Idiopathic non-familial Acro-osteolysis: A rare case report

Abstract

A 25-year-old woman patient presented with shortening of fingers with racket nails and numerous yellowish papules over the hands and forearms for 21 years. X-ray of the hands revealed destructive osteolytic changes in all the terminal phalanges. Skin biopsy from the yellowish papules showed epidermal proliferation, perivascular mononuclear infiltrate, thickening of dermal collagen, septal fibrosis and loss of adipocytes mimicking sclerodermatous changes in the dermis and hypodermis. The patient did not have any history of similar illness in the family or occupational exposure to vinyl chloride. After excluding all other possibilities of acral-osteolysis, we diagnosed the case as idiopathic non-familial variety of acro-osteolysis. This is a rare entity characterized by terminal resorption of fingers, sometimes associated with Raynaud's phenomena and yellowish cutaneous papules.

Nail changes in acro-osteolysis: A case report and review of the literature

“…Brachyonychia refers to short, broad, flat nails, and is the most commonly reported nail change 2 , 4 , 5 , 7, 8, 9, 10. The term racquet nail is synonymous but implies associated deformity of the bone and soft tissue 11, 12, 13. Other reports include anonychia, atrophy, transverse ridging, discoloration, thickening of the nail plate, hyperkeratosis of the cuticles, pincer nails pitting, and onycholysis 13, 14, 15, 16, 17, 18, 19, 20.…”

Idiopathic Acroosteolysis: A Novel Cutaneous Sign Can Help Identify the Condition Early

“…IAO typically begins in early childhood, adolescence, or early adulthood and progresses through life, although we have report of its onset in mature adulthood. It is an extremely rare condition with only very few cases reported in the English literature [1,3,[7][8][9][10]. Several cases of IAO of the hands have been associated with progressive osteolysis of the mandibular ramus and multiple cortical defects [9,10].…”

Brachyonychia Associated with Acroosteolysis in Chronic Kidney Disease: How Phalange Shape Influences Nail Morphology

“…AO describes the partial or total lytic destruction of the finger or toe phalanges, characterized by resorption of the bones [2-4, 8, 9]. It can be classified as primary or familial, idiopathic or nonfamilial, and occupational or secondary, as a consequence of several conditions.…”

“…Radiographic appearance of phalangeal osteolysis can be divided into three groups: those with resorption of terminal tufts, resorption of the midportion of the phalanges, and those with periarticular resorption [8]. Only two variants orient the clinical manifestations: transverse variant of AO presents an osteolytic band in the midportion of the phalange preserving its base and tuft, whereas those with tuft resorption present a pencil-like pattern, also known as longitudinal AO [1, 4] (Fig.…”

“…It can be classified as primary or familial, idiopathic or nonfamilial, and occupational or secondary, as a consequence of several conditions. The most common causes being inflammatory diseases, sensory neuropathy, vascular disease, metabolic diseases, exposure to vinyl chloride, and trauma (Table 2) [1, 2, 8-10]. …”