Lymphangioma Circumscriptum Research Articles

Acquired Lymphangioma Circumscriptum of Vulva in a Patient With Hidradenitis Suppurativa: A Rare Case at a Rare Site.

Lymphangioma circumscriptum, one of the common forms of lymphangioma, can be either congenital or acquired. Various acquired conditions can lead to its causation and one of the causes might be untreated hidradenitis suppurativa. So, this report highlights the need of early interventions for hidradenitis suppurativa as lymphangioma circumscriptum can be one of its consequences later in life. It can occur in various sites, often around neck, axilla, and groin. Post-surgery and radiotherapy, tuberculosis, Crohn's disease are well known cause to develop acquired vulval lymphangioma. Here, we present a case of a 20 years female with acquired vulval lymphangioma secondary to hidradenitis suppurativa which is one of the rare cause for vulval lymphangioma.

Clinical features and molecular genetics of patients with RASopathies: expanding the phenotype with rare genes and novel variants.

In this study, we described rare clinical manifestations and detected three novel variants in NF1, BRAF, and RIT1 genes. We propose that NGS technology enables the detection of variants in rare genes responsible for the etiology of RASopathies. The study, therefore, not only contributes to the existing literature but also expands the spectrum of genotype and phenotype of RASopathies. •RASopathies are a group of disorders caused by germline variants in genes involved in the Ras/mitogen-activated protein kinase (RAS/MAPK) pathway. •These disorders, including Noonan syndrome (NS), Cardiofaciocutaneous syndrome (CFC), Costello syndrome (CS), Legius syndrome, and Neurofibromatosis type 1 (NF1), share overlapping clinical features due to RAS/MAPK dysfunction. Molecular diagnosis of RASopathies is crucial for understanding the genetic basis and guiding clinical management, although the phenotype-genotype relationships remain incompletely defined. •This study provides new insights into the molecular and clinical characteristics of RASopathies by examining 149 patients from 146 families, with a focus on the genetic variants found in 24 RASopathy-related genes. Three novel variants were identified in the RIT1, BRAF, and NF1 genes, expanding the genetic spectrum of RASopathies. •Additionally, rare clinical findings, such as lymphangioma circumscriptum and corpus callosum thickness, were reported in patients with PTPN11 and SOS1 gene variations, respectively. These observations contribute new phenotypic data to the existing body of knowledge.

Exploring the Potential of Intralesional Radiofrequency Ablation in Dermatology: A Case Series

Intralesional radiofrequency (ILRF) ablation is a novel and safe dermatosurgical technique which, unlike conventional radiofrequency, minimizes the risk of epidermal scarring by precisely targeting the reticular dermis. ILRF’s simplicity, cost-effectiveness, and cosmetic advantages make it an attractive option for treating various skin conditions. While ILRF has found applications in various fields beyond dermatology, there exists a scarcity of data concerning its safety and effectiveness in dermatology. We successfully utilized this novel technique to manage conditions such as lymphangioma circumscriptum and molluscum contagiosum, highlighting the promising impact and benefits of this approach.

Zosteriform Cutaneous Metastasis from Breast Cancer: A Case Report

Introduction: Cutaneous metastases are observed in approximately 10% of oncology patients, often indicating either a persistent solid tumor or the recurrence of neoplastic disease. The most prevalent cause of cutaneous metastases in females is breast cancer. Patients with breast cancer may exhibit cutaneous manifestations at the initial diagnosis; however, these metastases more frequently emerge well after the initial diagnosis and treatment. The cutaneous lesions resulting from metastatic breast carcinoma display considerable variability in appearance. Typically, they manifest as firm, flesh-colored to red, smooth or ulcerated, or crusted nodules, papules, and plaques on the ipsilateral chest wall and breast. Additionally, unusual sites for breast cancer cutaneous metastases include the eyelids, inframammary folds, ipsilateral lymphedematous arm, scalp, subungual nail bed, and umbilicus. Skin metastases can also occur in mastectomy scars and radiation therapy ports. Several distinct patterns of skin metastases are recognized in breast cancer patients: carcinoma erysipelatoides, carcinoma telangiectoides, and carcinoma encuirasse. Recently, carcinoma hemorrhagiectoides has also been documented. The pleomorphic nature of breast cancer metastases can mimic various benign and malignant conditions, such as collision tumors, cysts, dermatofibromas, milia-en-plaque, melanoma, non-melanoma skin cancers, cellulitis, folliculitis, herpes zoster, paronychia, erythema annulare centrifugum, urticaria, alopecia areata, dermatitis, hidradenitis suppurativa, scleroderma, angiokeratoma, angiosarcoma, lymphangioma circumscriptum, purpura, and pyogenic granuloma. Consequently, it is crucial to consider the possibility of cutaneous metastasis from breast cancer in any patient, whether previously diagnosed with breast cancer or not, who presents with new or treatment-resistant cutaneous lesions. Confirmation of the diagnosis necessitates a biopsy of the skin lesion. ...........

