Dermatology Online Journal
Capillaritis as a potential harbinger to cutaneous T-cell lymphoma
- Author(s): Shen, Angela, MD
- Metz, Brandie J, MD
- Ehrig, Tosten, MD
- Sinkre, Prasanna, MD
- Hsu, Sylvia, MD
- et al.
Capillaritis as a potential harbinger of cutaneous T-cell lymphoma
Department of Dermatology, Baylor College of Medicine, Houston, TexasaCockerell and Associates, Dallas, Texasb. email@example.com
Angela Shen MDa, Brandie J Metz MDa, Tosten Ehrig MDb, Prasanna Sinkre MDb, and Sylvia Hsu MDa
Dermatology Online Journal 10 (2): 15
Mycosis fungoides (MF), a form of cutaneous T-cell lymphoma (CTCL), is a primary monoclonal T-cell lymphoma that initially manifests in skin but later may expand to involve the entire lymphoreticular system. The cutaneous manifestations range from refractory, eczematous, psoriasiform eruptions to nonspecific bullous, hypopigmented, hyperkeratotic, and granulomatous forms. We report a case of biopsy-proven cutaneous T-cell lymphoma that was preceded by pigmented purpuric dermatosis, or capillaritis, suggesting the importance of capillaritis as a potential harbinger to early CTCL.
Patient is an elderly 77-year-old man with a past medical history significant for hypertension, hypercholesterolemia, peripheral vascular disease, and coronary artery disease. He presented with a 2-month history of an asymptomatic rash on bilateral lower extremities nonresponsive to prior treatment with terbinafine cream, triamcinolone 0.1 percent cream, and mupirocin 2 percent ointment. His other medications included clopidogrel, atorvastatin, metoprolol, pentoxifylline, nizatadine, tramadol, acetaminophen, aspirin, glucosamine, and a daily multivitamin tablet. The lesions were initially noted as small annular erythematous macules and discrete brown patches on bilateral ankles, which then extended to involve both thighs (Fig. 1). Two 4-mm punch biopsies were obtained, and a diagnosis of purpura annularis telangiectodes of Majocchi was made. Microscopic examination revealed a histological picture consistent with capillaritis, characterized by a superficial perivascular infiltrate of lymphocytes and histiocytes with focal hemosiderin deposition and red blood cell extravasation (Fig. 2).
|Figure 3||Figure 4|
|Indurated violaceous plaques with erythematous halos of cutaneous T-cell lymphoma (Fig. 3).|
|Dermal infiltrate of neoplastic atypical lymphocytes with focal epidermal exocytosis (Fig. 4).|
Approximately 2 months after the initial diagnosis of capillaritis, the patient returned to us complaining of a 5-week history of new indurated lesions on the left thigh. Physical examination revealed four indurated violaceous plaques with erythematous halos on the left thigh (Fig. 3). Histological sections of two 4-mm punch biopsies demonstrated a dermal infiltrate of atypical lymphoid cells with prominent central nucleoli, nuclear atypia, and increased mitotic activity. Focal exocytosis into the superficial epidermis was also noted (Fig. 4). Immunohistochemical staining of the atypical lymphocytes were strongly positive for T-cell marker CD3 and negative for B-cell marker L26. The tumor cells also lacked staining for CD56 and CD30, excluding the diagnosis of lymphomatoid papulosis, Hodgkin disease, and natural killer cell neoplasms. The microscopic findings, combined with the immunohistochemical profile, rendered the final diagnosis of cutaneous T-cell lymphoma.
The term cutaneous T-cell lymphoma was formally adopted in 1979 at an international symposium held by the National Cancer Institute . Primary cutaneous non-Hodgkin T-cell lymphoma usually has a peak incidence in patients in the sixth or seventh decade of life, as exemplified by the patient in this case report. Clinical manifestations of early CTCL are protean, and cutaneous findings can range from early refractory eczematous and psoriatic plaques to progressive nodular stages with or without ulceration . Extensive cutaneous tumor infiltration can ultimately result in leonine facies, confluent erythroderma, palmoplantar keratoderma, and alopecia. A low threshold for the diagnosis of mycosis fungoides should exist when patients have persistent nondiagnostic patches and apparent intractable psoriasis and eczema. Other premalignant cutaneous T-cell dyscrasias include, but are not limited to, alopecia mucinosa, lymphomatoid papulosis, poikiloderma vasculare atrophicans, and large-plaque parapsoriasis .
The natural progression of CTCL is largely divided into four stages: the early patch stage, the plaque stage, the tumor stage, and the erythrodermic stage [3, 4]. Early patch stage is characterized by single or multiple erythematous to dusky, scaly macules and patches on unexposed body surfaces, mimicking eczema, psoriasis, or dermatophytosis. These initial lesions may spontaneously regress and recur. The patch stage is highly variable and can give rise to or coexist with the plaque stage. CTCL plaques can be annular in addition to having an increasing lichenoid and serpiginous appearance. In contrast to the patch and plaque stages, the tumor stage of MF has a predilection for the face and body folds and exemplifies the vertical nodular growth phase of malignant lymphocytes with extension into the subcutis. The final erythrodermic stage is also known as Sèzary syndrome, which represents a leukemic stage of CTCL . Patients usually present with generalized erythroderma and elevated peripheral Sèzary cell counts.
Because of the variable cutaneous findings, multiple biopsies at several-month intervals should be performed until a firm diagnosis of cutaneous lymphoma can be made. Similar to the diverse gross findings, microscopic examination of CTCL also demonstrate great variability. The histology usually reveals a dense mononuclear infiltrate in the papillary dermis with epidermotropism and Pautrier microabscess formation (discrete epidermal clusters of lymphocytes in close apposition) . Cytologic atypia of the infiltrative lymphocytes can range from mild to marked. Thus, architectural distortion rather than cytologic appearance is more reliable in the diagnosis of early CTCL . The atypical lymphocytes also have strong immunohistochemical staining for CD2 and CD3.
A plethora of nonspecific cutaneous findings have been reported as having the potential to give rise to CTCL. One such generic premalignant group includes the pigmented progressive purpuric dermatoses (PPPD), otherwise known as capillaritis. The term PPPD encompasses progressive pigmentary dermatosis (Schamberg purpura), purpura annularis telangiectodes (Majocchi disease), lichen aureus, eczematous purpura of Doucas and Kapetanakis, and purpuric lichenoid dermatosis of Gougerot-Blum . The skin histology shows a gamut of erythrocyte extravasation, hemosiderin deposition, and lymphocytic infiltrate. Currently a handful of cases of CTCL preceded by capillaritis has been reported [2, 7, 8, 9, 10, 11, 12, 13] although the majority of the cases of capillaritis are benign and spontaneously regress. Martinez et al. have recognized several important clinicopathological correlations that suggest increased evolution. Such findings include parapsoriasis, younger age at presentation (between the second and fourth decades), widespread eruptive lesions, polymorphous eosinophilic and plasmacytic infiltrate, epidermotropism, lichenoid bands in the papillary dermis, and the presence of atypical lymphocytes .
In summary, the present case study and prior reports indicate that the classic views of capillaritis as an entirely insignificant and banal clinical process should be reconsidered. Although the majority of pigmented progressive purpuric dermatosis are benign and incidental, the potential for the evolution of PPPDs into cutaneous lymphomas exists. The appearance of capillaritis should give a high index of suspicion for the diagnosis of cutaneous T-cell lymphoma, and patients should undergo close followup and biopsies if indicated. Capillaritis, therefore, may be an important potential harbinger to cutaneous T-cell lymphoma.
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