A young patient has white thickening of buccal mucosa since birth and it also occurs in nose and esophagus. Most likely diagnosis is:

 # A young patient has white thickening of buccal mucosa since birth and it also occurs in nose and esophagus. Most likely diagnosis is: 

a. accumulation of fordyces granules 

b. chronic hyperplastic candidiasis 

c. white sponge nevus 

d. porokeratosis 

The correct answer is C. White sponge nevus. 

This mucosal abnormality is congenital in many instances. In other cases, it does not appear until

infancy, childhood, or even adolescence, by which time it has generally reached the full extent of its severity. The oral lesions may be widespread, often involving the cheeks, palate, gingiva, floor of the mouth, and portions of the tongue. The mucosa appears thickened and folded or corrugated with a soft or spongy texture and a peculiar white opalescent hue. There is sometimes a minimal amount of folding present. Ragged white areas may also be present which can be removed sometimes by gentle rubbing without any ensuing bleeding. 

Organ involvement of SLE is in:

 # Organ involvement of SLE is in:

a. kidney and Lungs 

b. kidney and heart 

c. lungs and heart 

d. heart and spleen 

The correct answer is B. Kidney and heart.

Systemic lupus erythematosus is a serious cutaneous-systemic disorder which characteristically manifests repeated remissions and exacerbations. This disease has its peak age of onset at about 30 years in females but about 40 years in males. The disease may occur in childhood as reported by Jacobs. Prevalence rates are higher in females than in males. A female-to-male ratio of approximately 2 : 1 occurs before puberty, and a ratio of 4 : 1 occurs after puberty.

The generalized manifestations of the systemic disease are referable to the involvement of various organs, including the kidney and heart. In the kidney, fibrinoid thickening of glomerular capillaries occurs, producing the characteristic ‘wire loops,’ which may be sufficient to result in renal insufficiency. The heart may suffer from an atypical endocarditis involving the valves, as well as fibrinoid degeneration of the epicardium and myocardium. The widespread tissue involvement and the nature of the lesions have led to the inclusion of this disease in that group known as the ‘collagen diseases,’ which also includes rheumatic fever, rheumatoid arthritis, polyarteritis nodosa, scleroderma and dermatomyositis.

Prodromal symptom of OSMF:

 # Prodromal symptom of OSMF:

a. hypersalivation 

b. dryness of mouth 

c. both of the above 

d. none of the above 

The correct answer is C. Both of the above.

Prodromal Symptoms (Early OSF):

 This includes a burning sensation in the mouth when consuming spicy food, appearance of blisters especially on the palate, ulcerations or recurrent generalized inflammation of the oral mucosa,

excessive salivation, defective gustatory sensation and dryness of the mouth. There are periods of exacerbation manifested by the appearance of small vesicles in the cheek and palate. The intervals between such exacerbation vary from three months to one year. Focal vascular dilatations manifest

clinically as petechiae in the early stages of the disease. This may be part of a vascular response due to hypersensitivity of the oral mucosa towards some external irritant like arecanut products. Petechiae are observed in about 22% of OSF cases (Rajendran, 1994), mostly on the tongue followed by the labial and buccal mucosa with no sign of blood dyscrasias or systemic disorders. Pain in areas where submucosal fibrotic bands are developing when palpated is a useful clinical test. Histologically, they revealed a slightly hyperplastic epithelium, sometimes atrophic with numerous dilated and blood-filled capillaries juxtaepithelially. The inflammatory cells seen are mainly lymphocytes, plasma cells and occasional eosinophils. The presence together of large numbers of lymphocytes and fibroblasts as well as plasma cells in moderate numbers, suggests the importance of a sustained lymphocytic infiltration in the maintenance of the tissue reaction in OSF. 

Pathergy test is done in:

# Pathergy test is done in: 

a. bechet syndrome 

b. senear usher syndrome 

c. weber cockayna syndrome 

d. epidermolysis bullosa 

The correct answer is: A. Bechet syndrome.

Pathergy phenomenon has been well known to dermatologists since it was first described in 1937 by Blobner as a state of altered tissue reactivity in response to minor trauma. The pathergy test is a nonspecific hypersensitivity skin reaction induced by needle prick that is performed to look for evidence of this phenomenon. Pathergy lesions are generally manifested clinically by erythematous induration at the location of skin trauma, which may remain as papules or progress to sterile pustules. Although the precise mechanism of pathergy has not yet been entirely elucidated, the skin injury by needle prick in patients exhibiting pathergy is thought to trigger a cutaneous inflammatory response that is exaggerated and more prominent than that seen in normal skin. An increased release of cytokines from cells in the dermis or epidermis is implicated in this aberrant reaction, which results in the perivascular infiltrates that are characteristically observed on histopathologic studies. While pathergy has been reported in numerous diseases, pathergy testing is primarily used in the diagnosis of Behcet Disease (BD).