A case of lymphangioma circumscriptum successfully treated with topical sirolimus

A case of lymphangioma circumscriptum successfully treated with topical sirolimus

Treatment of Oral Lymphangiomas by Coblation.

Lymphangioma is a malformation of superficial lymphatic vessels. Tongue lymphangiomas are relatively uncommon. Multiple treatment modalities have been reported, with variable treatment responses. Most of the traditional treatment modalities have a high recurrence rate. We describe the use of coblation in the management of lymphangioma circumscriptum of the dorsum of tongue in two patients. Radiofrequency ablation of oral lymphangiomas showed early postoperative oral intake and minimal postoperative pain. There was no recurrence of disease on 1 year follow up. Improved wound healing, early postoperative oral intake and minimal postoperative pain, make radiofrequency ablation a highly valuable treatment modality for oral lymphangiomas and may be recommended as the treatment of choice.

Weeping blisters – Clinical presentation in an uncommon site

Lymphangioma Circumscriptum (LC), a microcystic hamartomatous lymphatic malformation is a rare benign condition characterized by the proliferation of dilated lymphatic vessels in the skin. Usually, it is congenital, but some may become clinically apparent later in life. They account for 4% of all vascular malformations. Usually seen in and around the shoulder and pelvic girdle. This case report discusses LC in a teenage girl with a 13-year-old history of painless, grouped fluid filled blisters on the right side of her chest, an uncommon site presenting after several years seeking intervention. The diagnosis was established through clinical examination and skin biopsy. With normal basic laboratory investigations, we initiated her with lesional cryotherapy as the primary efficacious intervention before planning for surgical excision. Cryotherapy is cheap, safe and easily available option.

A case of intralymphatic histiocytosis mimicking lymphangioma circumscriptum associated with a puncture wound caused by a thorn

A case of intralymphatic histiocytosis mimicking lymphangioma circumscriptum associated with a puncture wound caused by a thorn

Pubic and vulvar papules and vesicles.

We report the case of a 54-year-old woman, who had a hysterectomy and adjuvant radiation therapy for a history of cervical cancer, presenting with a 6-year history of diffuse vaginal papules and vesicles that ooze serous fluid.

Frog-spawn appearance: Diagnostic clue to lymphangioma circumscriptum

Frog-spawn appearance: Diagnostic clue to lymphangioma circumscriptum

Unexpected Axillary Lymph Node Uptake During Lower-Limb Lymphoscintigraphy in a Case With Vulvar Lymphangioma Circumscriptum.

Lymphoscintigraphy is a safe, minimally invasive, and well-established imaging modality for evaluating lymphatic drainage in patients with suspected lymphedema. We report a case of an 8-year-old girl with lymphangioma circumscriptum of the labium majora. She was referred to lymphoscintigraphy for swelling of the lower extremities. Scintigraphy showed widespread dermal backflow at bilateral lower limbs, aberrant uptake in the abdominal lymph nodes, and unexpected uptake in the right axillary lymph nodes.

Recurrent lymphangioma circumscriptum: Treated with microneedling radiofrequency and topical sirolimus

Abstract Introduction: Lymphangioma circumscriptum is a “microcytic” lymphatic malformation. Different treatments have been tried with varying success. We report here a case of recurrent lymphangioma circumscriptum who was treated with microneedling radiofrequency (MNRF) and topical preparation or sirolimus cream with good improvement. Case Report: A 14-year-old girl presented to outpatient department with a cluster of small asymptomatic blisters over the unilateral inguinal area which on histopathology was confirmed to be lymphangioma circumscriptum. The patient had similar lesions 3 years back which she got surgically excised. Since the last 6 months, she had a recurrence of the similar lesions so this time we chose MNRF every 40 days to treat this case which was combined with topical sirolimus 0.01% during and after the procedure. Discussion: MNRF helps in neoelastogenesis and neocollagenosis, which leads to dermal thickening. It also acts as a drug delivery system by creating dermal channels. Sirolimus with its antilymphangiogenic properties helps prevent recurrence.

