Pseudoxanthoma Elasticum and its Rare Co-Existence with Comedones and Neurofibroma

A 68 year old male patient, presented with multiple asymptomatic elevated skin lesions over the face, V area of neck since last 40 years. Physical examination revealed numerous open comedones all over the face, forehead and V area of neck. Numerous firm, skin colored to yellowish, non-follicular papules coalescing into plaques were seen around the neck, suprascapular region, both axillae, cubital and popliteal region with redundant lax skin folds. A single pedunculated nontender firm swelling of 1X1 cm size is seen over the left side of the chest. Histopathological examination of punch biopsy from lesional skin showed irregularly clumped faintly basophilic elastic fibres admixed with mucoid material in the mid and lower dermis. Excisional biopsy from the lesion over the chest showed skin with underlying dermal circumscribed, non encapsulated tumour composed of spindle cells arranged in fascicles. Patient improved with topical and systemic retinoids. We are reporting this case as it's a rare association with pseudoxanthoma elasticum.