持续性色素性紫癜性皮病样蕈样肉芽肿一例并文献复习

持续性色素性紫癜性皮病样蕈样肉芽肿是皮肤T细胞淋巴瘤中的罕见类型,具有特征性的临床表现、皮肤镜、病理及免疫组化特点。本文报道1例持续性色素性紫癜性皮病样蕈样肉芽肿病例并复习相关文献。     

色素性紫癜性皮病样蕈样肉芽肿一例

患者,女,17岁。躯干、双下肢黄褐色斑片7年。组织病理示：（腰背部皮损）表皮角化过度,灶性角化不全,棘层轻度增厚,单一核细胞移入,部分细胞核色深、不规则。免疫组化：CD2（＋）、CD3（＋）、CD4（＋）、CD8（＋）、CD5（＋）、Ki-67阳性细胞率约20%。诊断：蕈样肉芽肿（红斑期）。     

表现为进行性色素性紫癜性皮病的蕈样肉芽肿1例

患者,女,76岁。双下肢出现红色瘀点、进行性增多伴轻微瘙痒半年。组织病理示:表皮及真皮浅层单一核细胞浸润,表皮内可见Pautrier微脓肿。免疫组化:CD3(+)、CD4(+)、CD8(散在+)、CD45R0(+)、Ki-67(20%左右+)。诊断:蕈样肉芽肿。     

Mycosis fungoides presenting as pigmented purpuric eruption

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.     

Mycosis fungoides presenting as pigmented purpuric dermatitis

Mycosis fungoides, a cutaneous T-cell lymphoma, typically presents as indolent, progressive, and persistent erythematous patches or plaques with mild scaling and over time can evolve into tumor stage with tumor nodules. Other presentations include eczematous, psoriasiform, poikilodermatous, and hypopigmented patches. We report Mycosis fungoides in a 14-year-old boy presenting as pigmented purpuric dermatitis and review the relevant literature. This is a rare presentation of a condition that is uncommon in the pediatric population. In our patient, histologic features were typical of Mycosis fungoides presenting as pigmented purpuric dermatitis. The clinical features, pathology, molecular biology, and the relationship between these two entities are discussed.     

Granulomatous pigmented purpuric dermatosis

The granulomatous variant of the pigmented purpuric dermatoses (PPDs) is a rare and infrequently described condition, with a total of 16 cases published to date. We report a case of granulomatous PPD in a 59‐year‐old white woman who demonstrated involvement of the arms, legs, chest and back with concurrent hyperlipidaemia. Histopathological examination revealed a lymphohistiocytic infiltrate obscuring the dermoepidermal junction, and loose granuloma formation in the superficial dermis, with extravasated erythrocytes. Other conditions within the differential diagnosis such as atypical infection, papular sarcoidosis and generalized granuloma annulare were excluded on clinical and histological grounds. Our patient represents the ninth patient reported to have granulomatous PPD with coexisting hyperlipidaemia, and the fifth patient with granulomatous PPD and a lichenoid infiltrate.     

以色素性紫癜性皮病为表现的蕈样肉芽肿一例

患者,男,70岁。左下肢皮疹10余年,泛发全身伴痒3个月。皮肤科检查：四肢为主的广泛对称的紫癜性暗红色斑片、斑块,躯干散在的暗红色斑疹表面可见明显鳞屑。皮肤组织病理示符合蕈样肉芽肿诊断。患者给予重组人干扰素α2b,肌注,300MIU,1周3次,甲氨蝶呤10 mg,1周1次,同时配合窄谱中波紫外线（NB-UVB）1周3次治疗。经1年余治疗后患者明显好转。     

Persistent pigmented purpuric eruption associated with mycosis fungoides: a case report and review of the literature

A purpuric eruption may be an unusual early manifestation of mycosis fungoides (MF). On the other hand, persistent pigmented purpuric dermatoses (PPPD) may, occasionally, evolve to cutaneous T-cell lymphoma. Coexistence of these two conditions has been reported, but it is extremely rare. We present the case of an elderly woman with a long-standing pruritic, pigmented purpuric eruption. On 1-year follow-up, histological features suggesting early MF were observed and molecular analysis of the rearrangement of T-cell receptor genes revealed clonality. Our patient may represent a case of PPPD evolving to MF, a case of MF clinically featuring PPPD, or an intermediate condition in a nosological continuity extending from PPPD to MF. A persistent pigmented purpuric eruption may rarely be a harbinger of cutaneous T-cell lymphoma. Therefore, vigilant long-term follow-up of PPPD is highly recommended.     

