Cutaneous lymphoma manifesting as pigmented, purpuric capillaries]

INTRODUCTION: A few cases of patients with both purpuric pigmented dermatitis and cutaneous lymphoma have been reported. The aim of this study was to evaluate the prognosis of purpuric pigmented dermatitis. MATERIAL AND METHODS: This is a monocentric retrospective study at the dermatology department of the university hospital of Strasbourg. The records of all patients hospitalized for purpuric pigmented dermatitis between 1967 and 1997 have been reviewed. RESULTS: Eight women and 9 men aged between 17 and 84 years were hospitalized for purpuric pigmented dermatitis during the reference period. Except for one patient, all had had a cutaneous biopsy showing the typical features of purpuric pigmented dermatitis. On the basis of clinical signs and course, one patient was thought to have contact dermatitis and three patients were thought to have a purpuric pigmented dermatitis-like drug eruption (meprobannate, pefloxacine and lorazepam or aspirin). The mean follow-up was one year. During follow-up, two patients developed cutaneous T-cell lymphoma after two and four years respectively and one patient developed Hodgkin's disease with skin and lymph node involvement. Another patient who suffered from purpuric pigmented dermatitis for four years had a monoclonal T cell population within the dermal infiltrate. Two patients died of their lymphoma. DISCUSSION: This study shows that purpuric pigmented dermatitis can be associated to or evolve into lymphoproliferative disease. This course should be suspected when purpuric pigmented dermatitis is extensive, long-lasting (> 1 year), has a reticular arrangement and negative patch-testing. In this situation, a long-term follow-up and treatments indicated in the early patch stage of mycosis fungoides (PUVA therapy, chlormethine) seem adequate.     

Purpura annularis telangiectodes of Majocchi: case report and review of the literature

Background  Purpura annularis telangiectodes of Majocchi is an uncommon form of pigmented purpuric dermatosis which may present a therapeutic challenge. Given the rare nature of this condition, there is limited anecdotal information available regarding optimal therapy. Although pigmented purpuric dermatoses are generally innocuous, in some cases they may cause patients significant distress, and there is a need to exclude cutaneous T-cell lymphoma.
Methods  We reviewed the literature on the treatment of pigmented purpuric dermatoses and managed a 69-year-old woman who presented with purpuric annular patches on the legs.
Results  Three separate biopsies demonstrated an interstitial to perivascular lymphocytic infiltrate with erythrocyte extravasation, consistent with pigmented purpuric dermatosis. The patient's condition proved refractory to many of the previously reported modes of management, but markedly improved with methotrexate. Treatment alternatives for pigmented purpuric dermatosis are reviewed, and a treatment algorithm is proposed.
Conclusion  This is the first reported case regarding the successful use of methotrexate for pigmented purpuric dermatosis. Methotrexate may offer a therapeutic alternative to patients with highly symptomatic pigmented purpuric dermatosis refractory to other, more conservative, treatment modalities.     

Granulomatous pigmented purpuric dermatosis: report of a Latin-American case with blaschkoid distribution

Granulomatous pigmented purpuric dermatosis clinically manifests as hyperpigmented maculae and petechiae, predominantly on the lower extremities. Histopathologically, it is characterized by a lymphocytic infiltrate in the upper dermis, extravasated erythrocytes, and hemosiderin deposits. There is an infrequent variant called granulomatous pigmented purpuric dermatosis, which histologically is characterized by the presence of non-necrotizing granulomas associated with the classic findings of other pigmented purpuric dermatoses. It more frequently affects middle-aged women of Asian origin, and predominantly on the lower extremities. The authors present the case of a female patient with granulomatous pigmented purpuric dermatosis on the lower extremities with blaschkoid distribution.     

Purpura annularis telangiectodes of Majocchi

A case is presented of a man with a 3-year history of ulcers in the setting of pigmented, annular and purpuric lesions of the lower extremities. A skin biopsy suggested a diagnosis of purpura annularis telangiectodes of Majocchi. First described in 1896 by Majocchi [1], purpura annularis telangiectodes is an uncommon pigmented purpuric eruption, which is characterized by symmetrical, purpuric, telangiectatic, and atrophic patches with a predilection for the lower extremities and buttocks. Histopathology and immunopathogenesis of this disease are similar to the other subtypes of pigmented purpuric dermatoses.     

Unilateral linear capillaritis

We present four cases of a distinctive type of pigmented purpuric eruption occurring in a striking linear and pseudo-dermatomal distribution in young males. Whilst these cases share some of the clinical and histological features of other pigmented purpuric dermatoses, they are not readily classified with any of the entities so far defined in this group of disorders. We believe these cases represent a distinct group not previously described. Four patients with an unusual pigmented purpuric eruption are presented.     

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.     

Monoclonal rearrangement of the T cell receptor gamma-chain in lichenoid pigmented purpuric dermatitis of gougerot-blum responding to topical corticosteroid therapy

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 gamma-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.     

A report of two cases of pigmented purpuric dermatoses treated with PUVA therapy.

This is a report of 2 patients with pigmented purpuric dermatosis treated successfully with Psoralen photochemotherapy (PUVA). Treatment for pigmented purpuric dermatosis is generally unsatisfactory, and we believe this to be the first report of successful response of this condition to PUVA.     

