Acquired Microcystic Lymphatic Malformation of the Distal Upper Extremity Mimicking Verrucae Vulgaris

Abstract

An 18-year-old African American male with a history of congenital lymphedema of the right upper extremity presented for evaluation of multiple verrucous lesions on his right hand. Clusters of 2 to 4-mm dome-shaped vesicles were intermixed with scattered verrucous papules on the right forearm and the dorsal and palmar aspects of the hand. Histopathology of one the verrucous lesions showed well-circumscribed areas of dilated lymphatic vascular channels with lymph in the lumen. The patient was diagnosed with microcystic lymphatic malformation, verrucous type. This article reviews the literature regarding reports of this variant of microcystic lymphatic malformation in the pediatric population.

Microcystic lymphatic malformation presenting as firm, skin‐colored papules of the lips

“…Clinically, the entity may appear localized, but there is often a more extensive deeper malformation. MiLM may present either congenitally, within the first 2 years of life, or secondary to alterations in the lymphatic system 1 . The affected lymphatic endothelial cells may have somatic activating mutations in the PIK3CA gene.…”

S1 Guidelines – Dermatoses associated with dermal lymphostasis

“…These symptoms are usually much less pronounced when affected individuals do water exercises. Dermal lymphocysts and lymphocutaneous fistulas may be diagnosed by clinical examination and/or histopathology (dilated lymphatic capillaries in the papillary layer) (Table ) .…”

“…Clinical suspicion of any relevant differential diagnosis should prompt the appropriate diagnostic workup . The differential diagnoses differ with respect to their site of predilection and clinical presentation (Figure ) and may also play a relevant role in the form of comorbid skin conditions. LFSS Connective tissue diseases: progressive systemic sclerosis, Shulman's syndrome, mixed connective tissue disease/Sharp syndrome, circumscribed scleroderma/morphea. Pseudosclerodermas/mucinoses: scleredema/scleredema adultorum of Buschke, diffuse and pretibial myxedema, lichen myxedematosus/scleromyxedema, acral persistent papular mucinosis, cutaneous focal mucinosis. Others: angioedema, genital and extragenital lichen sclerosus et atrophicus, graft‐versus‐host disease, dermatomyositis, panniculitis, Sudeck's disease, calcified hematoma, malignant and benign tumors, superficial venous thrombosis (SVT), post‐thrombotic syndrome (PTS), hard chancre (syphilis), and granuloma inguinale. LFSS associated with epidermal conditions Common warts, contact dermatitis, actinic keratoses, psoriasis, atopic dermatitis, hypertrophic lichen planus. Lymphovascular reflux of the papillary dermis (dermal lymphocysts, lymphocutaneous fistulas with chylous or non‐chylous lymphorrhea, leg ulcer, lymphangioma circumscriptum) Tension blisters in cardiac edema, bullous disease of diabetes, autoimmune bullous dermatosis, recurrent herpes simplex, fibroma, HPV‐associated acanthoma. Lymphovascular reflux in the subcutis (lymphoceles) Subcutaneous LFSS, lipoma, lymphadenopathy, cutaneous metastases.…”

S1‐Leitlinie – Dermatosen bei dermaler Lymphostase

“…Bei Bewegung der Betroffenen im Wasser sind diese Symptome meist deutlich geringer ausgeprägt. Dermale Lymphzysten und lymphokutane Fisteln können durch Inspektion und/oder histopathologisch (dilatierte, initiale Lymphgefäße des Stratum papillare) diagnostiziert werden (Tabelle ) .…”

“…Lymphvaskulärer Reflux in der Subkutis (Lymphozelen) Subkutane LFSH, Lipom, vergrößerte Lymphknoten, kutane Metastasen. .…”