Panniculitis induced by MINE chemotherapy]

BACKGROUND: Drug-induced panniculitis are uncommon. We report the second case of panniculitis induced by MINE chemotherapy. CASE REPORT: A 31-year-old woman with relapsed Hodgkin disease was treated with MINE cytostatic regimen. Multiple erythematous and painful nodules of panniculitis developed on her chest, abdomen and thighs fifteen days after the beginning of drug administration with a second flare up after second administration of the same drugs. The eruption cleared slowly after treatment withdrawal. DISCUSSION: To our knowledge, our case is the second reported case of panniculitis induced by MINE chemotherapy. Drug-induced panniculitis is uncommon and usually induced by steroid treatment. Some cases of panniculitis induced by atenolol, potassium bromide, apomorphine, interferon alpha and interleukin 2 have been described. Few cutaneous adverse effects are reported with MINE chemotherapy: rash, erythema and swelling of extremities. A case of inflammatory swelling of thighs with hemorrhagic panniculitis due to this treatment has been described recently.     

Systemic toxicity following administration of sirolimus (formerly rapamycin) for psoriasis: association of capillary leak syndrome with apoptosis of lesional lymphocytes.

BACKGROUND: Sirolimus (formerly rapamycin) is an immunosuppressive agent that interferes with T-cell activation. After 2 individuals with psoriasis developed a capillary leak syndrome following treatment with oral sirolimus lesional skin cells and activated peripheral blood cells were analyzed for induction of apoptosis. OBSERVATIONS: A keratome skin specimen from 1 patient with sirolimus-induced capillary leak syndrome had a 2.3-fold increase in percentage of apoptotic cells (to 48%) compared with an unaffected sirolimus-treated patient with psoriasis (21%). Activated peripheral blood T cells from patients with psoriasis tended to exhibit greater spontaneous or dexamethasone-induced apoptosis than did normal T cells, particularly in the presence of sirolimus. CONCLUSIONS: Severe adverse effects of sirolimus include fever, anemia, and capillary leak syndrome. These symptoms may be the result of drug-induced apoptosis of lesional leukocytes, especially activated T lymphocytes, and possibly release of inflammatory mediators. Because patients with severe psoriasis may develop capillary leak from various systemic therapies, clinical monitoring is advisable for patients with inflammatory diseases who are treated with immune modulators.     

Angiotropic B-cell lymphoma with telangiectasia, accompanied by panniculitic formation

A 67-year-old female presented with diffuse edema, generalized telangiectasia, and indurated skin plaques. Histopathological findings of the skin lesion included CD45+/CD79+/CD20+ large lymphoma cells that filled the vascular lumina and infiltrated the subcutaneous tissues with panniculitic formations. Three cycles of a CHOP (cyclophosphamide, adriamycin, vincristine, and prednisolone) chemotherapy regimen resolved the skin manifestations. After five cycles of CHOP, however, she developed dementia-like symptoms. Three cycles of additional intrathecal chemotherapy (methotrexate, cytarabine and prednisolone) did not improve the neurological symptoms.     

Eosinophilia-myalgia syndrome and L-tryptophan intake]

We present the case of a 52-year-old woman who developed diffuse induration of the skin and severe edema of the subcutaneous tissue involving the extremities and the trunk, sparing hands, feet and face after 10 years of almost constant oral tryptophan medication. The skin manifestations were similar to those of eosinophilic fasciitis (Shulman syndrome). The patient complained of severe muscle pain and weakness. Laboratory studies revealed an elevated Westergren erythrocyte sedimentation rate and eosinophilia. There were no signs of internal organ involvement and no immunological parameters of progressive systemic scleroderma. Eosinophilia and myalgia resolved in response to intermittent systemic therapy with glucocorticosteroids, whereas the progressive scleroderma and the edema showed only slight improvement after the discontinuation of L-tryptophan.     

Cutaneous aseptic abscesses, manifestations of neutrophilic diseases]

BACKGROUND: Neutrophilic skin disease includes several entities: Sweet syndrome, pyoderma gangrenosum, erythema elevatum diutium, Sneddon-Wilkinson sub-keratous pustulosis, and neutrophilic eccrine hidradenitis. We report two cases of aseptic abscesses which correspond to the deepest anatomoclinical form of neutrophilic dermatosis. CASE REPORTS: A 28-year-old man was hospitalized for fever and abdominal pain with bloody diarrhea in relation with Crohn's disease. The patient also presented two skin abscesses on the lower limbs. Bacteriology specimens were negative. The histology specimen of a skin lesion revealed neutrophil infiltration of the hypodermis without granulomatosis. Systemic corticosteroid therapy was given and rapidly led to resolution of the inflammatory bowel disease and the skin lesions. The patient developed inflammatory spondylarthropathy several months later. The second patient was a 36-year-old woman with a history of splenomegaly with asceptic abscesses. She was admitted for abdominal pain with non-bloody diarrhea, fever and multiple joint pain related to spondylarthropathy. She developed several simultaneous abscessed nodules on the legs. Biopsy revealed neutrophil infiltration of the hypodermis. The diagnosis of neutrophilic disease with aseptic cutaneous and visceral abscesses was retained. Nonsteroidal antiinflammatory drugs and dapsone were given leading to regression of the skin lesions and the abdominal and joint pain. DISCUSSION: Aseptic skin abscesses result from a deep localization of neutrophilic disease. They suggest the presence of inflammatory bowel disease, spondylarthropathy or other aseptic visceral localizations.     