Lymphangioma circumscriptum of thigh in a 12-year-old girl: report of a rare case

Abstract Clinical diagnosis of grouped vesicular lesions in pediatric age group needs a detailed history and meticulous dermatological assessment of the morphology. Lymphangioma circumscriptum (LC) is one rare condition suspected with classic clinical presentation. Frog spawn appearance and black vesicles are two clues for making a clinical diagnosis. Here, we report a case of LC over thigh in an adolescent girl in view of its rare age of presentation and unusual location.

High-grade Undifferentiated Pleomorphic Sarcoma Presenting as Acquired Lymphangioma Circumscriptum

A previously healthy woman in her 40s presented to the dermatology clinic with a 1-year history of a tender, draining, enlarging mass on the left buttock. Physical examination revealed an extensively indurated hyperpigmented nodule with numerous overlying clustered exophytic verrucous papules on the L medial buttock (see figure 1). Initial skin biopsy of verrucous lesions was notable for lymphangioma with overlying condyloma, D2-40 positive. MRI abdomen and pelvis with contrast demonstrated a left gluteal enhancing subcutaneous soft tissue mass, 10.3 x 8.2 x 8.8 cm in size, overlying and involving the left gluteus maximus, along with satellite nodules present in the medial gluteus maximus and paraspinal muscles, with bilateral pelvic and inguinal adenopathy. Excisional deep tissue biopsy revealed a diagnosis of a high-grade, multifocal spindle and undifferentiated pleomorphic sarcoma. The patient subsequently underwent 4 cycles of neoadjuvant chemotherapy AIM (doxorubicin, iphosphamide, MESNA), followed by radical resection of the left gluteal region, with positive margins, followed by repeat surgical resection and reconstruction with left-sided latissimus dorsi myocutaneous free flap. The patient has now completed radiation therapy.
 Soft tissue sarcoma is a heterogeneous group of rare malignancies of mesenchymal origin, that represents 0.8% of all adult cancers. Prognostic factors associated with poor outcome include high histologic grade, large tumor size, presence of metastases, and unresectability1. Herein, we report a novel case of high-grade soft-tissue undifferentiated pleomorphic sarcoma, masquerading as acquired lymphangioma circumscriptum (ALC), as noted on initial skin biopsy of grouped flesh-colored verrucous papules overlying an indurated tender mass. ALC is a lymphatic malformation occurring as sequela to any process which interrupts previously normal lymphatic drainage. As such, subcutaneous lymphatic cisterns are unable to drain completely into the general lymphatic system, resulting in lymphatic dilation and subsequent development of vesicular and papular lesions on the trunk, extremities, and anogenital areas.2 ALC can mimic common infectious growths including molluscum contagiosum and condyloma accuminata, and accordingly be mismanaged. Etiologic causes of ALC including malignancy, and has previously been reported in the setting of cervical carcinoma, vulvar carcinoma, endometrial carcinoma, Hodgkin lymphoma, rhabdomyosarcoma, rectal carcinoma, melanoma, and high-grade penile dysplasia2,3,4. Other etiologic causes can include surgery, trauma, radiation therapy, chronic inflammatory conditions including hidradenitis suppurativa, and inflammatory bowel disease.
 To date, acquired lymphangioma circumscriptum has not been reported in the setting of underlying high-grade pleomorphic soft tissue sarcoma. As such, clinical presence of grouped frogspawn-like or verrucous papules, in an area of tender induration or lymphedema, should prompt a work-up to exclude an underlying malignant etiology as new onset ALC may be masquerading an underlying malignancy.

Cutaneous lymphangioma circumscriptum presenting as warty plaque

Cutaneous lymphangioma circumscriptum presenting as warty plaque

Acquired lymphangioma circumscriptum of the vulva in a middle aged woman with chronic hidradenitis suppurativa – A case report

Lymphangioma circumscriptum (LC) is a distention of lymphatic channels present in skin and subcutaneous tissues. LC is either primary (usually present at birth or develops in early childhood) or secondary due to diminished lymphatic outflow. It is commonly prone to develop in oral cavity, tongue, proximal regions of arms and legs, groin, axilla, and trunk. Primary vulvar involvement is unusual. Vulvar LC is a rare untoward outcome of hidradenitis suppurativa (HS), which causes significant physical and emotional discomfort. We report a case of acquired LC of vulva as a sequlae of HS in a 42 years old female who presented with painful papules, edema and thickening of the vulva with history of foul-smelling discharging sinuses. The patient was managed surgically by wide excision and reconstructive surgery by Plastic surgery unit.

