Case for diagnosis

Abstracts

A sarcoidose é uma doença sistêmica crônica idiopática caracterizada histologicamente por um acúmulo de fagócitos mononucleares e células gigantes, que levam à formação de granulomas não-caseosos em diversos órgãos. O envolvimento cutâneo ocorre, em aproximadamente, 25% dos casos, sendo representado por lesões específicas e não-específicas. Apresentamos caso raro de sarcoidose com lesões cutâneas papulosas e umbilicadas e envolvimento pulmonar.

Granuloma; Pele; Sarcoidose

WHAT IS YOUR DIAGNOSIS ?

Case for diagnosis

Elisangela Samartin Pegas Pereira^I; Maria do Rosário Vidigal^II; Antônio José Tebcherani^III; Ana Paula Galli Sanchez^IV

^IPhysician, Specialist in Dermatology. Former-resident, Service of Dermatology, Complexo Hospitalar Padre Bento – Guarulhos (SP), Brazil

^IIDermatologist, assistant physician, Service of Dermatology, Complexo Hospitalar Padre Bento – Guarulhos (SP), Brazil

^IIIPhysician, Pathologist, Service of Dermatology, Complexo Hospitalar Padre Bento – Guarulhos (SP), Brazil

^IVAssistant physician, Service of Dermatology, Complexo Hospitalar Padre Bento de Guarulhos – Guarulhos (SP). Master in Sciences, Medical School, Universidade de São Paulo (USP) – São Paulo (SP), Brazil

^{Mailing Address}

ABSTRACT

Sarcoidosis is a chronic idiopathic disorder characterized by the accumulation of mononuclear phagocytes with the formation of noncaseating granulomas in multiple organs. Cutaneous involvement occurs in 20-25% of cases and specific and nonspecific lesions are observed. We reported a rare case of umbilicated papular sarcoidosis with pulmonary involvement.

Keywords: GRANULOMA; SKIN; SARCOIDOSIS

HISTORY OF THE DISEASE

Female 58-year-old patient, with arthralgia and flat erythematous-violet infiltrate papules, some with central umbilication, distributed on the limbs (Figure 1), chest, abdomen, back and face. The histology resultant from biopsy of the right thigh lesion was nodular dermal granulomatous reaction, comprising well formed granulomas that contained histiocytes with broad and vacuolated cytoplasm. The absence of lymphocytarian halo was an unexpected finding, because it would characterize a complete granuloma (Figure 2). BAAR and fungal investigations using techniques Ziehl-Nielsen and PAS were negative. Culture for fungi and mycobacteria from the biopsied material did not show microorganism growth. Intradermoreactions (Montenegro and Mantoux reactions) and elbow and knee bacilloscopy were negative. Tests of liver and renal functions, glucose levels, complete blood count, electrolytes, electrocardiogram, and hand X-ray were within the normal range. Chest X-ray showed diffuse micronodular reticular infiltrate in both lungs, bilateral hilar thickness and calcified plaque of aortic button (Figure 3). Heart and ophthalmological assessments were normal. Three samples of sputum to test BAAR were all negative.

COMMENTS

Umbilicated Papulous Sarcoidosis

Sarcoidosis is a granulomatous multisystemic disease of unknown etiology, described in all races, genders and age ranges. Multiple organs may be involved, including the skin, lymph nodes, liver, spleen, eyes, parotids, central nervous system, bones, joints, heart and, more frequently, the lungs ^1,2.

When it affects Caucasians, it tends to be an asymptomatic disease with more favorable prognosis ³.

One third of the patients may present nonspecific symptoms such as fever, malaise, fatigue and weight loss ³. In 25% of the cases, there is skin involvement, especially in females.

The most common skin lesion, despite being nonspecific, is nodous erythema ².

Specific cutaneous lesions of sarcoidosis are polymorphic (infiltrated plaques, macular-papular eruption, subcutaneous nodules and scars)², which may mimic other dermatoses. The most typical one is lupus pernio Besnier, which affects predominantly Black descendants and has significant association with upper airways and bone involvement. Macular and papular lesions are less related with systemic diseases and have better prognosis. The worst prognosis and increased risk of systemic impairment are observed in annular and plaques lesions ^1,2.

Even though there may be skin lesions without systemic involvement, patients with sarcoidosis should undergo detailed clinical history and physical examination, emphasizing the lungs, eyes, liver and heart ¹. Complementary tests such as complete blood count, intradermoreactions, chest and hand X-rays and calcium dosing should be carried out. Serum dosage of angiotensin converting enzyme have low specificity and limited value ⁴.

The key for histopathological diagnosis is the presence of noncaseating granuloma, comprising mononuclear inflammatory cells ad multinucleated giant cells ⁴.

In Brazil, granulomatous infectious diseases should be ruled out to confirm the diagnosis of sarcoidosis.

The treatment of choice is the use of topical or systemic glucocorticoids. Therapeutic alternatives include oral retinoid, allopurinol, talidomide, azathioprine, methotrexate, chloroquine, PUVA or UVA, levamizole, and even plastic surgery on disfiguring lesions ¹.

Our patient had papulous lesions, which are specific but not infrequent ². Despite the fact that papulous sarcoidosis is less associated with systemic diseases ¹, our patient had pulmonary impairment. It is important to point out that there are few reports of sarcoidosis with umbilicated lesions, stressing the rarity of the case ⁵. The favorable therapeutic response with systemic corticoid confirms the literature data, which supports the use of corticoid therapy in cases of disseminated cutaneous cases with systemic involvement ^3,4.

REFERENCES

Conflict of interest: None

Financial funding: None

How to cite this article: Pereira ESP, Vidigal MR, Tebcherani AJ, Sanchez APG. Caso para diagnóstico. Sarcoidose papulosa umbilicada. An Bras Dermatol. 2009;84(5):543-5.

• 1. Young RJ, Gilson RT, Yanase D, Elston DM. Cutaneous sarcoidosis. Int J Dermatol. 2001;40:249-53
• 2. Yanardag H, Pamuk ON, Karayel T. Cutaneous involvement in sarcoidosis: analysis of the features in 170 patients. Respir Med. 2003;97:978-82
• 3. English JC 3rd, Patel PJ, Greer KE. Sarcoidosis. J Am Acad Dermatol. 2001;44:725-46
• 4. Newman LS, Rose CS, Maier LA. Sarcoidosis. N Engl J Med. 1997;336:1224-34
• 5. Kashyap S, Kumar M, Thami GP, Saini V. Umbilicated papular sarcoidosis. Clin Exp Dermatol. 1996;21:395-6

Publication Dates

• Publication in this collection
  07 Jan 2010
• Date of issue
  Oct 2009

History

• Accepted
  08 Jan 2009
• Received
  15 May 2008