Systemic amyloidosis presenting with glans penis involvement

Abstract

Penile amyloidosis has been reported on many occasions in the literature, but all of these have been forms of primary cutaneous amyloidosis. Systemic amyloidosis presenting with a penile ulcer as the first manifestation has not previously been reported. We present two patients in whom an ulcer of the glans penis was the first complaint that led to a diagnosis of systemic amyloidosis. In both patients, lambda light chain type amyloid was showed immunohistochemically. Both patients presented with other manifestations of systemic amyloidosis, including nail dystrophy characterized by onycholisis, trachyonychia and onychoschizia.

Trachyonychia and Twenty-Nail Dystrophy: A Comprehensive Review and Discussion of Diagnostic Accuracy

“…A number of other cutaneous and systemic diseases have been associated with trachyonychia including the following: amyloidosis [5], atopic dermatitis [6], treatment with chemotherapeutic agents [7,8], hematologic abnormalities (i.e. idiopathic thrombocytopenia) [9], ichthyosis vulgaris [10,11], immune dysregulation polyendocrinopathy enteropathy X-linked syndrome (IPEX) [12], immunoglobulin A deficiency [13], incontinentia pigmenti (Bloch-Sulzberger syndrome) [14], pemphigus vulgaris [15], primary biliary cirrhosis [16], reflex sympathetic dystrophy [17], and sarcoidosis [18,19].…”

“…For example, although trachyonychia has been associated with amyloidosis, the pictures illustrating the case reports with this association show lichen planus-like changes with severe onychorrhexis but not trachyonychia [5,23]. …”

“…We found that trachyonychia and twenty-nail dystrophy have been misdiagnosed in the literature in several cases [5,35,41,48,49,50,51,52](table 1). The diagnosis is often incorrectly based on the presence of dystrophic nail changes on all twenty nails, despite the fact that the nails do not show specific signs of trachyonychia.…”

Systemic amyloidosis‐induced nail dystrophy

“…SAND can be the presenting sign of systemic amyloidosis [2]. Cases of SAND have been misdiagnosed as trachyonychia [1,[4][5][6]. Differentiating between these entities is crucial, since systemic amyloidosis can cause multiple organ failure.…”

Nail Biopsy in the Diagnosis of Systemic Amyloidosis

“…While nail changes are a relatively rare finding of systemic amyloidosis, they have been noted in at least 32 cases to date (Table 1). 1,[3][4][5][6][7][8][9][10][11][12] The nail changes most often described include increased brittleness, longitudinal ridging, and onycholysis, though chronic paronychia and verrucous subungual plaques have also been observed. 1,[10][11] Easy bruising, purpura, ecchymoses, and/or petechiae represent the most common cutaneous findings overall, present in 44 percent of the cases.…”