1.Clinical, dermoscopy and histopathological findings in a case of lipoid proteinosis
Miguel Remigio T. Maralit ; Johannes F. Dayrit ; Emmerson Gale S. Vista ; Abigael T. Villanueva
Journal of the Philippine Dermatological Society 2019;28(1):51-53
Introduction:
Lipoid proteinosis is a rare autosomal recessive genodermatosis with only approximately 300 cases
reported worldwide. This condition is due to the loss of function mutation of the ECM1 gene which encodes for
extracellular matrix protein 1 (ECM1), a secretory protein that promotes angiogenesis and is a negative regulator of
endochondral bone formation.
Case summary:
We report a case of a 23-year-old male who presented with chronic recurrent verrucous and waxy
lesions on the face and body, diffuse alopecia of the scalp, and hoarseness of voice. Lesions heal leaving scars.
Dermoscopy of the eyelid margins show white to yellowish clods with accentuated brownish halo. Lesions on the
nape show multiple follicular symmetric whitish-yellow clods with brownish halo and well-defined borders. Lesions
on the elbows show a reticular pattern with white clods and diffusely distributed brown dots. Histopathology shows
basket-woven stratum corneum, spongiosis of the epidermis with vacuolar alteration, and formation of clefts in the
basal cell layer. The papillary dermis shows hyaline material which also surrounds the blood vessel and the adnexal
structures. There is mild superficial and mid dermal perivascular inflammatory infiltrate of lymphocytes. PAS
highlights eosinophilic material in the papillary dermis which also surrounds the blood vessels and the adnexal
structures. Alcian blue staining reveals positive staining for hyaline deposits.
Conclusion
This report highlights the importance to recognize the clinical, dermoscopic and histopathological
findings of lipoid proteinosis.
Lipoid Proteinosis of Urbach and Wiethe
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Dermoscopy
2.Rhinophyma on hemangioma: A diagnostic conundrum
Danica-Grace R. Tungol ; Miguel Remigio T. Maralit ; Johannes F. Dayrit ;
Journal of the Philippine Dermatological Society 2020;29(1):93-95
INTRODUCTION: Rhinophyma, aside from persistent centrofacial redness is a major diagnostic criteria for rosacea. Phyma may be mistaken for hypertrophy of tissue due to an underlying hemangioma.
CASE REPORT: A 35-year-old female presented with few erythematous papules on the face and nose 19 years prior to consult. Lesions evolved into multiple erythematous nodules on nose and was mistaken for tissue hypertrophy due to an adjacent congenital hemangioma. Her hemangioma was treated with pulsed dye laser 16 years prior with noted decrease in size and erythema. Recently she noticed enlargement of her nose with persistent redness.
She presented with multiple firm, thick irregularly shaped erythematous nodules with prominent pilosebaceous pores and telangiectasia on the nose. Skin punch biopsy was done which revealed hypertrophy and lysis of sebaceous lobules with a moderately dense inflammatory infiltrate of lymphocytes. Histopathological diagnosis was rhinophyma. Patient was treated with low dose isotretinoin (0.20 mkd) with marked flattening of lesions in just one month.
CONCLUSION: Distinguishing phyma from tissue hypertrophy caused by hemangioma poses as a diagnostic challenge. Careful dermatological examination and histopathological findings will aid in correct diagnosis. Low dose oral isotretinoin is an effective treatment for rhinophyma.
Rhinophyma
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Isotretinoin
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Rosacea
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Hemangioma
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Hydrozoa