Systemic Hodgkin's lymphoma in a patient with Sézary syndrome

We report a case of a 71-year-old male with Sézary syndrome diagnosed in 1996 who subsequently developed systemic Hodgkin's lymphoma. His only past treatment was bath psoralen plus ultraviolet A. He has since been treated with multiagent chemotherapy (ChlVPP/PABLOE) which induced a remission in his Hodgkin's disease. Eighteen months later he remains in remission from Hodgkin's disease but the Sézary syndrome remains active. He has also developed a squamous cell carcinoma on the upper lip. Sézary syndrome is a primary cutaneous T-cell lymphoma characterized by a malignant proliferation of CD4-positive cells in the skin and peripheral circulation. The CD4 count may be markedly elevated but this results from expansion of a neoplastic T-cell clone and there is a relative lymphopenia of normal T cells leading to a degree of immunoparesis. Immunosuppression is known to be associated with an increased rate of malignancies and this may account for the occurrence of Hodgkin's disease and squamous cell carcinoma in this patient with Sézary syndrome.     

Reflex sympathetic dystrophy: description of a case with skin lesions

Reflex sympathetic dystrophy or algodystrophy is a poorly defined syndrome in which the patient develops pain disproportionate to the cause. It is included among the complex regional pain syndromes. The symptoms are triggered by some type of trauma, at times trivial, and consist of burning pain, edema, changes in skin color, alterations in vascularization, temperature changes, hyperhidrosis and skin disorders, which primarily consist of atrophic changes. Other less frequent cutaneous manifestations have been described in patients with this syndrome. These include papules, blisters, inflammatory lesions and reticulated hyperpigmentation. We discuss the case of a patient with reflex sympathetic dystrophy who presented with superficial ulcers on the affected limb, which mimicked dermatitis artefacta.     

Familial yellow nail syndrome

A 70-year-old woman with yellow nail syndrome and right-sided pleural effusion, lower extremity edema, and hypoalbuminemia was followed for 18 months. She reported an 8-year history of asthma. She had four children (3 boys and 1 girl). Dystrophy, changes in color and shape of nails both hands and foot, along with lower extremity edema was observed in the daughter and two of her sons. One son had asthma. The patient reported that her grandmother had similar nail abnormality and lower extremity edema. Other family members and patient's grandchildren were healthy. This report demonstrates a case of familial yellow-nail syndrome.     

Steroid atrophy scarring treated with fat grafting in a patient with complex regional pain syndrome: A case report

Subcutaneous atrophy is a known complication of steroid injections. Excellent results with fat grafting for the treatment of steroid atrophy have been documented. However, the benefit of treating steroid-induced subcutaneous atrophy in an extremity diagnosed with complex regional pain syndrome (CRPS) has not been described. CRPS, known formerly as reflex sympathetic dystrophy or RSD, causalgia, or reflex neurovascular dystrophy, is a severe, progressive musculoskeletal pain syndrome characterized by pain which is disproportionate to the severity of the inciting event, edema, or skin changes. Common treatment modalities include pharmacotherapy, physical therapy, and nerve blocks—each therapy producing varying results. We present a literature review of CRPS and the case of a 15-year-old female who developed CRPS of the left lower leg after arthroscopic debridement with retrograde drilling of an osteochondral lesion. Steroid atrophy of the involved area following a saphenous nerve block complicated the patient's treatment course. The area of atrophy was treated with autologous fat grafting. Following the adipose injection procedure, the patient experienced almost complete resolution of her CPRS-associated pain symptoms, along with improved cosmetic appearance of the area.     

