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                   Cutaneous Sarcoidosis

          Dr Sampurna Roy MD

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Skin sarcoidosis occurs in about 25% of patients with systemic disease and may also arise in isolation.

A wide range of clinical presentations of cutaneous sarcoidosis is recognised.

The diagnosis rests on the presence of non-caseating granulomas on skin biopsy and the exclusion of other granulomatous skin disease.

The treatment and overall prognosis of cutaneous sarcoidosis is primarily dependent on the degree of systemic involvement.

In patients with aggressive disease limited to the skin immunosuppressive therapy may be indicated.  

Visit:  Sarcoidosis   ;  Cutaneous Granulomatous Reaction Pattern

Microscopic Images of Cutaneous Sarcoidal  Granulomas

          

                         

          

               

Clinical spectrum and histological analysis of 32 cases of specific cutaneous sarcoidosis.J Cutan Pathol. 2006 Dec;33(12):772-7.

BACKGROUND: Sarcoidosis is a multisystem granulomatous disorder of unknown aetiology in which skin involvement is frequent. OBJECTIVE: To review histological characteristics of biopsies of specific cutaneous lesions of sarcoidosis and their relationship with clinical course. PATIENTS AND METHODS: Biopsies from 32 patients with specific cutaneous sarcoidosis were reviewed. Histological findings and clinical characteristics of these patients were analysed. RESULTS: The initial clinical lesions of the patients were ten infiltrated nodule-plaques, eight papules, four maculopapular eruptions, five scar sarcoidosis, four subcutaneous nodules and one lupus pernio. Sarcoidal granulomas were located at dermis in 31 cases (74%) and at subcutaneous fat in 12 (28%) but only four were subcutaneous exclusively. Perivascular or periannexial distribution of granulomas was observed in eight cases (19%) and they had coalescence in 29 samples. The presence of foreign material was demonstrated in 11 cases (26%). CONCLUSIONS: Clinical spectrum of specific lesions of cutaneous sarcoidosis showed a good correlation with granulomas localization in the biopsies. However, traditional classification of specific cutaneous sarcoidosis is often overlapping. On the other hand, foreign bodies and other atypical histological findings were more common than initially expected.

Subcutaneous sarcoidosis: is it a specific subset of cutaneous sarcoidosis frequently associated with systemic disease?J Am Acad Dermatol.2006 Jan;54(1):55-60. Epub 2005 Dec 2.

BACKGROUND: Skin is involved in 25% of cases of sarcoidosis. The lesions are specific and nonspecific depending on the presence or absence of granulomas, respectively. Specific lesions are not thought to have prognostic significance and are not associated with systemic disease. OBJECTIVE: We sought to evaluate for the presence or absence of systemic disease in patients with subcutaneous sarcoidosis. METHODS: With diagnostic criteria of subcutaneous sarcoidosis, 33 cases were identified in the literature and 21 cases in our institutional database. A retrospective clinical and pathologic review of these cases was conducted. RESULTS: Subcutaneous sarcoidosis is characterized by a peak incidence during the fourth decade; female predisposition; asymptomatic to slightly tender lesions typically involving the upper extremities; cutaneous lesional clustering and multiplicity; autoimmune disease associations at time of diagnosis in a subset of cases; systemic disease associations at diagnosis in most patients, typically consisting of bilateral hilar adenopathy; and a favorable response to oral corticosteroid therapy. LIMITATIONS: Retrospective analysis with inadequate documentation of therapeutic regimens and their responses in some cases is a limitation of this study. CONCLUSIONS: The confirmatory diagnosis of subcutaneous sarcoidosis depends on identifying pannicular noninfectious sarcoidal or epithelioid granulomas with minimal lymphocytic inflammation. Subcutaneous sarcoidosis is the only specific subset of cutaneous sarcoidosis frequently associated with systemic disease.

The histologic spectrum of cutaneous sarcoidosis: a study of twenty-eight cases. J Cutan Pathol.2004 Feb;31(2):160-8

BACKGROUND: Naked sarcoidal granulomas (NSGs) are the characteristic histologic finding in sarcoidosis. This descriptive study was designed to identify the frequency of other histologic changes in cutaneous sarcoidosis. METHODS: The slides from 28 sequential biopsies previously diagnosed as sarcoidosis in patients with known systemic sarcoidosis were reviewed. RESULTS: Classic NSGs were identified in 25 biopsies (89%). Four biopsies contained tuberculoid granulomas, two with neutrophils suggesting infection (cultures negative). Five biopsies contained interstitial granulomas that resembled granuloma annulare and necrobiosis lipoidica in one case each. Additional histologic findings included birefringent foreign material in 14 biopsies (50%), focal necrosis (43%), elastophagocytosis (39%), linear peri-neural granulomas resembling leprosy (25%), increased dermal mucin (18%) and lichenoid inflammation (14%) [two with plasma cells resembling syphilis (7%)]. In all but three cases, the clinical morphology of the lesions suggested sarcoidosis. Special stains for mycobacteria and fungi were negative. CONCLUSIONS: The histologic changes in cutaneous sarcoidosis are more diverse than previously recognized. In sarcoidosis, foreign material may be a frequent nidus for cutaneous granuloma formation. Histologic examination without the clinical history could lead to a misdiagnosis of leprosy, syphilis, other infectious granulomas, rosacea, granuloma annulare, necrobiosis lipoidica, and foreign body reaction in selected cases from this series.

Cutaneous involvement in sarcoidosis. Relationship to systemic disease. Arch Dermatol.1997 Jul;133(7):882-8

Sarcoidosis is an antigen-mediated disease defined by granuloma formation in different organs. It involves mainly the mediastinal and peripheral lymph nodes, lungs, eyes, skin, liver, and spleen. Cutaneous lesions of sarcoidosis may be specific, showing histologically noncaseating granulomas, or nonspecific, most typically erythema nodosum. Frequently, both types of skin lesions are the means of presentation of the disease and may contribute to the diagnosis. A workup for systemic sarcoidosis should be undertaken in every patient with sarcoid cutaneous granulomas. Some types of cutaneous lesions have prognostic significance. Lupus pernio and plaques are associated with more severe systemic involvement and more chronic course, while erythema nodosum is the hallmark of acute and benign disease.

Sarcoidosis of the skin. Nippon Rinsho.1994 Jun;52(6):1603-7

Sarcoidosis is characterized by formation of epithelioid-cell tubercules, without caseation, of the affected organ systems. The mediastinum, peripheral lymph nodes and eyes, in addition to the skin, are most frequently affected. Between 10% and 30% of patients with systemic sarcoidosis in Japan have skin lesions. Skin sarcoidosis is morphologically classified into three basic groups, erythema nodosum, scar sarcoidosis and skin sarcoid. Skin sarcoid is characterized by specific cutaneous lesions of sarcoidosis, and may take nodular, plaque, angiolupoid, subcutaneous and some other forms. Clinical manifestations of the cutaneous lesions are usually asymptomatic and polymorphous. Skin biopsy is, however, often highly useful for confirming a diagnosis of sarcoidosis.

Abstracts:

Cutaneous sarcoidosis therapy updated.J Am Acad Dermatol. 2007 Jan;56(1):69-83.

Cutaneous sarcoidosis: the "great imitator": etiopathogenesis, morphology, differential diagnosis, and clinical management.
Am J Clin Dermatol. 2006;7(6):375-82.

Scar sarcoidosis: a case report and brief review.Cutis. 2006 Dec;78(6):418-22.

Skin manifestations of sarcoidosis. Review of the literature and 53 personal cases.Ann Med Interne (Paris). 1984;135(2):97-101

 
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