hemangiomas.

Infantile hemangioma (IH) is a benign vascular tumor that gradually involutes over several years. Rapidly involuting congenital hemangioma (RICH) is the relatively rare congenital vascular tumor that is fully grown at birth and does not undergo postnatal growth and involutes during the first year. However, after involution of both IH and RICH, some have severe sequelae, such as redundant skin or conspicuous scarring, requiring additional treatment. We present the case of a 6-year-old girl with a concave deformity due to subcutaneous atrophy, skin darkening, and altered skin texture of her left zygomatic region following involution of a hemangioma. We successfully treated this patient by transferring a dermal fat graft. This technique can be beneficial for atrophic sequelae after regression of a hemangioma and is easy to perform and cosmetically effective.

Bullous sweet syndrome healing with prominent secondary anetoderma.

A 15-month-old boy presented with 1-4 cm, pink edematous plaques with overlying round erosions and hemorrhagic bullae in the setting of a gastrointestinal illness and was ultimately diagnosed with bullous-type Sweet syndrome. Despite appropriate treatment with oral steroids, the patient's cutaneous lesions healed with secondary anetoderma. This case should prompt practitioners to be aware of bullous-type Sweet syndrome and the possibility of lesions healing with postinflammatory scarring.

[Multifocal chalazodermic amyloidosis: The concept of immunoglobulinemic elastopathy]. / Chalazodermie amyloïde multifocale. Le concept d'élastopathie immunoglobulinémique.

INTRODUCTION: Impairment of dermal elastic tissue occurs in different entities associated with immunoglobulins or immunoglobulin-derived protein-secreting clonal plasma cell proliferations, such as amyloid elastosis, anetodermic nodular amyloidosis or monoclonal gammopathy-associated cutis laxa. We report a case of cutaneous immunoglobulinemic amyloidosis revealed by a unique chalazodermic presentation and we review elastic tissue impairment in patients with monoclonal gammopathies. OBSERVATION: A 67-year-old woman consulted for non-infiltrated anetodermic lesions on the upper left quadrant of her abdomen present for ten years. She also had a chalazodermic plaque with abnormal skin wrinkling and laxity in her right axilla. Biopsies revealed deep dermal and subcutaneous amyloid deposits. Immunohistochemistry with lambda light chain was positive. Orcein staining and electron microscopy showed extensive elastolysis. The patient presented no signs of systemic involvement, but a very small amount of monoclonal IgGλ gammopathy was detected during follow-up. DISCUSSION: This is a unique chalazodermic presentation of immunoglobulinemic amyloidosis that does not fit into a clearly-defined nosological setting. It highlights the complex interactions between immunoglobulin-derived proteins, including light and heavy chains, and elastic tissue components, leading to different types of impairment of the latter. We therefore suggest the unifying concept of immunoglobulinemic elastopathy, underscoring the need to screen for monoclonal gammopathy in patients presenting elastic tissue impairments.

Ultrastructural aspects of primary anetoderma.

Anetoderma is a rare cutaneous disorder characterized by focal loss of dermal elastic tissue due to unknown mechanisms. Primary anetoderma develops on clinical normal skin, without any preceding dermatosis and it can be associated with autoimmune conditions. Secondary anetoderma develops on the same area of a previous disorder, such as infectious, neoplastic or inflammatory diseases. A 37-year-old female patient noticed for 4 years circumscribed, normochromic, asymptomatic herniated plaques on the trunk and upper limbs. Family history was negative. Only a positive antinuclear factor (ANF) test, with titer of 1:160 and nuclear homogeneous pattern was found. Light microscopy with Weigert staining showed a lessening of elastic fibers with fragmentation; the oxytalanic fibers were also affected or absent. Transmission electron microscopy showed fragmentation and granular degeneration of elastic fibers. With greater magnification, fragments similar to those seen with optical microscopy were identified. The collagen fibers did not present any alteration. The examination of the dermis with scanning electron microscopy also identified fragmentation and significant fissures of the elastic tissue, granular degeneration was also observed. With greater magnification fragmented elastic fibers were seen.

Anetoderma treated with combined 595-nm pulsed-dye laser and 1550-nm non-ablative fractionated laser.