Acquired lymphangioma circumscriptum of the vulva (secondary to lymphatic obstruction) occurring ten years after a hysterectomy and radiotherapy: A case report

Acquired lymphangioma circumscriptum of the vulva (secondary to lymphatic obstruction) occurring ten years after a hysterectomy and radiotherapy: A case report

Lymphangioma CircumscriptaA Diagnostic Dilemma

Lymphangiomas are developmental malformations that present as benign hamartomas of lymphatic channels. They present at birth or before two years of age, with 50-70% predilection for the head and neck region and less frequently reported in the oral cavity. However, affected sites in the oral cavity include the tongue, palate, gingiva, buccal mucosa, lips and alveolar ridge. Lymphangioma of the buccal mucosa is rare, only 27 cases of lymphangioma of buccal mucosa have been reported till date, out of which seven were in children. The prognosis is good for most patients, but recurrence has also been reported in some cases. This present case is of Lymphangioma Circumscriptum in a 13-year-old child involving right buccal mucosa and lip since birth. This lesion was surgically removed under general anaesthesia and there was a recurrence of the lesion after one year of follow-up. In the literature there was no reported case of Lymphangioma Circumscriptum involving the lip of a child, so this present case was the firstreported case of Lymphangioma Circumscripta involving the lip in a child with recurrence.

Acquired Cutaneous Lymphangiectasia of Vulva: A Novel Dermoscope Finding.

A 61-year-old woman presented with a 1-year history of multiple, oozy, raised pruritic lesions over the genitalia. Four years back, she had undergone radical hysterectomy followed by loco-regional radiotherapy for her stage IIA carcinoma cervix. On physical examination, she had multiple, grouped, translucent, thin-walled vesicles resembling “frogspawn” on the bilateral labia majora [Figure 1]. Histopathological examination showed a hyperkeratotic epidermis with multiple dilated lymphatic channels in the superficial and deep dermis. Dermoscopy (Dermlite 4) revealed clustered, translucent, reddish-white lacunae surrounded by whitish septa. Few lacunae showed radially arranged, hairpin-like vessels [Figure 2]. The features were consistent with acquired cutaneous lymphangiectasia of vulva (ACLV). Radiofrequency ablation of the lesions was performed and she was under regular follow-up.Figure 1: Multiple, grouped, translucent to flesh-colored, fluid-filled vesicles - ”frogspawn appearance” on labia majoraFigure 2: Dermoscopy (Dermlite 4: Polarized view, 10× magnification) showing clustered, translucent, reddish-white lacunae surrounded by whitish septa (yellow circle). Few lacunae showed radially arranged hairpin-like vessels (black circles)ACLV is a rare entity, which occurs secondary to conditions like trauma, surgery, keloids, radiation, scleroderma, pregnancy, malignancies, Crohn’s disease, or infections like erysipelas, tuberculosis, filariasis, and lymphogranuloma venereum. These conditions cause destruction of lymph nodes and impair lymphatic flow thereby resulting in the accumulation of lymph in dermal lymphatics producing vesicles.[1] The differential diagnosis includes lymphangioma circumscriptum, syringoma, condyloma, herpes, warts, molluscum contagiosum, and benign hemangioendothelioma.[12] Errichetti et al.[3] in their dermoscopy of ACLV reported a vascular pattern with well-demarcated, round to oval red lacunae surrounded by white areas/lines with several punctiform and a few irregular vessels. In addition, we have observed radially arranged hairpin-like vessels in our case. In dermoscopy, similar hairpin-like vessels are found in seborrheic keratosis, keratoacanthoma, squamous cell carcinoma, amelanotic melanoma, superficial basal cell carcinoma (BCC), nodular BCC, eccrine poroma, porocarcinoma, and pilomatrixoma.[4] We report this case for the novel dermoscopic finding. Financial support and sponsorship Nil. Conflicts of interest There are no conflicts of interest.

A Patient with Lymphangioma Circumscriptum on Breast Skin

A Patient with Lymphangioma Circumscriptum on Breast Skin