Sarcoidosis is highly polymorphic in its clinical manifestations [1]. Clinically, sarcoidosis can mimic many skin diseases. However, Panniculitis as presenting feature in sarcoidosis is rarely reported [1] [2] [3].

We report a case of generalized granulomatous panniculitis in a patient with sarcoidosis.

A 62-year-old male, without remarkable past medical history, was admitted for a large erythematous indurated plaque on abdominopelvic region lasted for two years. Physical examination showed an erythematous indurated plaque with an orange peel appearance in abdominal lower quadrant, pubic, external genitalia and thighs. The plaque was studded with hyperkeratotic papule especially on pubic region. A generalized ichthyosis sparing scalp and palmar-plantar region was noted (Figure 1). There were also consistent, painless, fixed lymphadenopathies of 2 to 7 cm in diameter, in auxiliary and inguinal regions. The skin and lymph node histopathologic studies revealed typical sarcoidosis granulomas. In skin, these granulomas were deeply situated in subcutaneous tissues and occupying fat lobules (Figure 2). The abdomen and thoracic CT scan was normal. Tuberculin test was anergic and HIV serological test was negative. CBC and blood smear were normal. After six months of treatment, combining methotrexate 25 mg intramuscularly per week and prednisone 0.5 mg / kg /day, the outcome was favorable, marked by fast skin lesions desinfiltration, disappearance of ichthyosis and lymphadenopathy regression (Figure 3).

We report a cutaneous and lymph node sarcoidosis in which skin involvement was confirmed by histological examination as panniculitis. Specific manifestations of cutaneous sarcoidosis are related with the location of granulomas in the dermis. In literature, the frequency varies between 9% and 30% in different studies [4] [5] .

They are characterized by small and large nodules called sarcoid, infiltrating sarcoid and sarcoid on scars. Hypodermic sarcoid of Darier-Roussy represents 4–12% of the specific lesions of sarcoidosis [5] [6] and appear as cold nodules developing in a normal-looking skin. They are located more frequently on the limbs and rarely on the trunk.

Cutaneous sarcoidosis with panniculitis as clinical presentation, it has rarely been reported [1] [7] [8]. To our knowledge, only 2 cases of sarcoidosis with extensive panniculitis have been reported. In one case, it was only a localized lesion on one limb [8]. In another case, the lesions were on supraclavicular, shoulder and upper back [7].

In our patient, the lesions were more generalized, on abdomen, pelvis, genitals and thighs. This very unusual clinical presentation can rise a problem of differential diagnosis with cutaneous lymphoma. However, the histopathologic features of sarcoidosis can make the difference with the skins as well as the lymph node lymphoma.

The treatment of sarcoidosis remains is poorly codified [9]. Corticosteroids are the standard treatment, but with a purely suspensive effect [10] [11]. It is indicated in severe eye, neurological, cardiac, renal, laryngeal and lung involvement or progressive disabling lesions and lupus pernio with severe hypercalcemia. However, glucocorticoids cause many side effects at more than 50% of patients.

Side effects are even more frequent in height doses and long duration treatment [12] thus, methotrexate is often proposed as an alternative, especially as a reserve when long-term treatment with prednisone is obligatory [12]. In our case, treatment with methotrexate and prednisone had achieved a complete remission of lesions after six months. Other studies [13] [14][15] have confirmed the effectiveness of methotrexate, especially as steroid sparing [16] treatment. This effectiveness would be the result of an increase in adenosine production and a decrease in the secretion and TNF a at granulomatous lesions [12].

Diffuse panniculitis as a presenting feature of sarcoidosis is rare. In such cases, only histopathology can confirm the diagnosis. Although treatment is not well classified, Methotrexate associated with prednisone seems to work well.

1. Descamps V, Bouscarat F, Marinho E. Manifestations cutanées de la sarcoïdose EMC-Dermatologie-Cosmétologie 2005;(2):177–88.    
2. Kong F, Leng XM, Li L, Zhang FC. The clinical analysis of 35 patients with cutaneous sarcoidosis. Zhonghua Nei Ke Za Zhi 2011 May;50(5):397–400.   [Pubmed]    
3. Papadavid E, Dalamaga M, Stavrianeas N, Papiris SA. Subcutaneous sarcoidosis masquerading as cellulitis. Dermatology. 2008;217(3):212–4.   [CrossRef]   [Pubmed]    
4. Descamps V, Bouscarat F. Manifestations cutanées de la sarcoïdose. Encycl Med Chir. (Elsevier Paris), Dermatologie, 1999 98-470-A10, 7p.    
5. Bessis D, Huet P. Sarcoïdose. In: Bessis D, Francés C, Guillot B, Guilhou JJ, éds, Dermatologie et Médecine, vol. 1 : Manifestations dermatologiques des connectivites, vasculites et affections systémiques apparentées. Springer-Verlag France 2006. p. 17.1–17.    
6. Khaled A, Zribi H, Zeglaoui F, El Fekih N, Ezzine N, Fazaa B, Mokhtar I, Kamoun Mr. Les manifestations cutanées de la sarcoïdose à travers une série hospitaliére de 128 cas Ann Dermatol Venereol 2005;132:9S71–9S279.    
7. Tada Y, Kanda N, Ohnishi T, Watanabe S. Atypical cutaneous sarcoidosis with diffuse, indurated erythema. Eur J Dermatol 2009 Nov-Dec;19(6):639.   [Pubmed]    
8. Requena L. Normal subcutaneous fat, necrosis of adipocytes and classification of the panniculitides. Semin Cutan Med Surg 2007 Jun;26(2):66–70.   [CrossRef]   [Pubmed]    
9. Gary A, Modeste AB, Richard C, et al. Methotrexate for the treatment of patients with chronic cutaneous sarcoidosis: 4 cases.[Article in French] Ann Dermatol Venereol 2005 Aug-Sep;132(8-9 Pt 1):659–62.   [Pubmed]    
10. Baughman RP, Costabel U, du Bois RM. Treatment of sarcoidosis. Clin Chest Med 2008 Sep;29(3):533–48.   [CrossRef]   [Pubmed]    
11. Paramothayan S, Jones PW. Corticosteroid therapy in sarcoidosis: A systematic review. JAMA 2002 Mar 13;287(10):1301–7.   [CrossRef]   [Pubmed]    
12. Londner C, Zendah I, Freynet O, et al. Traitement de la sarcoïdose. Rev Med Interne 2011 Feb;32(2):109–13.   [Pubmed]    
13. Veien NK, Brodthagen H. Cutaneous sarcoidosis treated with methotrexate. Br J Dermatol 1977 Aug;97(2):213–6.   [CrossRef]   [Pubmed]    
14. Lower EE, Baughman RP. The use of low dose methotrexate in refractory sarcoidosis. Am J Med Sci 1990 Mar;299(3):153–7.   [CrossRef]   [Pubmed]    
15. Lower EE, Baughman RP. Prolonged use of methotrexate for sarcoidosis. Arch Intern Med 1995 Apr 24;155(8):846–51.   [CrossRef]   [Pubmed]    
16. Müller-Quernheim J, Kienast K, Held M, Pfeifer S, Costabel U. Treatment of chronic sarcoidosis with an azathioprine/prednisolone regimen. Eur Respir J 1999 Nov;14(5):1117–22.   [CrossRef]   [Pubmed]