Anetoderma is a skin disorder characterized by a focal loss of dermal elastic tissue whereby patients present with soft, depressible lesions. We postulated that a series of combination treatment using the 595-nm pulsed dye laser (PDL) and the 1550-nm non-ablative fractionated laser (NAFL) would improve the anetoderma lesions. Our patient with biopsy proven anetoderma received 3 treatments with a combination of 595-nm PDL and 1550-nm NAFL spaced 3 weeks apart. Skin biopsies were performed at baseline and immediately prior to the third treatment. Stains for hematoxylin and eosin and Verhoeff Van Gieson (VVG) were performed. Improvement in lesion color, texture, and overall appearance was noted after the second treatment and continued following the third treatment. Post-treatment VVG staining demonstrated an increase in dermal elastin fibers and a decrease in elastin fiber fragmentation. Thus, the combination of 595-nm PDL and 1550-nm NAFL should be considered as a treatment modality for anetoderma.

Anetoderma in a patient with terminal osseous dysplasia with pigmentary defects.

Terminal osseous dysplasia with pigmentary defects (TODPD) is a rare, X-linked syndrome classically characterized by distal limb anomalies, pigmented skin defects of the face, and recurrent digital fibromas. X-inactivation plays a major role in determining the range of phenotypic expression. Thus, patients can demonstrate a wide spectrum of disease severity, making accurate diagnosis more challenging. Recent studies have identified a FLNA c.5217G>A mutation as the cause of TODPD, allowing for diagnostic genetic testing. We present a case of molecularly confirmed TODPD in a girl with the 47,XXX chromosomal complement and deformities of the hands and feet, craniofacial abnormalities, and discolored, linear facial lesions. Skin biopsy of the patient's facial lesion revealed absent papillary dermal elastic fibers, consistent with anetoderma, which contrasts with the dermal hypoplasia described in the only other such facial biopsy reported in the literature. The finding of absent elastic fibers in the skin lesions suggests that mutated filamin A, in part, exerts its effects through dysregulated elastin biology, which may explain the nature of many connective tissue pleotropic effects in FLNA-related disorders.

Anetoderma arising in Reed syndrome: a case report and review of the literature.

Anetoderma is a cutaneous disorder characterized by loss of dermal elastic tissue resulting in papules from herniation of subcutaneous tissue or circumscribed areas of atrophic, wrinkled skin. Familial leiomyomatosis cutis et uteri (Reed syndrome) is an autosomal dominant disorder characterized by cutaneous and uterine leiomyomas. We report a 23-year-old male with Reed syndrome who presented with asymptomatic pearly white, atrophic, flaccid papules on the upper back and shoulder that depressed when palpated. Pathologic examination showed an unremarkable epidermis and central loss of dermal elastin, bordered by clumped elastin, as revealed with an elastin stain. The correlation of clinical and pathologic findings indicated a diagnosis of anetoderma arising in a patient with Reed syndrome.

Acral Folliculocentric Extragenital Lichen Sclerosus: A Case Report.

Introduction: Lichen sclerosus is a chronic inflammatory dermatological condition of unknown etiology, primarily impacting the genital epidermis in individuals of all genders, with a higher prevalence observed among postmenopausal women and prepubescent girls. Additionally, extragenital manifestations occur in approximately 20% of the patients diagnosed with genital lichen sclerosus. Notably, folliculocentric extragenital lichen sclerosus is rare and unusual, with only limited instances documented in existing literature. Case Description: We report a 33 years old lady presented with multiple asymptomatic lesions on the dorsal feet for 1 year and similar lesions on the left hand for 4 months. On examination: folliculocentric, shiny, atrophic papules coalescing into reticulated plaques over the dorsum of both feet and few shiny, flat-topped, pink papules over the dorsum of the left hand. A skin biopsy was performed and confirmed the diagnosis of extragenital lichen sclerosus. Conclusion: Acral folliculocentric extragenital lichen sclerosus is an unusual and rare clinical variant. Clinicopathologic correlation is necessary to establish the correct diagnosis. Contribution to the Literature: Herein, we present an unusual presentation of extragenital lichen sclerosus, and we highlight the importance of considering it in the differential diagnosis of guttate acral skin lesions. We also review and summarize relevant cases from the literature in hope to aid physicians, especially dermatologists, to consider and swiftly reach the diagnosis and offer appropriate management. We also hope to bring about new insights and broaden future research efforts regarding lichen sclerosus especially and atrophic skin disease in general.

The skin and hypercoagulable states.

