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Granulomatous Slack Skin


Presentation

  • The clinical and pathological aspects of the 20 cases of GSS reported in the recent literature are reviewed and compared with those of the present case.[ncbi.nlm.nih.gov]
  • We describe a case of granulomatous slack skin in a 31-year-old woman with an unusual presentation of acquired ichthyosis and muscular masses involving four limbs over 3 years.[ncbi.nlm.nih.gov]
  • The clinical, histological, immunohistochemical, ultrastructural, molecular biological and genetic studies with a review of 39 other cases described in the literature are presented.[ncbi.nlm.nih.gov]
  • We present here a patient with areas of lax skin for several years who developed a generalized erythroderma with associated immunoactivation and a deterioration in his general condition.[ncbi.nlm.nih.gov]
  • Presented in part at the XXVIII Symposium of the International Society of Dermatopathology, Paris, November 14-17, 2007. A 13-year-old boy developed a bulky mass close to the right axillary region and an infiltrated plaque on the abdominal skin.[ncbi.nlm.nih.gov]
Inguinal Hernia
  • The surgical treatment of bilateral inguinal hernia with reposition of inguinal dislocated vasculature is also reported.[ncbi.nlm.nih.gov]
  • hernia with dislocation of great saphenous vein as complication of long-standing granulomatous slack skin: a case report. ( 16684291 ) Benedetti M...Borroni G 2006 35 Granulomatous slack skin. ( 17459306 ) Kavusi S...Nasertork A 2006 36 Granulomatous[malacards.org]
Inguinal Lesions
  • The inguinal lesions progressively enlarged to become bulky pendulous tumors Pathology Skin biopsy reveals a patchy lichenoid and nodular infiltrate of lymphocytes extending from the superficial dermis to the subcutaneous fat and even to the skeletal[plasticsurgerykey.com]
Aspiration
  • Complete blood count, biochemistry, chest X-ray, fine-needle aspiration of the inguinal lymph node, and bone marrow biopsy were performed without alterations.[ncbi.nlm.nih.gov]
  • Computerized tomography of the chest, abdomen and pelvis showed shotty pulmonary, mesenteric and retroperitoneal lymph nodes; however fine needle aspiration cytology/biopsy could not done as the patient did not give her consent.[ijdvl.com]
Persistent Cough
  • The patient also developed a persistent cough and worsening exertional dyspnea five years after her diagnosis.[omicsonline.org]
Abdominal Mass
  • Histologic examination of a specimen from the abdominal mass revealed an extensive lymphoid infiltrate with scattered multinucleated giant cells extending from the papillary dermis to the subcutis.[ncbi.nlm.nih.gov]
Cardiomegaly
  • Repeat CT of the chest showed progression of ground-glass opacities in the right lung apex, chronic multilobar bronchiectasis with cicatrization atelectasis, cardiomegaly and dilated pulmonary artery compatible with pulmonary hypertension.[omicsonline.org]
Cutis Laxa
  • A 39-year-old man had a two-year history of three indurated plaques that progressively underwent a cutis laxa-like transformation. Histopathologically a granulomatous, deep, dermal infiltrate was observed.[ncbi.nlm.nih.gov]
  • Granulomatous slack skin is a rare variant of indolent cutaneous lymphoma, characterized by a cutis laxa-like clinical appearance and widespread granulomatous infiltration.[ncbi.nlm.nih.gov]
  • Marked cutis laxa skin laxity in the axillary region and cervical adenopathy. Cutis laxa skin laxity in the occipital region. Tumor with multiple fistulous orifices in the right internal thigh.[ncbi.nlm.nih.gov]

Workup

  • Further invasive workup such as additional lung biopsies or bronchoscopic studies was not pursued because the previous studies were unremarkable.[omicsonline.org]
Atelectasis
  • Repeat CT of the chest showed progression of ground-glass opacities in the right lung apex, chronic multilobar bronchiectasis with cicatrization atelectasis, cardiomegaly and dilated pulmonary artery compatible with pulmonary hypertension.[omicsonline.org]

