Granulomatous variant of chronic pigmented purpuric dermatosis: report of two cases

Two patients presenting with chronic pigmented purpuric dermatosis (CPPD) on the dorsum of both feet were found to show granulomatous inflammation superimposed on the pathological changes of CPPD. Two similar cases have been reported from Japan. The unique clinicopathological features of this group of patients suggest that they have a rare granulomatous variant of CPPD.     

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 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.     

Mycosis fungoides exhibiting features of a dermatofibroma: a case report and review of the literature

We present the case of a 60-year-old male with known stage IA mycosis fungoides (MF) who developed a suspicious tumor within a pre-existing lymphomatous plaque of the right forearm. Microscopy revealed an unusual mixed fibrohistiocytic proliferation as well as atypical intraepidermal and dermal lymphocytes. There was no evidence of large-cell transformation. Histopathological, immunohistochemical and clinical clues suggest this lesion might not have represented a dermatofibroma, but rather a hitherto unreported dermatofibroma-like process arising within a lesion of MF. The potential impact of this atypical lesion on the prognosis and clinical management of this patient highlights the importance of recognizing unusual clinical presentations of more common, benign lesions within this patient population.     

Manual microdissection technique in a case of subcutaneous panniculitis-like T-cell lymphoma: a case report and review

Demonstration of T-cell receptor gene monoclonality often plays an important role in the diagnosis of T-cell lymphoma. When a test to detect monoclonality is performed on whole tissue sections, the presence of a reactive lymphocyte population may reduce sensitivity. This may be especially true for early or borderline cases of lymphoma. Microdissection techniques may be utilized to more readily identify a clonal population of lymphocytes. Subcutaneous panniculitis-like T-cell lymphoma represents a cutaneous lymphoid neoplasm whose clinical course may vary from an indolent, waxing and waning course to an aggressive course resulting in death. We report the first case of a microdissection technique used to facilitate diagnosing a case of subcutaneous panniculitis-like T-cell lympoma.     

Lymphomatoid papulosis subtype C: A case report and literature review

Lymphomatoid papulosis (LyP) is a rare CD30⁺ lymphoproliferative primary skin disease with a benign clinical course and malignant histopathology. LyP is classified into seven subtypes based on histopathology: subtypes A through F and LyP with 6p25.3 chromosome rearrangement. We present here, a case report of a 51‐year‐old man, afflicted with multiple papules and nodules on his left arm for over 3 months and diagnosed with LyP subtype C. The patient refused treatment, and his lesions faded with no visible rash on the left arm 14 months after diagnosis.     

Lymphoma or pseudolymphoma: A report of six cases and review of the literature

To explore and compare the clinical presentations and pathologic features of cutaneous pseudolymphomas (CPL) with primary cutaneous lymphomas. Review literature in order to improve the treatment of CPL. Six cases of CPLs were collected. The clinical, pathologic, and immunohistochemical features were performed and analyzed in Peking Union Medical College Hospital in 2018. Of six patients, the distributions and clinical manifestations of skin lesions are varied. The pathologic features consisted of atypical prominent lymphocytes infiltration. Of them, two cases imitated mycosis fungoides, one case mimicked primary cutaneous aggressive pidermotropic CD8+ cytotoxic T‐cell lymphoma, one case was diagnosed as Jessner‐Kanof lymphocyte infiltration and two cases primary cutaneous CD4+ small/medium T‐cell lymphoproliferative disorder. With respect to etiology, one was result from insects bite and the others were on account of drugs. All cases were treated with systemic or local glucocorticoid. The skin lesions and systemic symptoms showed notable improvement after treatment. Follow‐up visits were 2 years, half a year, and months, respectively, without relapse. These unique types of CPL were similar to cutaneous lymphomas in clinical manifestation and pathology. They were all sensitive to the treatment of externally or orally using glucocorticoid. The prognosis is generally good but needs long‐term follow‐up.     

T-zone lymphoma with cutaneous involvement: a case report and review of the literature

T-zone lymphoma (TZL) is a rare subtype of nodal peripheral T-cell lymphoma characterized by a clonal expansion of T-zone lymphocytes accompanied by a proliferation of other T-zone constituents. Non-specific cutaneous alterations are seen in about one-third of all cases, but specific cutaneous involvement is extremely rare. We present a case of TZL with secondary skin infiltration, review the literature on cutaneous manifestations of TZL and discuss the differential diagnosis of TZL.     

Mycosis fungoides involving an acrochordon: a case report

We present the case of a 77‐year‐old male undergoing treatment for mycosis fungoides (MF) who presented for removal of an acrochordon on his mid back. Histopathologic examination of the acrochordon revealed a dense, band‐like lymphocytic inflammatory infiltrate in the dermis with epidermotropism of single lymphocytes and small nests of lymphocytes into the lower epidermis. Immunohistochemical staining characterized the dermal and epidermal lymphocytic population as CD3‐positive T lymphocytes with a predominance of CD4‐positive over CD8‐positive lymphocytes. These findings were consistent with the patient's known MF and molecular identification of a clonal T‐cell receptor gene rearrangement further supported the diagnosis. Our unusual case reports MF involving an acrochordon and provides evidence to support the importance of submitting acrochordons for histopathologic examination.     

