Monoclonal Rearrangement of the T Cell Receptor Gamma-Chain in Lichenoid Pigmented Purpuric Dermatitis of Gougerot-Blum Responding to Topical Corticosteroid Therapy

Abstract

Lichenoid pigmented purpuric dermatitis of Gougerot-Blum belongs to a group of closely related disorders which are termed pigmented purpuric dermatoses. It clinically manifests itself with grouped lichenoid papules in association with purpuric lesions. We report a case of lichenoid pigmented purpuric dermatitis of Gougerot-Blum with a heavy band-like CD4-positive lymphocytic infiltrate and clonal rearrangements of the γ-chain of the T cell receptors as detected by polymerase chain reaction/denaturing gradient gel electrophoresis. Monoclonal expansion of T cells in combination with certain histological features of mycosis fungoides (MF) might support a biological relationship between lichenoid pigmented purpuric dermatitis of Gougerot-Blum and MF. However, prompt clinical response to topical steroid therapy supports the benign clinical nature of our case.

CCR4 Expression by Atypical T Cells in Systemic Pilotropic Lymphoma: Its Behavior under Treatment with Interferon Gamma, Topical PUVA and Systemic Retinoid

“…There are several clinical subtypes in MF, i.e. granulomatous MF [2], hyperkeratotic MF [3], hypopigmented MF [4], MF associated with follicular mucinosis [5], MF bullosa [6], MF palmaris et plantaris [7, 8, 9], ichthyosiform MF [10], granulomatous slack skin [11], MF evolving from pityriasis lichenoides chronica [12], lichenoid pigmented purpuric dermatitis of Gougerot-Blum [13]and pagetoid reticulosis [14]. Recently, in relation to MF, a distinct clinicopathological variant, pilotropic lymphoma, has been described [15, 16].…”

Pigmented Purpuric Dermatosis

“…19,21 Earlier studies have demonstrated clonality in PPD. [7][8][9]13 Almost 50% of the cases in our study showed a monoclonal or oligoclonal pattern. From a molecular perspective, the demonstration of an identical T-cell clone at multiple sites over time in several of our patients is corroborative evidence that this process is a clonally driven one without a discernible antigenic trigger.…”

“…7 There is an emerging body of clinical, phenotypic, and molecular data suggesting that PPD may represent an insidious epitheliotropic T-cell dyscrasia. [7][8][9][10][11][12][13][14][15] Although progression to mycosis fungoides (MF) is described, the literature precedent in this regard is limited, with only 11 reported cases. 9,11,12,[14][15][16] However, it is possible that underreporting accounts for the few patients described in the literature.…”

Pigmented Purpuric Dermatitis Preceding the Diagnosis of Mycosis fungoides by 24 Years

“…Thus, it is not possible to know if histological signs of MF were present from the beginning. However, histology, immunohistochemistry and TCR gene analysis are not always sufficient to distinguish PPD from early MF [3,4].…”

“…Since the beginning of the 1990s, MMF has been used as an immunosuppressive agent to prevent acute rejection after organ transplantation [2]. MMF has also been used alone or in combination with corticosteroids in autoimmune blistering diseases such as bullous pemphigoid [3][4][5], pemphigus vulgaris [4][5][6] and pemphigus foliaceus [4,7]. Recently, 2 reports on the therapeutic effect of MMF in epidermolysis bullosa acquisita (EBA) have been published with conflicting results [8,9].…”