A Variant of Palpable Migratory Arciform Erythema
October 2014 | Volume 13 | Issue 10 | Case Reports | 1288 | Copyright © October 2014
Ellinor R. Quay MD,a Jyoti P. Mundi MD,b Jo-Ann M. Latkowski MD,b
Hideko Kamino MD,b and Jesse M. Lewin MDb
aDepartment of Dermatology, Boston University School of Medicine, Boston Medical Center, Boston, MA
bNew York University Langone Medical Center, Ronald O. Perelman Department of Dermatology, New York, NY
Palpable migratory arciform erythema is a T-cell pseudolymphoma with no well-established treatment. The disease is rarely reported in the literature, perhaps because it is difficult to diagnose. We present a case of a variant of PMAE in a 30-year-old healthy man with no history of medication use and erythematous to violaceous annular and arciform plaques on his face, scalp and trunk. This case is of particular significance because gene rearrangement studies and histopathologic findings are concerning for folliculotropic mycosis fungoides while the clinical course does not support this diagnosis. The authors' emphasize that clinical history is imperative for definitive diagnosis of palpable migratory arciform erythema as it can clinically and histopathologically resemble other cutaneous lymphocytic diseases. J Drugs Dermatol.
A 30-year-old healthy man with no history of medication use presented with a two-year history of a pruritic, waxing and waning rash on his head, neck and trunk. On physical examination he had erythematous to violaceous annular and arciform plaques ranging from 1cm to 2.5cm on his face, frontal scalp, and post-auricular regions (Figure 1) with sparing of the rest of his body and mucosa. No hepatosplenomegaly or lymphadenopathy were noted. Individual skin lesions lasted days to weeks and no identifiable triggers or remitting factors were identified by the patient. Two years prior, the patient had similar lesions and underwent a systemic work-up. Upon presentation to our department, a punch biopsy was obtained from an annular plaque from the left post-auricular region.
Histopathologic examination of the skin biopsy specimen showed a superficial and mid-dermal perivascular and perifollicular infiltrate of lymphocytes with mild follicular mucinosis (Figure 2). A few lymphocytes extended to the dermoepidermal junction. Immunostaining demonstrated a predominantly CD3 positive T-lymphocyte infiltrate with a 4:1 ratio of CD4 to CD8 positive lymphocytes and 50% and 60% loss of CD5 and CD7 expression, respectively. A few CD30 positive intermediate-sized mononuclear cells and CD20 positive B-lymphocytes were also noted.
Biopsies of similar lesions on the scalp and back performed two years prior revealed comparable findings. At that time, gene rearrangement studies demonstrated clonal T-cell receptor gamma chain gene rearrangement and an extensive systemic work-up including laboratory tests, PET-CT and a bone marrow biopsy showed no hematological or immunological abnormalities.
Per patient report, the initial lesions were refractory to several topical steroids but resolved spontaneously after four months. The patient presented to our department two years later when the lesions recurred in the absence of any new medications. Another trial of topical steroids was initiated but the lesions remained. Within two months off topical medications, the plaques spontaneously regressed.
Palpable migratory arciform erythema is characterized by sharply demarcated, annular erythematous patches and plaques that wax and wane over the course of days to weeks, in an arciform progression.1,2 First described in 1974 as a unique disease entity, it has been suggested that palpable migratory arciform erythema is a type of T-cell pseudolymphoma.2 Histopathology demonstrates perivascular and periadnexal lymphocytic infiltrate with no epidermal involvement and an absence of plasma cells and copious mucin.1 Immunophenotyping reveals a predominantly CD4+ T-cell infiltrate and gene rearrangement studies traditionally demonstrate polyclonality.1 There is no well-established treatment for palpable migratory arciform erythema. While antibiotics have been linked to the clearance of the lesions in palpable migratory arciform erythema, no causative bacteria has been detected.1 Topical steroids have also been used but were not successful in clearing the lesions in our patient.5 Despite medical therapy, the lesions often recur.1,5
Palpable migratory arciform erythema remains an infrequently reported disease. Some authors believe that palpable migratory arciform erythema is a form of lymphocytic infiltration of Jessner-Kanof (LIS-JK), rather than a distinct entity.4 However, these two entities can be differentiated based on location and