UPMC Dermatopathology "Case of the Month" Presentations
UPP - Department of Dermatology, Dermatopathology Unit
Case Authors: Justin Vujevich MD, Cynthia Mates MD, Drazen Jukic MD
JANUARY 2005 CASE OF THE MONTH
DISCUSSION & DIAGNOSIS
Diagnosis
Acrokeratoelastoidosis of Costa
Discussion
Acrokeratoelasoidosis is a rare dermatological condition originally reported by a Brazilian dermatologist, Oswaldo Costa, in 1953 (1). The majority of cases present during infancy or childhood. Cases have been reported with autosomal dominant transmission or sporadic occurrence.
Clinically, patients present with small, flesh-colored or yellowish, flat-topped papules and plaques on the hands and feet. Lesions are typically located on thenar and hypothenar skin, Wallace’s lines (the junction between palmoplantar and glaborous skin), knuckles, and nail folds. Lesions are usually asymptomatic.
Histology shows prominent hyperkeratosis, hypertrophy, and hypergranulosis in the epidermis. In the dermis, there is decreased numbers of elastic fibers, with fragmentation of the elastic fibers (elastorrhexis). There is no inflammatory infiltrate, nor additional characteristic features of the epidermis or dermis.
The pathophysiolgy is unknown. Johansson et al. have suggested a defect in the secretion of elastic fibers from fibroblasts (2). Differential diagnosis includes other marginal keratodermas, including: focal acral hyperkeratoisis (FAH), degenerative collagenous plaques of the hands (DCP), and keratoelastoidosis marginalis (KEM).
FAH clinically resembles acrokeratoelastoidosis of Costa, but does not demonstrate elastorrhexis in the dermis on skin biopsy (3). DCP and KEM lesions are typically found in older Caucasians with a history of chronic sun exposure and manual labor, with lesion distribution on the index finger and opposing margin of the thumb. Histological exam demonstrates marked degeneration of collagen and elastin fibers in DCP and senile elastosis and papillary vascular ectasia in KEM (4,5).
Other diseases in the differential diagnosis include verrucae planae, acrokeratosis verruciformis of Hopf, palmar xanthomas, sarcoidosis, and puctate keratoderma of the palms and soles.
Treatment is typically not required, but if symptomatic, can include cryotherapy, topical retinoids, salicylic acid, oral retinoids, or oral prednisone.
References:
1.Costa O. Acrokeratoelastoidosis. Dermatologica. 1953;107:164-168.
2. Johansson EA, Kariniemi AL, Niemi KM. Palmoplantar keratoderma of punctate type: acrokeratoeleastoidosis of Costa. Acta Derm Venereol. 1980;60:149-153.
3. Hu W, Cook TF, Vicki GJ, Glaser DA. Acrokeratoelastoidosis. Pediatr Dermatol. 2002;19:320-322.
4. Burks, JW, Wise LJ Jr., Clark WH. Degenerative collagenous plaques of the hands. Arch Dermatol. 1960;82:362-366.
5. Kocsard E. Keratoelastoidosis marginalis of the hands. Dermatologica. 1965;131:169-175.