Hypercoagulable states (HS) are inherited or acquired conditions that predispose an individual to venous and/or arterial thrombosis. The dermatologist can play a vital role in diagnosing a patient's HS by recognizing the associated cutaneous manifestations, such as purpura, purpura fulminans, livedo reticularis, livedo vasculopathy (atrophie blanche), anetoderma, chronic venous ulcers, and superficial venous thrombosis. The cutaneous manifestations of HS are generally nonspecific, but identification of an abnormal finding can warrant a further workup for an underlying thrombophilic disorder. This review will focus on the basic science of hemostasis, the evaluation of HS, the skin manifestations associated with hypercoagulability, and the use of antiplatelet and anticoagulant therapy in dermatology.

Follmann balanitis and anetoderma in secondary syphilis.

Syphilitic balanitis of Follmann (FB) is a rarely described manifestation of primary syphilis that was first reported in 1948. Its clinical appearance may be heterogeneous varying from painful edematous balanoposthitis to superficial erosive balanitis and asymptomatic glans induration. We described a patient presenting with FB, as manifestation of primary syphilis, and concurrent anetoderma, as manifestation of secondary syphilis. The association of these lesions was never described to date.

Bullous Pilomatrixoma After COVID-19 Vaccination.

Pilomatrixoma, or calcifying epithelioma of Malherbe, is a benign tumor with differentiation toward the hair matrix cells and is one of childhood's most common epithelial tumors. Bullous pilomatrixoma has an extremely low incidence of occurrence, usually appears in the upper extremities, and is frequently associated with trauma. We report the case of a bullous pilomatrixoma in a patient with a rapid-growing neoformation one month after receiving a coronavirus disease 2019 (COVID-19) vaccine in his left upper arm, and we discuss whether the bullous appearance is part of the biology of the tumor or a secondary anetoderma.

Generalized anetoderma: An unusual manifestation of secondary syphilis treated with injection penicillin.

Anetoderma also called macular atrophy is a rare, benign disorder characterized microscopically by the pan-dermal loss of elastic fibers in the dermis and presenting clinically as circumscribed, skin-colored or gray-white atrophic macules and/or patches on the trunk and/or extremities. Lesions are described as having a "sac-like" appearance, since they bulge or herniate upon palpation. It is a rare benign condition of diverse etiology; whose characteristic is the diminution or absence of the dermal elastic fibers. Anetoderma is divided into primary (idiopathic) and secondary anetoderma, with the former occurring in areas of previously normal skin and the latter developing in areas of prior skin pathology. Both may occur in association with underlying systemic conditions and warrant evaluation for associated disorders. There are no effective treatment options for anetoderma at present. We report here an unusual case of generalized anetoderma occurring in association with secondary syphilis treated with injection benzathine penicillin.

Anetoderma in a patient with a history of primary syphilis.

Anetoderma is a rare cutaneous disorder presenting with atrophic skin lesions. It can be associated with several autoimmune and infectious diseases. With the current resurgence of syphilis, clinicians must be aware of its association with anetoderma.

Six cases of perforating pilomatricoma: Anetodermic changes with expression of matrix metalloproteinases.

Perforating pilomatricoma (PP) is a rare clinical variant of pilomatricoma presenting as a crusted or ulcerated nodule. Previous reports have suggested that the tumor cells perforate the epidermis through a process of transepithelial elimination. Here, we report six cases of PP and examine the mechanism of transepithelial elimination in PP. Histologically, the dermis above or around the tumor nest exhibited edema, dilated vascular spaces, sparse collagen bundles and absence of elastic fibers, suggesting anetodermic changes in all cases. Immunohistochemistry demonstrated many CD68-positive macrophages around the tumor nests. Matrix metallopeptidase (MMP)-9 and MMP-12 were expressed in the inflammatory cells and tumor cells, and were also present in the epidermis and fibroblasts in all cases. We speculate that in PP anetodermic change caused by MMP and elastases including MMP-9 and MMP-12 may precede elimination of the tumor.

Anetoderma Schweninger-Buzzi: Two Case Reports.

BACKGROUND: Anetodermas are rare disorders of connective tissue with a focal loss of elastic fibres in the upper and mid dermis. Two types are separated, inflammatory and non-inflammatory. CASE REPORTS: We report two cases of acquired anetoderma Schweniger-Buzzi type. This non-inflammatory subtype is characterised by skin-coloured or whitish atrophic sac-like protrusions of trunk skin in adult males. Chronic infections and autoimmune disorders have been excluded. The diagnosis had been confirmed by characteristic histopathology. CONCLUSIONS: Anetodermas are symptomless disorders. They can be easily overlooked. The knowledge of such conditions is of importance to identify patients with a risk of thromboembolic events and underlying infections or autoimmune connective tissue diseases.