Primary Atrophic Profound Linear Scleroderma

Abstract

We present 3 unusual cases of deep linear, primary atrophic scleroderma, not preceded by inflammatory reaction and sclerosis, involving the subcutis and deeper tissues. These cases differ in the course and prognosis from typical profound scleroderma since they do not lead to disfiguration and crippling deformities. In contrast to the atrophies left after regression of morphea or linear scleroderma, they do not involve the dermis, which does not show discoloration or changes in texture. Infiltrates in the endomysium, involvement of deeper tissues and the progressive character of the disease argue for atypical primary atrophic profound scleroderma. The coexistence in one case of primary facial hemiatrophy appears to indicate also its relationship with primary linear atrophies of the limbs.

Primary Atrophic Solitary Morphea Profunda

“…Thus, the features were similar to those described by Jablonska and colleagues [10 ]in 3 patients with primary atrophic profound scleroderma. However, in these cases, the involvement was linear, band-like and more extensive.…”

Bilateral linear scleroderma "en coup de sabre" associated with facial atrophy and neurological complications

“…There are no reliable histological criteria to differentiate PRS from involutionary scleroderma. Recently, Blaszczyk et al [7] reported three cases of primary atrophic profound linear scleroderma with involvement of the subcutis and deeper tissues. In accordance with a previous report of Malandrini et al [8], deep skin biopsies showed inflammatory infiltrates in the endomysium and perimysium.…”

“…Interestingly, atrophy was not preceded by clinical evidence of inflammation, discoloration of the skin, or sclerosis. Blaszczyk et al [7] concluded that PRS could be regarded as a variety of deep linear scleroderma [7,9]. …”

Unexplained muscle atrophy as the unique preceding symptom of bilateral linear morphea

“…Blaszczyk et al 4 delineated primary atrophic profound linear scleroderma ( = deep atrophy of subcutis and deeper tissues without preceding inflammatory changes) as a new entity. Accordingly, the distinct muscle atrophy of our patient may be a primary atrophic profound linear scleroderma followed by the classical form of linear scleroderma.…”