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Mycosis fungoides presenting as pigmented purpuric eruption

European Journal of Dermatology. Volume 15, Number 6, 489-91, November-December 2005, Clinical report

Summary

Author(s) : Tsukasa Ugajin, Takahiro Satoh, Hiroo Yokozeki, Kiyoshi Nishioka , Department of Dermatology, Saiseikai Kawaguchi General Hospital 5-11-5 Nishikawaguchi, Kawaguchi-shi, Saitama-pref, 332-8558, Japan, Immunodermatology, Graduate School, Tokyo Medical and Dental University, Tokyo, Japan.

Summary : A case of pigmented purpuric eruptions evolving to mycosis fungoides during the 4-year follow-up period is described. Clinical manifestation was characterized by petechial lesions with irregular shaped, diffusely pigmented plaques partly sharing morphological similarities with chronic pigmented purpura. Histologically, lymphocytes infiltrated around the capillaries of the superficial dermis with extravasated erythrocytes as well as into the epidermis to form Pautrier microabscesses. Whereas CD4+ cells were observed in the epidermis and upper dermis, CD8+ cells tended to be distributed around the capillaries. Notably, the Rumpel-Leede test revealed extensive punctuate purpura limited to the lesional skin. The aggregation response of platelets was not impaired. Either CD4+ tumor lymphocytes or CD8+ reactive lymphocytes appeared to induce capillary damage resulting in the formation of petechial lesions. Pigmented purpuric eruptions, such as atypical chronic pigmented purpura, is thus an important initial clinical manifestation of mycosis fungoides.

Keywords : capillaritis CLA, mycosis fungoides, pigmented purpura, Rumpel-Leede test

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ARTICLE

Auteur(s) : Tsukasa Ugajin, Takahiro Satoh, Hiroo Yokozeki, Kiyoshi Nishioka

Department of Dermatology, Saiseikai Kawaguchi General Hospital 5-11-5 Nishikawaguchi, Kawaguchi-shi, Saitama-pref, 332-8558, Japan
Immunodermatology, Graduate School, Tokyo Medical and Dental University, Tokyo, Japan

accepté le 8 Decembre 2004

The early stages of mycosis fungoides (MF) are characterized by a variety of skin features including erythematous, eczematous, poikilodermatous, or verrucous plaques. Here, we report a patient with purpuric lesions accompanied by extensive pigmentation as an initial clinical manifestation of mycosis fungoides. The pathomechanisms of pigmented purpuric eruptions and the relationship between MF and chronic pigmented purpura are also discussed.

Case report

A 71-year-old female presented with a 1-year history of rashes on her lower extremities. Physical examination revealed irregularly shaped brown macules with miliary-sized purpuric spots scattered on her lower extremities ( (figure 1A) ). She also reported moderate itching. She had hypertension, which was being controlled with a calcium channel blocker. Histologically, a weak cellular infiltrate, consisting of lymphocytes and some extravasated erythrocytes, was observed around the capillaries of the papillary dermis ( (figure 1B) ). Berlin-blue staining revealed extensive deposition of hemosiderin in the upper dermis. Differential diagnoses included purpuric drug eruption, chronic pigmented purpura, stasis dermatitis, and mycosis fungoides. Appearance of purpuric spots did not cease despite changing hypertension medications. Southern blot analysis of the extracted DNA from the skin lesions did not reveal evidence of clonal rearrangement of the T cell receptor C beta 1 gene and the lesions were resistant to a regimen of topical corticosteroids.

During the 4-year follow-up period, the rash became extensive. Various-sized, diffuse, dark-brown pigmented macules coalesced and were observed not only on the lower extremities ( (figures 2A,B) ), but also on the forearms and lower abdomen. There were dark-red punctiform petechiae within and around the macules. Platelet count, prothrombin time, and partial thromboplastin time were within normal limits. Platelet aggregation in response to collagen and ADP was not impaired. Interestingly, however, the Rumpel-Leede test revealed extensive punctuate purpura on the lesional skin of the forearms and legs. A new biopsy was performed from the plaque. Histological examination revealed lymphocytes with hyperchromatic small-medium sized nuclei around the capillaries of the upper dermis with extravasation of erythrocytes. The cells infiltrated into the epidermis to form Pautrier microabscesses ( (figure 2C) ). The cellular infiltrate largely consisted of CD4+ cells, as determined by immunohistochemical staining ( (figure 3A) ). CD8+ cells were preferentially distributed around the capillaries ( (figure 3B) ). The majority of infiltrative cells were positive for CLA (cutaneous lymphocyte associated antigen) ( (figure 3C) ). VLA-4 was expressed on infiltrate around the capillaries (data not shown). Endothelial cells expressed E-selectin ( (figure 3D) ), VCAM-1 and ICAM-1 (data not shown). Rearrangement of the T cell receptor C beta 1 gene was detected by Southern blot analysis. Computed tomography and scintigraphy showed no internal organ involvement. Patient condition is currently being controlled with PUVA therapy without progression.

Discussion

The present case showed persistent petechial lesions with extensive dark-brown pigmentation. It was difficult to make an initial diagnosis of MF due to the absence of atypical lymphocytes and epidermotropism. However, during the 4-year follow-up period, Pautrier microabscesses became apparent and Southern blot analysis of the T cell receptor C beta 1 gene indicated clonal expansion of infiltrating lymphocytes.

Pigmented purpuric lesions are uncommon, but may be a characteristic and important feature of MF [1, 2]. A close relationship between MF and chronic pigmented purpura has recently been argued [3-5]. Barnhill and Braverman reported three patients with pigmented purpuric eruptions evolving to MF during follow-up periods averaging 8.4 years [3]. Lipsker et al. [4] analyzed 17 patients who were diagnosed clinically and histologically as having chronic pigmented purpura and of these, 2 developed cutaneous T cell lymphoma within a few years. Moreover, the first case reported in the American literature as having lichen aureus later developed into MF [6]. Analysis of a large number of cases with persistent pigmented purpuric dermatitis demonstrated that a lichenoid variant of pigmented purpuric dermatitis is probably related to MF [7].

Chronic pigmented purpura comprises a group of vascular disorders and is histologically characterized by lymphocytic capillaritis. There are five major clinical subtypes: progressive pigmented purpuric dermatosis of Schamberg; purpura annularis telangiectodes of Majocchi; pigmented purpuric lichenoid dermatosis of Gougerot and Blum; eczematoid-like purpura; and lichen aureus. Although the precise etiology remains unknown, several lines of evidence have suggested involvement of humoral or cellular immunity, hemostasis, certain drugs, and/or focal infections in the pathogenesis of this disease [8-13]. Our case was similar to the disease spectrum of chronic pigmented purpura, but somewhat different from the typical clinical subtypes of the disease in that each lesion was a coalesced plaque of homogeneous dark-brown color, varying in size, and irregular but not oval or annular in shape. Histological changes did not resemble the lichenoid variant.

The mechanisms underlying the formation of purpuric lesions in MF remain unknown. It should be noted that the Rumpel-Leede test induced petechial lesions within the pigmented plaques, suggesting that the appearance of purpura was due to increased capillary fragility. CD4+ tumor lymphocytes may have induced capillary damage during transmigration. Alternatively, reactive CD8+ cells distributed around vessels may have attacked endothelial cells resulting in the capillaritis in the papillary dermis.

Collectively, it is likely that MF could manifest as an atypical clinical subtype of chronic pigmented purpura in its early stages. Careful examination and long-term follow-up is crucial for management of the disease. However, further study is required to determine whether chronic pigmented purpura with typical clinical manifestations is a premycotic stage of MF.

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