Poikilodermatous Subacute Cutaneous Lupus erythematosus

Abstract

Background: Subacute cutaneous lupus erythematosus (SCLE) is a distinct subset of lupus erythematosus with unique clinical, immunological and genetic features. Among the unusual variants of SCLE, there is a poikilodermic presentation. However, to date, only 1 case of poikilodermatous SCLE has been reported. Objective: Our goal was to summarize the clinical characteristics and course as well as the pathological, laboratory and immunofluorescence findings of 4 patients with poikilodermatous SCLE. Methods: A retrospective study was conducted including 54 patients diagnosed as having SCLE between 1980 and 2002. Results: Four patients (7.4%) had SCLE. All patients were alive, and none developed severe systemic involvement in up to 36 years (median, 24 years) after the onset of disease. The most noteworthy laboratory finding was the cutaneous deposition of amyloid. Conclusion: Poikilodermatous SCLE represents an uncommon variant within the clinicopathological spectrum of SCLE following a favorable course, in spite of extensive cutaneous involvement. Photosensitivity is the pathomechanism explaining, theoretically, the development of both poikiloderma and cutaneous amyloidosis in such cases.

What Is Poikiloderma?

“…The number of diseases or syndromes manifesting as acquired poikiloderma is limited. Thus, the report of Marzano et al [1] of 4 patients with poikilodermatous subacute cutaneous lupus erythematosus is certainly of clinical interest. The authors included only patients with ascertained subacute cutaneous lupus erythematosus, with a typical immunological profile, who had authentic poikiloderma and not only atrophy, pigmentary changes or telangiectases.…”

“…In this issue of Dermatology, Marzano et al [1] report a poikilodermatous variant of subacute cutaneous lupus erythematosus. But what is poikiloderma?…”

Drug-induced subacute cutaneous lupus erythematosus: evidence for differences from its idiopathic counterpart

“…Clinically, SCLE presents with nonscarring annular or papulosquamous eruptions on sun‐exposed skin, mainly the upper back, chest, dorsal arms and lateral neck. Rare pityriasiform, bullous, erythrodermic, poikilodermatous, toxic epidermal necrolysis‐like and erythema multiforme (EM)‐like presentations have been described 6–10 . Anti‐Ro/SSA antibodies are classically regarded as the serological marker for this subset 11 .…”

Subacute cutaneous lupus erythematosus presenting in twins

“…They may also present with scale, pigment alteration, and telangiectases . Less common presentations of SCLE include poikilodermatous, erythrodermic, and bullous variants, which require a higher index of suspicion for diagnosis. Histopathologic analysis shows dermal edema and mucin, as well as a perivascular infiltrate by mononuclear cells.…”

“…Histopathologic analysis shows dermal edema and mucin, as well as a perivascular infiltrate by mononuclear cells. Vacuolar degeneration of the dermoepidermal junction is seen, along with epidermal atrophy . Direct immunofluorescence may demonstrate a granular IgG pattern in SCLE …”