Granulomatous slack skin. Histopathology diagnosis preceding clinical manifestations by 12 years.

Goldsztajn, Karen O; Trope, Beatriz Moritz; Lenzi, Maria Elisa Ribeiro; Cuzzi, Tullia; Ramos‐e‐Silva, Márcia

doi:10.3315/jdcr.2012.1117

Cited by 10 publications

(12 citation statements)

References 14 publications

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“…The primary distinction between the two entities is established by clinical evolution and prognosis. 4 , 9 GMF prognosis is restricted, with an overall 5-year survival rate of 66%, unlike GSS, which is indolent. 7 , 8 Other differential diagnoses include: anetodermia, which are smaller and circumscribed lesions; generalized elastolysis (cutis laxa), which covers the face and other organs with absent granulomatous infiltration; and other granulomatous diseases, such as granuloma annulare and sarcoidosis, both without the dense atypical lymphocytic infiltrate.…”

Section: Discussionmentioning

confidence: 99%

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Granulomatous slack skin: a rare subtype of mycosis fungoides

Motta

Soares

Nakandakari

et al. 2017

An. Bras. Dermatol.

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We report a case of granulomatous slack skin, a rare and indolent subtype of mycosis fungoides. It affects mainly men between the third and fourth decades. It is characterized by hardened and erithematous plaques that mainly affect flexural areas and become pedunculated after some years. Histological examination shows a dense infiltrate of small atypical lymphocytes involving the dermis (and sometimes the subcutaneous tissue) associated with histiocytic and multinucleated giant cells containing lymphocytes and elastic fibers (lymphophagocytosis and elastophagocytosis, respectively). Patients affected by this entity can develop secondary lymphomas. There are several but little effective therapeutic modalities described. 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.

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Section: Discussionmentioning

confidence: 99%

“…Multinucleated giant cells at the center of the lesions are positive for CD68, surrounded by CD1a-positive cells. 1 , 4 - 7 TCR monoclonal gene rearrangement has been identified. 8 The main differential diagnosis is with granulomatous mycosis fungoides (GMF), since elastophagocytosis can be found in both.…”

Section: Introductionmentioning

confidence: 99%

Granulomatous slack skin: a rare subtype of mycosis fungoides

Motta

Soares

Nakandakari

et al. 2017

An. Bras. Dermatol.

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“…Although these are symptoms of cutis laxa, they can overlap with other diseases such as mid-dermal elastolysis, which presents with erythema reticulated plaques with patches of fine wrinkling [12]. Granulomatous slack skin may also present in the same way as acquired cutis laxa, but it is characterized by painless purple plaques and redundant sagging skin [13].…”

Section: Discussionmentioning

confidence: 99%

Type II acquired cutis laxa associated with recurrent urticarial vasculitis: brief report

Nabatanzi

Male

et al. 2020

Allergy Asthma Clin Immunol

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Background: Cutis laxa is a connective tissue disease characterized by loose, wrinkled, and redundant skin. It is either inherited or acquired. In most cases, acquired cutis laxa is associated with neoplasms, drugs, and autoimmune diseases. We present a rare case of acquired cutis laxa following a recurrent urticaria-like eruption in the absence of an autoimmune disease, neoplasm, drugs and or syndrome. Case presentation:We report a case of a 45-year-old Chinese lady with a 1-year history of widespread pruritic urticarial eruption and a 6-month history of progressive skin wrinkling. On examination, the patient appeared older than her actual age, with apparent wrinkling on the mid-torso with generalized smooth, erythematous macules and wheals. A family history of similar conditions was absent. Biopsy revealed hypersensitivity and atrophy. Following the Food and Drug Administration (FDA) guidelines, we administered antihistamines, which relieved the itching, but her hyperpigmentation and cutis laxa never improved. Conclusion:Our case shows that the decrease of elastic fibers may be associated with the infiltration of inflammatory cells in the dermis. This supports the hypothesis that chemical mediators may play a major role in the destruction of elastic fibers, thus causing cutis laxa. In addition, we advise practitioners to take a complete clinical and family history to determine if the condition is inherited or acquired. Publisher's NoteSpringer Nature remains neutral with regard to jurisdictional claims in published maps and institutional affiliations.

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“…The distinguishing feature of GSS is the development of pendulous skin folds in the intertriginous regions, resembling cutis laxA [1,2]. Skin lesions in the nonintertriginous regions tend to not undergo this transformation, which is the result of elastophagocytosis by histiocytic giant cells [1][2][3].…”

Section: Discussionmentioning

confidence: 99%

Slack Skin Syndrome with Granulomatous Involvement of the Lungs: A Case Report and Literature Review

Vangipuram¹,

Wang²

2015

Clin Exp Dermatol Res

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Granulomatous slack skin. Histopathology diagnosis preceding clinical manifestations by 12 years.

Abstract: During the twelve years of follow-up, the clinical manifestation evolved to marked skin looseness, most predominant in flexural regions, illustrating the clinical hallmark of granulomatous slack skin, long after first histological abnormalities were observed.

Cited by 10 publications

References 14 publications

Granulomatous slack skin: a rare subtype of mycosis fungoides

Granulomatous slack skin: a rare subtype of mycosis fungoides

Type II acquired cutis laxa associated with recurrent urticarial vasculitis: brief report

Slack Skin Syndrome with Granulomatous Involvement of the Lungs: A Case Report and Literature Review

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