SkIndia Quiz 31: Erythematous plaque over the chin.

A 35-year-old female presented with an asymptomatic, erythematous plaque over the chin since five months. There was no history of cough, weight loss, fever or sensory deficit. Cutaneous examination revealed a well-defined, oval erythematous plaque on left side of chin 2 cm × 1.5 cm in size with a depression in the center of chin [Figure 1]. Sensations were intact and there was neither nerve thickening nor tenderness. Diascopy revealed apple jelly nodules. Routine blood investigations were normal except for raised erythrocyte sedimentation rate (90 mm in first hour). Mantoux test was strongly positive (18 mm × 16 mm). Chest skiagram and ultrasonography of the abdomen were normal. Serum calcium, angiotensin converting enzyme and serum globulins were normal. Slit skin smear taken from the plaque for acid fast bacilli was negative. Lesional tissue for culture on Lowenstein-Jensen's medium and for mycobacterial DNA was negative. Skin biopsy showed epidermal atrophy and dermis showed multiple granulomas containing lymphocytes, histiocytes and epitheloid cells along with a few Langhan's type of multinucleated giant cells [Figures 2–4] and intact reticulin network [Figure 5]. No caseation was seen. Fite-Faraco stain for acid fast bacilli was negative.

Figure 1

Well defined, oval and erythematous plaque on the left side of chin with depression of chin

Figure 2

Histopathology scanner view with H and E stain showing atrophy of the epidermis. Dermis shows multiple granulomas

Figure 4

Histopathology ×40 H and E stain showing Langhans giant cell in the epitheloid cell granuloma with surrounding lymphocytes

Figure 5

Histopathology ×40 showing reticulin fibers around the granulomas

Histopathology ×10 H and E stain. Showing atrophy of epidermis. Dermis shows multiple granulomas containing lymphocytes, histiocytes and epitheloid cells along with few Langhan's type of multinucleated giant cells

ANSWER

Cutaneous sarcoidosis.

DISCUSSION

Histopathologically, sarcoidosis is characterized by non caseating epitheloid cell granulomas distributed mostly in dermis with few lymphoid cells at the periphery of the granulomas giving the appearance of naked granuloma, whereas granulomas are close to epidermis in lupus vulgaris and the lymphocytic infiltrate is distributed throughout and around the granuloma. Moreover, giant cells may be seen in both the conditions, however, asteroid bodies and Schumann bodies may be seen in the giant cells in sarcoidosis and reticulin network around the granuloma is intact in sarcoidosis whereas it is lost in lupus vulgaris. Leprosy can be differentiated by the presence of granulomas around nerve twigs along with abundant lymphocytic infiltration.

Sarcoidosis means flesh like condition and is a multisystem inflammatory disease of unknown etiology affecting various organs.[1] Although the lungs are the most common site of inflammation, it can involve other organs such as the skin, lymphnodes, eyes, liver, spleen, heart, nervous system, kidneys, and salivary glands.

Specific cutaneous manifestations of sarcoidosis include plaques, lupus pernio, micropapular rash, subcutaneous nodules, and scars.[2] Plaques are the most frequently encountered specific cutaneous manifestation of sarcoidosis and they are usually indurated and reddish brown in color and may extend centrifugally, leaving behind mild atrophy. Face, scalp, and limbs are the sites of predilection. The most common non specific lesion is erythema nodosum.[3] Treatment of sarcoidosis depends upon the extent of the disease. Topical and intralesional steroids are indicated for localized cutaneous sarcoidosis whereas systemic agents are used in widespread disease. The various systemic drugs in vogue are systemic steroids, hydroxychloroquine, methotrexate, azathioprine, infliximab, minocycline, doxycycline, fumaric acid esters, CO2 laser, and ultraviolet phototherapy.