Granulomatous mycosis fungoides presenting as poikiloderma

Granulomatous mycosis fungoides (MF) is a rare subtype of MF, characterized by the histological presence of a granulomatous reaction, but distinct clinical characteristics are not present. A 41-year-old healthy man presented with poikiloderma, ichthyosis and erythematous scaly plaque. Histological examination of a biopsy taken from poikilodermic skin showed a granulomatous reaction to epidermotropic atypical lymphocytes. However, in other areas there were only findings of conventional MF without granuloma. Granulomatous MF may be associated with poikiloderma.     

Two cases of unilateral pigmented purpuric dermatosis

Two cases of pigmented purpuric dermatosis showing unusual distribution are reported. Case 1: 35-year-old female. A group of three asymptomatic purpuric macules developed on the ulnar side of the elbow flexure of the left forear 3-6 months before presentation. Histopathological analysis revealed a band-like inflammatory infiltrate in the Uppermost dermis and extravasation of red blood cells just beneath the epidermis, but there was no evidence of lichefaction degeneration in the basal layer of the epidermis. Case 2: 26-year-old female. Asymptomatic purpuric pigmentations developed on the flexure side of the right lower extremity in a linear arrangement 2-3 months before presentation. Histopathological analysis revealed focally perivascular inflammatatory infiltrates consisting mainly of lymphocytes and conspicuous extravasation of red blood cells into the papillary dermis. There was no histological evidence of vasculitis in either case. Hemosiderin deposits were more limited than expected in both patients. Both cases are compatible with diagnoses of pigmented purpuric dermatosis. Case 1 on the left arm was compatible with lichen purpuricus. Case 2 occurred in a linear arrangement on the right leg and had a history of aspirin use for chronic headache. We therefore suspected that medication had been an etiological or contributing factor.     

Atypical clinical presentation of pigmented purpuric dermatosis

The term pigmented purpuric dermatosis (PPD) is used for a group of mainly asymptomatic, sometimes pruritic dermatoses that are clinically characterized by an eruption of pinpoint purpuric lesions along with yellow, orange, red and/or brown, often patchy pigmented areas. Traditionally five subtypes have been distinguished, but atypical clinical patterns may also occur. Because of the variable clinical spectrum and the similar histopathologic findings, a strict nosological classification is sometimes difficult or even impossible to achieve. We report a case of PPD with atypical clinical features in a young woman, underlining the difficulties in the clinical classification of this spectrum of diseases.     

Diet‐induced pigmented purpuric dermatosis

Pigmented purpuric dermatoses (PPD) are chronic and relapsing disorders characterised by a localised or generalised purpuric rash. Even though the clinical presentation of PPD subtypes varies, they have a similar histopathology. The aetiology is largely unknown, but trigger factors, such as drugs, infections and systemic illnesses have been described. To our knowledge, this is the only case showing widespread PPD lesions not only induced but also rapidly provoked by dietary factors, namely Coca Cola and apple‐cherry fruit spritzer.     

Localized morphoea preceded by a pigmented purpuric dermatosis

Localized morphoea uncommonly occurs in a linear distribution and may present following trauma, although most cases are idiopathic. Pigmented purpuric dermatoses such as lichen aureus may also rarely occur in a linear distribution and have been associated with trauma. A middle-aged man is described who initially presented with lesions typical of lichen aureus in a linear distribution at a site exposed to chronic low-grade trauma. This eruption was transient and clinically underwent spontaneous complete resolution. Several months later he developed localized morphoea in an identical distribution. To our knowledge this is the first reported case of a pigmented purpuric dermatosis, presumably precipitated by trauma, evolving into linear morphoea.     

Granulomatous mycosis fungoides presenting as an acquired ichthyosis

We report a case of a 69-year-old gentleman who presented with a 3-month history of unexplained fevers and malaise who developed generalized pruritus, alopecia and an ichthyosiform erythematous eruption on his forearms, legs, chest and back. Skin histology, immunophenotyping and molecular features were consistent with granulomatous mycosis fungoides. He has been successfully treated with twice weekly PUVA photochemotherapy.