Subcutaneous panniculitic T-cell lymphoma and cytophagic histiocytic panniculitis

A 43-year-old Maori man presented with a 1 month history of malaise, weight loss, anorexia, arthralgia, recurrent fever and tender erythematous subcutaneous skin lesions. Histological examination of an incisional biopsy of a lesion revealed a lobular panniculitis with an inflammatory infiltrate of atypical lymphocytes and evidence of cytophagocytosis consistent with a diagnosis of subcutaneous T-cell lymphoma. The systemic symptoms and skin lesions resolved spontaneously within 3 weeks, only to recur 2 months later, requiring treatment with oral prednisolone. T-cell gene rearrangement studies demonstrated a monoclonal T-cell receptor (gamma-chain) gene rearrangement, further supporting the diagnosis of subcutaneous panniculitic T-cell lymphoma. Treatment with chemotherapy (cyclophosphamide, doxorubicin, vincristine and prednisone) led to remission of symptoms. Four months after completing chemotherapy, the patient remained asymptomatic with a few indurated subcutaneous plaques on the chest. Biopsy of these areas revealed lobular panniculitis, lymphocytic infiltrate without cytological atypia, abundant lipophages and fibrosis and sclerosis consistent with a healing response. He remains well 24 months following chemotherapy.     

A case of subcutaneous panniculitis-like T-cell lymphoma with haemophagocytosis developing secondary to chemotherapy

A 45-year-old woman presented with fever, generalized skin lesions and multiple lymphadenopathies. In her past history she had had six courses of cyclophosphamide and cisplatin combination chemotherapy 7 years ago because of an ovarian carcinoma. We found pancytopenia in the peripheral blood examination. Skin biopsy showed diffuse subcutaneous infiltration reminiscent of panniculitis but composed of malignant lymphoid cells that were of T lineage. Bone marrow biopsy showed normocellular myeloid tissue with abundant haemophagocytic macrophages. Subcutaneous panniculitis-like T-cell lymphoma with haemophagocytic syndrome was diagnosed. This is the first case reported of subcutaneous panniculitis-like lymphoma occurring secondary to chemotherapy.     

Unusual case of subcutaneous panniculitis-like T-cell lymphoma

An unusual case of subcutaneous panniculitis-like T-cell lymphoma is presented involving multiple organ systems, which eventually culminated in rapid demise from the haemophagocytic syndrome, after an initial protracted course. A 44-year-old man presented in April 2001 with bronchiolitis obliterans organising pneumonia that initially responded well to corticosteroids. However, the condition relapsed on attempted prednisone withdrawal in January 2002 and the patient was noted to have developed truncal subcutaneous nodules. Initial skin biopsy revealed lobular panniculitis, with negative microbiological culture. In July 2002, mononeuritis multiplex was diagnosed after the patient presented with paresthesiae and was treated with pulse cyclophosphamide therapy. By November 2002 there was ulceration of the subcutaneous nodules. Repeat skin biopsy revealed subcutaneous panniculitis-like T-cell lymphoma. The clinical manifestations were supportive of an unifying diagnosis of malignancy involving pulmonary, cutaneous and nervous systems. Combination chemotherapy with fludarabine, mitoxantrone and dexamethasone was commenced. However, the patient deteriorated, with fevers, weight loss, pancytopenia and laboratory features consistent with the haemophagocytic syndrome. Despite maximal supportive therapy the patient succumbed to his disease.     

Atypical manifestations of pityriasis lichenoides chronica: development into paraneoplasia and non-Hodgkin lymphomas of the skin.

Three patients with atypical courses and manifestations of pityriasis lichenoides chronica (PLC) are presented. The first patient is a 21-year-old white woman who showed a good response of her PLC lesions as well as her reactive oligoarthritis to repeated PUVA treatments combined with oral prednisone during 1 year. The effect of the treatment then decreased. The patient developed a low-grade malignant lymphoma of the lung. When the lymphoma of the lung improved after chemotherapy, the PLC eruptions improved, too. The second patient is a 41-year-old man, whose Hodgkin's disease stage IVa was successfully treated by chemotherapy and radiotherapy in 1984. In 1987 he showed PLC lesions which responded well to PUVA therapy, later also in combination with etretinate. Until 1988 repeated skin biopsies revealed a non-specific eczematous pattern. In 1989 the recalcitrant PLC eruptions finally revealed a pleomorphic non-Hodgkin lymphoma of the skin with medium-sized cells. The third patient had a PLC for about 9 years when Hodgkin's disease stage Ia was diagnosed. At the beginning the skin biopsy showed an eczematous pattern, but 2 years later, in 1990, skin infiltrations of a large-cell, anaplastic non-Hodgkin lymphoma were seen. These cases show that PLC in rare cases may either represent a paraneoplastic skin disease or may itself develop into cutaneous lymphomas.     