Treatment

  • Stable or progressive disease was observed in most patients despite various treatment modalities.[ncbi.nlm.nih.gov]
  • Ultraviolet A1 (UVA1) phototherapy has previously been reported to be useful in the treatment of CTCL such as mycosis fungoides.[ncbi.nlm.nih.gov]
  • Notwithstanding its indolent behavior, treatment is often disappointing. We describe an additional case of this rare disorder in a 44-year-old male patient.[ncbi.nlm.nih.gov]
  • There is no known curative treatment, but surgery, radiation therapy, systemic corticosteroids, and chemotherapy have slowed the progression of some lesions.[ncbi.nlm.nih.gov]
  • When this treatment was stopped, the disease relapsed again. Extensive surgery was undertaken. Recently, a new relapse has occurred which was again treated by extensive surgery. No other manifestation of a lymphoproliferative disorder appeared.[ncbi.nlm.nih.gov]

Prognosis

  • The prognosis of GMF appears worse than that of classic nongranulomatous mycosis fungoides.[ncbi.nlm.nih.gov]
  • Diffuse positivity with CD4 in the lymphoid cell infiltrate (immunoperoxidase, x100) DISCUSSION GSS is a rare entity with good prognosis, recognized as a variant of mycosis fungoides.[ncbi.nlm.nih.gov]

Etiology

  • This article reviews its literature on the etiology, clinical findings, and treatment of Granulomatous Slack Skin Disease.[ncbi.nlm.nih.gov]
  • McKee's Pathology of the Skin is the most complete, in-depth resource on dermatopathology, covering etiology, pathogenesis, disease mechanisms, and recent genetic, molecular, and basic science data. Drs. J.[books.google.com]
  • Other possible etiologies that may contribute to the patient’s pulmonary findings include radiation induced lung disease given her multiple radiation treatments, reactive granulomatous conditions, cryptogenic infection, and sarcoid-lymphoma-syndrome.[omicsonline.org]
  • The exact etiology of this condition is presently unknown. A chromosomal translocation involving t (3;9) (q12; p24) has been described. The rearrangement of T-beta cell receptor gene is seen in molecular analysis.[ijdvl.com]
  • […] a good term since nonspecific as to histological type) Epidemiology Most common type of cutaneous T cell lymphoma, almost 50% of all primary cutaneous lymphomas Adults / elderly but also children and adolescents M:F ratio 2:1, black:white ratio 1.7 Etiology[pathologyoutlines.com]

Epidemiology

  • (effective October 01, 2015) Signs and Symptoms Itchy, painful, peeling and reddened skin Ulcerated skin lesion s, which may become infected Diagnostic Exams Flow cytometry Immunophenotyping Peripheral blood smear Progression and Transformation None Epidemiology[seer.cancer.gov]
  • Data from cancer registries of the Surveillance, Epidemiology, and End Results (SEER) Program found the incidence of MF to be 0.64 per 100,000 person years, with a median age at diagnosis of 55 to 60 years. 1-3 The incidence of MF/Sezary syndrome (SS)[jddonline.com]
  • 1814), because it resembles yaws (caused by Treponema), which is also called pian Later renamed as mycosis fungoides, due to its resemblence to mushrooms Also called cutaneous T cell lymphoma (not a good term since nonspecific as to histological type) Epidemiology[pathologyoutlines.com]
  • Epidemiology [ 3 ] CTCLs typically affect adults with a median age of 55-60 years; the annual incidence is about 0.5 per 100,000.[patient.info]
Sex distribution
Age distribution

Prevention

  • Despite the indolent behavior of granulomatous slack skin, its early recognition and continuous monitoring by a dermatologist becomes essential for its management and prevention of an unfavorable outcome.[ncbi.nlm.nih.gov]
  • The information presented here is not intended to diagnose, treat, cure or prevent any disease. Read disclaimer.[gulfdoctor.net]
  • Your skin Holds body fluids in, preventing dehydration Keeps harmful microbes out, preventing infections Helps you feel things like heat, cold, and pain Keeps your body temperature even Makes vitamin D when the sun shines on it Anything that irritates[icdlist.com]

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