DERMATOPATHOLOGY CASES: Self-Assessment Cases: Editor - Dr Sampurna Roy MD

Digital Images of interesting cases that will include the full spectrum of Dermatopathology, presented in the form of quiz.

The answer of the cases include related links and recent abstracts of articles.

Sunday, August 22, 2010

Answer of Dermatopathology Case 63

Cutaneous Sarcoidosis

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Sarcoidosis of the skin--a dermatological puzzle: important differential diagnostic aspects and guidelines for clinical and histopathological recognition. J Eur Acad Dermatol Venereol.2010 Feb;24(2):125-37. Epub 2009 Aug 17.
Sarcoidosis of the skin may have an extremely heterogeneous clinical presentation, so that the definitions of 'great imitator' and 'clinical chameleon' have long been used. There is, in fact, a large group of skin diseases that can enter the differential diagnosis with cutaneous sarcoid manifestations, either clinically or/and pathologically. As the clinical consequences and the prognosis of these groups of diseases are often very different, it is important to correctly plan the diagnostic workup. The diagnostic process in this case often presents a challenge as no single test is sufficiently specific, so that a certain diagnosis can be only made in the presence of a compatible clinical and radiographic picture, along with histopathological evidence of non-necrotizing, epithelioid cell granulomas, and exclusion of other potential aetiologies. For practical reasons, four main groups of skin conditions capable of mimicking sarcoidosis can be identified: (i) transmissible, infectious diseases; (ii) allergic and immunological manifestations of various aetiologies; (iii) granulomatous diseases of various aetiologies; and (iv) lymphomas and pseudolymphomas. The aim of this article is to describe the main clinical and histopathological findings of such disease entities, and to discuss the role of those features (morphological, pathological and laboratory) that can help distinguish them from sarcoidosis of the skin.

Cutaneous sarcoidosis.Semin Respir Crit Care Med. 2010 Aug;31(4):442-51. Epub 2010 Jul 27.
Sarcoidosis is a systemic disease with skin manifestations. Skin manifestations are classified as nonspecific if they are not characterized by granulomatous inflammation and specific if the lesions have granulomas histologically. Erythema nodosum is the most common nonspecific skin manifestation, and it portends a good prognosis. Specific skin lesions have a varied clinical appearance, although often they can be distinguished by their yellow translucent character. Despite the potential variable appearance, there are common clinical presentations. Lupus pernio lesions are nodular violaceous specific skin lesions found predominantly on the face associated with scarring and a poor prognosis. Treatment of cutaneous sarcoidosis is primarily done to avoid scarring and cosmetic disfigurement. Local and systemic corticosteroids are the mainstay of treatment for the disease. Corticosteroid-sparing agents used to manage the disease include antimalarials, methotrexate, and tetracycline antibiotics. Tumor necrosis factor-alpha (TNF-alpha) antagonists such as infliximab may have a role in cutaneous sarcoidosis, especially in refractory cases that are resistant to the standard regimens.

Cutaneous sarcoidosis presenting as multiple erythematous macules and patches.Ann Dermatol. 2009 May;21(2):168-70. Epub 2009 May 31.
Sarcoidosis is an idiopathic multisystemic disorder with variable cutaneous presentations that are classified as specific or non-specific according to the presence of non-caseating granulomas on histologic examination. Specific manifestations can include papules, scar sarcoidosis, ulcers, or even alopecia. Herein, we present a case of cutaneous sarcoidosis that presented as multiple erythematous macules and patches on the trunk and extremities of a 32-year-old man. The clinical appearance was unlike any other form reported in the literature.

Cutaneous sarcoidosis: the "great imitator": etiopathogenesis, morphology, differential diagnosis, and clinical management.Am J Clin Dermatol.2006;7(6):375-82.
Sarcoidosis is a multisystem disease that can involve almost any organ system. The underlying cause of the disease remains unknown. Immunopathologically and histologically, cutaneous sarcoidosis is characterized by a macrophage/T helper-1 cell-mediated, non-caseating, granulomatous inflammation process. An imbalance between proinflammatory and anti-inflammatory cytokines plays an important role in the development of cutaneous granulomas. Recognition of cutaneous sarcoidosis lesions is very important because they provide a visible clue to the diagnosis and are an easily accessible source of tissue for histologic examination. Because skin lesions of patients with the disease can exhibit many different morphologies, cutaneous sarcoidosis is known as one of the "great imitators" in dermatology. Specific manifestations can include patches (sometimes hypopigmented), papules, scar sarcoidosis, ulcers, ichthyosis, and alopecia. The treatment of cutaneous sarcoidosis is often frustrating because some of the skin lesions may be refractory to treatment or may recur following successful treatment. Systemic and topical corticosteroids are the most effective treatments for cutaneous sarcoidosis. This article focuses on the dermatologic aspects of sarcoidosis and includes a review of the most recent literature, which includes new data on the diagnosis, differential diagnosis, pathogenesis, and treatment of the disease.