Narrowband UVB treatment of pigmented purpuric lichenoid dermatitis (Gougerot–Blum)

Pigmented purpuric dermatoses are a group of dermatoses characterized clinically by pinpoint petechia and purpura on a brown, red or yellow base. Five diseases are included in the group of pigmented purpuric eruptions which significantly overlap clinically and histologically. Phototherapy in various forms of purpuric dermatoses has been reported in literature previously. Here we present a case of pigmented purpuric lichenoid dermatitis which rapidly cleared with narrowband UVB therapy.     

Granulomatous pigmented purpuric dermatoses: report of three cases and review of the literature

Purpuric pigmented dermatoses represent a form of chronic, recurrent capillaritis characterized by petechiae and purpuric macules over the lower limbs. We report three female cases who presented with clinical features of purpuric pigmented dermatosis with unusual histopathologic features. The first patient had golden-brown pigmented purpura over bilateral knees, shins and dorsal feet. The second patient showed purpuric papules on the medial aspects of the ankles. The third patient presented with numerous petechiae on her lower legs. All three patients demonstrated granulomatous inflammation in addition to the histologic features of pigmented purpuric dermatosis. A total of 11 patients have currently been reported in the literature. A review of the clinical details of the cases revealed a high incidence of hyperlipidemia (7/11) and hypertension (4/11) among these patients.     

Idiopathic pigmented purpuric eruption with palmar-plantar involvement

A case of generalized idiopathic pigmented purpuric eruption with distinctive and rare palmar-plantar involvement is presented. The clinical and histopathologic features, differential diagnosis, and nosology of the idiopathic pigmented purpuric eruptions are presented.     

A case of unilateral linear capillaritis

Unilateral linear capillaritis is a rare variant of the pigmented purpuric dermatoses that can be misdiagnosed due to confusion with other cutaneous diseases that follow a linear distribution. We present the case of an 8-year-old boy with hyperpigmented patches linearly distributed on the right arm, initially diagnosed with segmental neurofibromatosis.     

Drug-induced chronic pigmented purpura

A close correlation between purpuric reaction and drugs was observed in seven cases of chronic pigmented purpura. The patients developed purpuric lesions after taking certain drugs for more than 3 years, were thiamine propyldisulfide in 2 cases, and chlordiazepoxide in 1 case. The purpuric lesions stopped recurring after removal of the drugs in the rest of the cases. It is suggested that drugs are among the etiological factors in chronic pigmented purpura.     

Successful treatment of Schamberg’s disease with fractional non-ablative 1540 nm erbium:glass laser

Schamberg’s disease is one of the pigmented purpuric dermatoses (PPD). PPD encompass a large and heterogeneous group of dermatologic disorders featuring purpuric lesions often located on the lower limbs. The various forms of PPD are notoriously known to be resistant to treatment. Fractional photothermolysis has been described as a successful and safe method to induce dermal remodeling. We report three patients with Schamberg’s disease who were successfully treated with 4 monthly sessions of fractional non-ablative 1540 nm erbium:glass laser, with resolution of their purpuric pigmented rash lasting up to 9 months after the last treatment session.     

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。     

Granulomatous Pigmented Purpuric Dermatosis

We report two cases of brown pigmentation accompanied by hemorrhagic papules. A 61-year-old woman and a 53-year-old woman presented with brown pigmentation accompanied with hemorrhagic papules occurring bilaterally from the dorsal aspect of the feet to the lower legs. The clinical picture was of pigmented purpuric dermatosis, but histopathologically, they were hemorrhagic granulomatous inflammation of the upper dermis. As no systemic granulomatous lesions were observed, these cases were considered to be granulomatous pigmented purpuric dermatosis.     

Linear morphea overlying site of previous lichen aureus in a pediatric patient

Lichen aureus is a pigmented purpuric dermatosis that tends to occur on the lower extremities, and linear morphea is an inflammatory disease of the dermis and subcutaneous fat that leads to a scarlike induration. We present a pediatric case of linear morphea developing in the same anatomic distribution as prior lichen aureus. Due to the possible progression of lichen aureus to morphea described herein and the difficulty in diagnosing early morphea, close follow‐up may be considered for pediatric patients who present with this pigmented purpuric dermatosis.     

Kontaktallergie durch Bufexamac unter dem Bild einer chronischen Pigmentpurpura

We report on a case of a bufexamac-induced allergic contact dermatitis with hematogenous dissemination presenting with the clinical and histological picture of a pigmented purpuric eruption. To our knowledge this is the first report on a bufexamac-induced pigmented purpuric dermatosis. It represents a further example of the clinical variety of cutaneous side-effects caused by bufexamac.     

进行性色素性紫癜性皮病27例临床分析

目的探讨进行性色素性紫癜性皮病的发病原因、临床特点和治疗方案,提高对本病的认识。方法回顾性分析本科1995年1月-2010年10月收治的27例进行性色素性紫癜性皮病患者的临床和实验室检查资料。结果 27例患者中男女比例为1.7:1,15例(55.56%)合并有基础疾病。18例(66.67%)皮损位于双下肢,6例患者(22.22%)累及四肢,3例患者(11.11%)累及四肢、臀部和躯干,糖皮质激素、雷公藤多甙、沙利度胺、氨苯砜等不同药物联合治疗后21例患者皮损消退,6例患者皮损大部分消退。其中19例患者出现反复复发。结论进行性色素性紫癜性皮病病程慢性、易复发,目前尚无特效治疗;糖皮质激素、雷公藤多甙、沙利度胺、氨苯砜等治疗均有效,且以糖皮质激素效果明显,但不能预防复发,光疗可作为一有效而安全的联合或单独治疗选择。     

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.