Acral localized acquired cutis laxa: Report of a case associated with inflammatory arthritis

Abstract

classification of Clark et al, 1 this case suggests that we should keep verrucous-keratotic malignant melanoma in mind as an important differential diagnosis for verrucous, hyperkeratotic lesions. In this case, the subtle perilesional clinical appearance of dark infiltration at the ridges might have been a clue to the diagnosis of melanoma.The intercellular adhesion between melanoma cells is much weaker than that between normal keratinocytes, because melanocytes or melanoma cells do not have desmosomes. In addition, membranous expression of E-cadherin, which is responsible for melanocyte-keratinocyte adhesion, is sometimes decreased in melanoma progression. 2 Thinning of the epidermis with attenuation of the basal and suprabasal layers, described as ''consumption of the epidermis,'' is frequently seen in areas of direct contact between the epidermis and melanoma cell nests. 3 Consumption of the epidermis occasionally results in the formation of a cleft separating the thin epidermis and melanoma cell nests. 3 Braun-Falco et al 4 reported that the clefts are a reliable diagnostic criterion for malignant melanoma. It has been suggested that the greater the Breslow depth, the greater the consumption of the epidermis in a melanoma lesion. 5 However, the Breslow depth of the current lesion did not appear to be very great, although we were unable to measure it accurately because of the large size of the cleft. The presence of the verrucous architecture may be associated with the presence of epidermal consumption. In the current case, melanoma cells completely replaced the basal layer keratinocytes, forming only a single-layer lining on the basement membrane. This characteristic infiltration pattern of melanoma cells with fragile cell adhesion might have lead to the large cleft with features mimicking the tombstone appearance of pemphigus vulgaris, although in the current lesion, the tombstones were melanoma cells instead of the basal keratinocyte tombstones of pemphigus vulgaris lesions.

Generalized acquired cutis laxa type 1: a case report and brief review of literature

“…Although the localized variant has a pure cutaneous involvement, the generalized variant in addition to cutaneous changes has a wide spectrum of systemic involvement in the form of pulmonary emphysema, pneumothorax, aortic ectasia, cor pulmonale, inguinal and umbilical hernias, gastrointestinal and urogenic diverticulae, inflammatory arthritis, cutaneous mastocytosis, and developmental retardation [7,[13][14][15]. Our patient did not have any of the above mentioned systemic manifestations.…”

“…Evidence in favor of this mechanism is the occurrence of acral or generalized CL in association with monoclonal gammopathies and observation of IgG, IgA and/or amyloid deposition in the lesional skin [1,11]. In addition, abnormal copper metabolism, deficiency of copper, decreased production of tropo-elastin, and genetic susceptibility for the development of CL have all been postulated [1,12,13].…”

Acral localized acquired cutis laxa as presenting sign of underlying systemic amyloidosis

“…This form of cutis laxa appears to be an acquired condition, and it has been associated with either preceding inflammatory conditions or amyloid deposition with an underlying plasma cell dyscrasia. [1][2][3][4][5][6][7][8][9][10] The diagnosis can be challenging, because of both its rarity and the subtle histopathologic findings of involved skin.…”

“…ALACL is rare condition characterized by loose, redundant skin of the hands or feet which histopathologically is consistent with other forms of cutis laxa with reduced or fragmented dermal elastic fibers. This form of cutis laxa appears to be an acquired condition, and it has been associated with either preceding inflammatory conditions or amyloid deposition with an underlying plasma cell dyscrasia 1‐10 . The diagnosis can be challenging, because of both its rarity and the subtle histopathologic findings of involved skin.…”

“…Since then, 10 additional cases have been reported. These cases have been associated with episodes of recurrent inflammation, [2][3][4][5] or with amyloid deposition within the affected tissue as a presenting sign of systemic plasma cell dyscrasia. [6][7][8][9] One case reported ALACL in a patient with chronic urticarial dermatosis and IgA myeloma but without amyloid deposition.…”