Elastosis Perforans Serpiginosa: a D-penicillamine induced dermatoses in a patient with Wilson s disease

Abstract

Long term use of D-penicillamine for Wilson's disease can be associated with many adverse reactions and systemic side effects. We report the case of a 28-year-old male patient diagnosed with Wilson's disease presenting with a serpiginous raised violaceous skin lesion in the anterior aspect of the neck over the last six months and two small papules with central umbilication during the last month. Histopathological examination of skin lesions demonstrated transepidermal perforating channel, and the Verhoeff's-van Gieson stain showed marked increase number of irregular serrated elastic fibers suggesting the diagnosis of D-penicillamine induced elastosis perforans serpiginosa.

The Role of Zinc in the Treatment of Wilson’s Disease

“…The brain is more sensitive to increased levels of free copper [ 1 , 14 , 111 ]. Moreover, several studies, including some long-term investigations, indicate that treatment with penicillamine, trientine, or tetrathiomolybdate may be accompanied by serious adverse effects [ 4 , 31 , 111 , 112 , 113 , 114 , 115 , 116 , 117 , 118 , 119 , 120 , 121 ], some of which are irreversible and fatal [ 106 , 115 , 122 ]. These drugs are also expensive for patients and health services [ 123 , 124 ].…”

Elastic oddity: A case report of a rare occurrence of elastosis perforans serpiginosa in Down’s syndrome

“…It is associated with connective tissue disorders like Ehlers-Danlos syndrome, Marfan syndrome, cutis laxa and osteogenesis imperfecta. Drug induced EPS is associated with D-penicillamine [1,2].…”

“…It is characterized clinically by papules and keratotic, desquamative, normochromic or erythematous plaques, usually affecting the face, neck, trunk and upper limbs. Histopathology is the gold standard for diagnosis where thickened, vertically arranged elastic fibres are seen penetrating the skin forming narrow canals [1,2].…”

“…Dammert and Putkonem changed into EPS in 1958 [1,3]. EPS usually presents between 5 and 20 years of age, but the age can range from 5 to 89 years [2]. More than 75% of the affected cases are male, and there appears to be no race predilection [1][2][3].…”

Generalized scleroderma‐like induration associated with D‐penicillamine elastosis perforans serpiginosa in Wilson's disease

“…Literature retrieval to find previous description of such widespread indurated skin alteration addressed the differential diagnosis with other known DPA-induced dermal damages, [3][4][5][6][7][8][9][10][11][12] which could be expressed concomitantly in our patient.…”