New variant lymphomatoid papulosis type E preceding and coexisting with mycosis fungoides – a case report and review of the literature

Angioinvasive (type E) lymphomatoid papulosis (LyP) is a recently described subtype of LyP presenting with eschar‐like lesions that can be mistaken for aggressive forms of angiocentric cutaneous T‐cell lymphoma. None of the cases of angioinvasive LyP described thus far have been associated with mycosis fungoides (MF). Herein, we describe a case of angioinvasive LyP type E coexisting with MF. The patient presented with an eschar on his chest and over time developed new nodules and large plaques with eschar formation, all of which resolved spontaneously over a period of a few weeks without intentional therapy. Biopsy revealed a CD30+ atypical inflammatory cell infiltrate with marked angiocentricity. Later, he developed erythematous annular scaly patches histologically consistent with MF. Our patient's clinical course confirms the indolent behavior characteristic of LyP despite the aggressive clinical and histologic appearance of lesions. The co‐occurrence of angioinvasive LyP and MF in our patient highlights the propensity for LyP type E to coexist with MF, as is characteristic of other LyP subtypes, and supports the theory that LyP and MF are related T‐cell lymphoproliferative disorders. Patients with LyP can present with large lesions exhibiting eschar formation and an atypical angiocentric/angiodestructive lymphoid infiltrate and should be spared overtreatment.     

桃红四物汤治疗色素性紫癜性皮肤病临床及实验室观察

目的 观察桃红四物汤治疗色素性紫癜性皮肤病的临床疗效及血液流变学变化。方法 将61例随机分为两组，治疗组31例用桃红四物汤加味，对照组30例用西药治疗。结果 疗效统计：治疗组有效率70.97％，对照组43．33％，差异有显著性（P〈0.05）。血液流变学检测：治疗组治疗前后各项指标差异有非常显著性（P〈0．01），对照组治疗前后各项指标差异无显著性（P〉0.05），两组治疗后差异有非常显著性（P〈0．01）。毛细血管扩张性环状紫癜疗效较好，差异有显著性（P〈0.05）。结论 桃红四物汤加味治疗色素性紫癜性皮肤病有较好的临床效果和改善微循环的作用。     

Lymphomatoid granulomatosis presenting with cutaneous involvement: a case report and review of the literature

Lymphomatoid granulomatosis (LG) is a rare Epstein–Barr virus (EBV)‐associated B‐cell lymphoproliferative disorder presenting in middle adulthood that nearly always affects the lungs and shows cutaneous involvement in up to 50% of cases. Skin lesions are present at the time of diagnosis in roughly one‐third of patients and may precede the development of lung lesions in as many as 10–15%. Recognition by both the dermatologist and dermatopathologist is therefore crucial for early and accurate diagnosis. While skin involvement is grossly and microscopically diverse, the disease most commonly presents as erythematous subcutaneous and dermal nodules showing the classic histopathologic triad of transmural lymphocytic angiitis, atypical B‐lymphocytes in a polymorphous T‐cell background, and necrotic foci within lymphoid aggregates. We present a case of lymphomatoid granulomatosis initially presenting with cutaneous lesions, with an accompanying review of the literature.     

Primary subcutaneous follicular centre cell lymphoma with involvement of the galea: a case report and short review of the literature

Primary cutaneous follicular centre cell lymphoma (FCCL) is a distinct subtype of cutaneous lymphoma that originates from germinal centre cells. Histologically, the disease is typified by a bottom-heavy infiltrate with a diffuse or follicular growth pattern situated in the mid or deep dermis. In some cases, the neoplastic infiltrate may involve the underlying subcutaneous tissue, but so far primary subcutaneous FCCL has not been reported. We report the first case of primary FCCL located primarily in the deep subcutis with extension into the galea and review the literature on primary subcutaneous B-cell lymphomas.     

Two cases of eczematid‐like purpura of Doucas and Kapetanakis responsive to narrow band ultraviolet B treatment

Eczematid‐like purpura of Doucas and Kapetanakis is a type of pigmented purpuric dermatoses (PPDs) with eczematous changes in the purpuric surface. A 10‐year‐old male and a 44‐year‐old male patients were admitted to our clinics for itching and flaking of the skin rashes. Based on the clinical and histopathological evaluations, the rashes were identified as eczematid‐like PPDs of Doucas and Kapetanakis. Both patients were treated with narrow band ultraviolet B. The lesions were remarkably regressed following the treatment. These cases reported due its rarity and good response to narrow band ultraviolet B.     