Docetaxel-induced Hand and Foot Syndrome in a Patient with Metastatic Breast Carcinoma

Hand and foot syndrome (HFS) is a well-known complication of chemotherapeutic drugs given in a dose-dense manner. Our patient was a 52-year-old female with metastatic breast carcinoma on salvage chemotherapy regimen with docetaxel at a dose of 60 mg/m². The patient had grade 3 HFS characterized by symmetrical, tender, and erythematous skin lesions over the palms and soles associated with dysesthesia necessitating interruption of treatment. She developed this syndrome at a much lower dose than previously described due to her altered hepatic function. An insight regarding this unique distressing side-effect and assessment of various contributing factors would help us identify and treat the patient at the earliest.     

Sweet's syndrome--like neutrophilic lobular panniculitis associated with all-trans-retinoic acid chemotherapy in a patient with acute promyelocytic leukemia

Sweet's syndrome-like (Sweet's-like) neutrophilic panniculitis is usually idiopathic, but is frequently associated with hematologic, inflammatory, and immunologic disease. Drug-related cases of Sweet's syndrome have been reported. Of relevance, chemotherapy with the retinoid all-trans-retinoic acid (ATRA) infrequently induces Sweet's-like neutrophilic panniculitis that occurs concomitantly with neutrophilic differentiation. The pathologic features are limited to the adipose tissue or include both the dermis and the subcutaneous fat; the neutrophilic infiltrate can be observed in the lobules, the septae, or both. We present this case because of the unusual subcutaneous neutrophilic infiltrate consistent with Sweet's-like neutrophilic lobular panniculitis in a patient with acute promyelocytic leukemia receiving ATRA chemotherapy. This case highlights the important connection between ATRA and Sweet's syndrome.     

The dysmorphic syndrome

A 49-year-old woman complained of pain and distortion of the facial skin of five years' duration. She had notable psychological depression, and the distortion she described was not apparent to others. She had sought relief for many years from many physicians and had tried numerous therapeutic regimens to no avail. After a diagnosis of "dysmorphic syndrome," haloperidol was prescribed at a dosage of 1 mg twice daily. With this regimen, the patient experienced a slow but steady improvement, although her compliance was erratic.     

Dermatomyositis associated with generalized subcutaneous edema and Evans syndrome

Although periorbital edema is a common manifestation of dermatomyositis (DM), generalized subcutaneous edema associated with DM is extremely rare. Evans syndrome is an autoimmune disease in which an individual's antibodies attack one's own red blood cells and platelets. Evans syndrome is rarely a presenting feature of DM. DM has been rarely reported to be associated with either generalized edema or Evans syndrome. We report the case of a 52-year-old Korean woman who presented with generalized subcutaneous edema, an erythematous rash, dysphagia, and proximal muscle weakness, and subsequently developed features of Evans syndrome. Treatment with high-dose glucocorticoids and an immunosuppressive agent controlled the DM, the generalized subcutaneous edema, and the Evans syndrome.     

Cutaneous angiomas in POEMS syndrome. An ultrastructural and immunohistochemical study

Cutaneous manifestations of POEMS (polyneuropathy, organomegaly, endocrinopathies, M-protein, and skin changes) syndrome in a 51-year-old white man included two types of hemangiomas, ie, cherry-type and subcutaneous hemangiomas. By light microscopy and immunohistochemistry, both types of tumors showed features of capillary hemangiomas. By electron microscopy, a peculiar finding of subcutaneous hemangiomas was the presence of slender abluminal processes of endothelial cells that were frequently anastomosing to form electron-lucent blebs. Although the precise etiopathogenesis of the vascular proliferations remains speculative, these lesions merit study since they constitute an easily recognizable marker of POEMS syndrome.     

A case of symmetrical leukemia cutis on the eyelids complicated by B-cell chronic lymphocytic lymphoma

We describe herein a case of symmetrical bilateral leukemia cutis on the eyelids, especially on the lower lids, in a patient with B-cell chronic lymphocytic leukemia (CLL). The patient was a 63-year-old Japanese man who had been diagnosed with CLL. Eyelid swelling is well known to result from local skin or systemic diseases including tumors, hyperthyroidism, nephritic syndrome, and hypoalbuminemia. To our knowledge, symmetrical, bilateral, flesh-colored leukemia cutis on the eyelids has not been previously reported.     

Linear IgA dermatosis and Hodgkin's lymphoma--report of a case in an African and review of the literature

Linear IgA dermatosis and Hodgkin's lymphoma were diagnosed at the same time in a 47-year-old Black South African man. Skin changes of linear IgA dermatosis responded to a combined treatment with dapsone and MOPP regimen. Prior administration of dapsone alone did not result in improvement of the skin condition. Four documented cases of linear IgA dermatosis and lymphoma outside the gastrointestinal tract, three of them of Hodgkin's type, have been reported in the literature. Review of the literature suggests that different forms of lymphoma seem to be associated with linear IgA dermatosis and dermatitis herpetiformis. The present patient represents the first immunologically verified case of linear IgA dermatosis in an adult Black African.