UPMC Dermatopathology "Case of the Month" Presentations
UPP - Department of Dermatology, Dermatopathology Unit
Case Authors: Joanne Simpson MD, MPH, Larissa Geskin MD, Drazen M. Jukic, MD, PhD
JUNE 2005 CASE OF THE MONTH
DISCUSSION & DIAGNOSIS
Diagnosis
Lymphomatoid papulosis, Type 3A4
Discussion
Lymphomatoid papulosis (LyP) is an indolent chronic, recurrent lymphoproliferative disorder that lies in a spectrum of CD30-positive low-grade spectrum cutaneous T-cell lymphomas. The hallmark of these disorders is the presence of highly atypical tumor lymphocytes expressing CD30 (BerH2) antigen and similar T-cell receptor clonal rearrangement (1).
Clinically, LyP presents as recurrent crops of papulonodular or plaque like lesions, often with hemorrhagic crusting or ulceration most commonly seen on the trunk and extremities. Although its clinical appearance is benign and its prognosis is favorable, the histologic and cytologic features of LyP demonstrate features of malignancy. LyP has a variable histologic presentation. The most common form of LyP, LyP type A, is characterized by a wedge-shaped dermal infiltrate comprised of anaplastic lymphoid cells in a background of small lymphocytes, often with neutrophils, eosinophils, or both. Type B LyP is typified by lichenoid dermal infiltrate of small lymphocytes with epidermotropic infiltration of the epidermis in a manor that resembles mycosis fungoides. LyP Type C, on the other hand, demonstrates diffuse infiltration of the dermis by sheets of tumor cells and sparse neutrophils (2). Immunophenotypically, each of these forms of LyP demonstrates clonal, activated helper T cells with positive expression of CD3, CD4, CD5, and CD30 markers. These cells stain negatively for CD8, CD15, and EMA (3).
Grover's disease, also known as transient acantholytic dermatosis, demonstrates discrete pruritic, hyperkeratotic papules and papulovesicles seen mainly on the back and thighs of middle-aged to elderly males. Histologically, the hallmark of Grover's disease is the presence of focal acantholysis. Four variants of Grover's disease exist, Darier's disease-like (acantholytic dyskeratosis), Hailey-Hailey disease like (suprabasal cleft with significant acantholysis), pemphigus vulgaris-like (suprabasal acantholysis), pemphigus foliaceus-like (superficial acantholysis). Immunofluorescence and immunohistochemical studies have not demonstrated consistent patterns of positivity in this disease (2).
Grover's disease and lymphomatoid papulosis can often clinically resemble one another, however, these two entities are usually distinctive histologically. This case represents an unusual presentation of persistent, recurrent, pruritic papules in a 56 year-old man?a clinical presentation suggestive of Grover's disease, but with histologic findings of both Grover's disease and lymphomatoid papulosis, type A. To date, there are only 2 reports in the literature of a similar histologic pattern, in which there is significant epidermal atypia in association with CD30+ dermal infiltrate (4,5). Both of these reports included cases in which squamous cell carcinoma atypical lymphocytic proliferation with features of CD30+ lymphoma and LyP. Our patient's histopathologic findings of epidermal acantholysis superimposed on a wedge-shaped dermal infiltrate of atypical CD 30-lymphocytes may represent an atypical variant of lymphomatoid papulosis, in which the epidermal component resembles Grover's disease.