Papular mycosis fungoides: report of two patients,literature review,and conceptual re‐appraisal

Recent reports of 10 patients have proposed a papular variant of mycosis fungoides (MF), characterized by the appearance of papules in the absence of patches and the presence of histopathologic features of classic patch/plaque stage MF. Given the overlapping clinical and pathologic features between this proposed entity and lymphomatoid papulosis (LyP)‐type B, however, the optimal classification for such cases remains somewhat unclear. Herein, two patients are described who presented to the dermatology clinic with persistent erythematous papular eruptions on the trunk, upper and lower extremities and histopathology compatible with MF. Of note, these patients represent the oldest reported cases of papular MF, and one patient had documented peripheral blood involvement by atypical CD4+ cells. The clinicopathologic characteristics of this purported entity suggest that it may occupy the intersection between MF and CD30+ lymphoproliferative disorders. These two cases expand the clinicopathologic spectrum of papular MF to include older individuals, and further emphasize the importance of recognition of this morphology as a possible MF manifestation. Furthermore, consideration of our cases in conjunction with the previously documented 10 other patients in the literature, offers potential insight into the relationship between MF and CD30+ lymphoproliferative disorders.     

Venoprotective drugs in pigmented purpuric dermatoses: A case report

Pigmented purpuric dermatoses (PPD) or capillaritis represent a benign condition, presenting with extravasation of erythrocytes in the skin and prominent hemosiderin deposition. The eruption runs a chronic relapsing course and is resistant to therapy. Capillary fragility, cellular immunity, and microvascular inflammation play important roles in the pathogenesis of PPD. Bioflavonoids, currently used as venoprotective agents for the management of chronic venous insufficiency, have been shown to reduce capillary fragility and permeability by inhibiting endothelial cell activation and modulating the leukocyte‐endothelium interaction. We report a case of PPD with dramatic improvement consequent to a therapy with a fixed dose combination containing flavonoids like diosmin, hesperidin, and Euphorbia prostata extract along with calcium dobesilate.     

Extracavitary primary effusion lymphoma presenting as a cutaneous tumor: a case report and literature review

Primary effusion lymphoma is an unusual form of aggressive B‐cell lymphoma universally associated with human herpesvirus 8 (HHV8) that involves mostly human immunodeficiency virus (HIV)‐infected patients. Characteristically, it presents as a malignant serous effusion involving body cavities, but without associated tumor mass. Exceptionally, HHV8‐positive lymphomas with features identical to primary effusion lymphoma may present as mass lesions in the absence of cavity effusions along the course of the disease, and are regarded as extracavitary or solid variants of the disorder. These rare forms are extremely rare in the skin. We report a case of extracavitary primary effusion lymphoma arising in a HIV‐infected male, who presented with two subcutaneous masses involving the skin of the abdominal and inguinal regions as the first manifestation of the process. Kaposi sarcoma was not present in the skin surface or mucous membranes. Extensive studies failed to demonstrate involvement of other organs and the case was considered as an example of extracavitary primary effusion lymphoma originating primarily in the skin. Herein, we review the few reported cases of solid primary effusion lymphoma involving the skin in order to delineate the clinicopathologic, immunohistochemical and molecular characteristics of this rare lymphoma in the skin.     

暂时性棘层松解性皮病1例并文献复习

报道1例暂时性棘层松解性皮病,并对国内外相关文献进行复习回顾。本例患者以丘疹、丘疱疹、水疱为主要临床表现,病理见局限性基底细胞层上裂隙,少量棘层松解细胞,表皮内水疱。     

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.     

Cutaneous involvement by colonic extranodal NK/T-cell lymphoma mimicking mycosis fungoides: a case report*

We report a 51-year-old woman with cutaneous involvement by extranodal NK/T-cell lymphoma (TCL) of the colon that microscopically mimicked mycosis fungoides (MF). She had a history of fever of unknown origin for 2 months and then developed multiple erythematous papules on her trunk and extremities. A skin biopsy revealed superficial infiltration by atypical small to medium-sized lymphocytes with epidermotropism and Pautrier collections. Immunohistochemical studies showed expression of CD3 and TIA-1 with lack of expression (double negative) of CD4 and CD8. Initially, we reported the diagnosis as MF, cytotoxic variant. Thereafter, computerized tomography scan incidentally identified a colonic mass. A colonic biopsy revealed infiltration of atypical lymphoid cells with the same morphology and immunophenotype as those found in the skin. Additionally, CD56 and Epstein-Barr virus-encoded RNA in situ hybridization in both skin and colonic biopsies were diffusely positive. Thus, extranodal NK/TCL was diagnosed. Delta T-cell receptor (TCR) gene rearrangement was documented in the skin biopsy by polyacrylamide gel electrophoresis and fluorescence capillary gel electrophoresis methods. There was no TCR gene rearrangement detected in the colonic biopsy. Unfortunately, the patient died within 2 months of diagnosis.