Netherton syndrome in a 1-year-old Filipino female

@#Netherton syndrome (NS) is an autosomal recessive genodermatosis characterized by cutaneous and systemic complications (recurrent infections, dehydration, and sepsis). This highlights the urgency of making an accurate diagnosis, especially in infants and children. However, it is important to note that the recognition of NS is usually delayed due to its rarity and similarity to cutaneous disorders with atopiform, erythrodermic, and ichthyosiform presentations. We report a case of a 1-year-old female who was previously diagnosed with a case of infantile psoriasis and was subsequently treated with topical emollients. However, after months of surveillance, the patient developed feeding problems, failure to thrive, recurrent diarrhea, upper respiratory tract, and gastrointestinal infection, leading to repeated hospitalizations. The patient then underwent further clinical examinations and laboratory analysis, which revealed abnormal hair shaft findings, elevated immunoglobulin (Ig) E levels, and normal chromosomal analysis. Multispecialty referrals with other services were done to address the current problems and ensure holistic care for the patient. On her last admission, the patient was given three doses of intravenous Ig therapy with noted improvement in lesion presentation without any systemic symptoms.

Scrofuloderma and tuberculous gumma in a young Filipino adult: A rare presentation of multifocal tuberculosis

@#<p style="text-align: justify;"><strong>INTRODUCTION:</strong> Cutaneous involvement is relatively uncommon representing a small fraction (1-2%) of the localizations of extrapulmonary tuberculosis. Cutaneous TB presents with several clinical forms, wherein one of the most common is scrofuloderma resulting from the direct extension of a tuberculous focus from a deeper structure such as the lymph node into the overlying skin. Tuberculous gumma is a rare form which occurs due to hematogenous spread of the TB bacilli. Although presenting with a wide clinical spectrum, it is believed that the association of different morphologies as well as numerous lesions and sites of cutaneous TB in a same patient is very rare.</p><p style="text-align: justify;"><strong>CASE REPORT:</strong> This is a case of a 20-year-old Filipino male presented with a fi ve-month history of several progressive cutaneous lesions initially presenting as subcutaneous nodules evolving into well-demarcated suppurative painless ulcers which were unresponsive to topical antibiotic. Skin punch biopsy from the medial malleolar area of the right foot revealed dilated blood vessels with a diffuse inflammatory infiltrate of lymphocytes, histiocytes, and few multinucleated giant cells. Clinical and laboratory findings were consistent with cutaneous tuberculosis. Patient was started on anti-Koch's treatment regimen and presented an excellent response to treatment showing resolution of the skin lesions on the neck and forearms and notable regression of the lesions on the right foot within four (4) months.</p><p style="text-align: justify;"><strong>CONCLUSION:</strong> This case serves as a reminder that cutaneous tuberculosis can manifest with a wide spectrum of clinical presentation which can mimic diverse dermatological conditions and may present with high rates of negative or equivocal diagnostic testing results. This report highlights the importance of a high index of suspicion in the timely diagnosis and management of tuberculosis in countries wherein tuberculosis remains a significant health burden such as the Philippines.</p><p style="text-align: justify;"><strong>KEYWORDS:</strong> Cutaneous tuberculosis, Scrofuloderma, Tuberculous gumma, Metastatic tuberculous abscess</p>

Relapse rate among smear-positive leprosy cases after 12 blister packs and 24 blister packs of multibacillary drug therapy in a tertiary hospital

@#BACKGROUND: Several trials on relapse rates on duration of multibacillary regimens have varying results. OBJECTIVE: To compare the relapse rate among smear-positive Leprosy patients receiving 12 blister packs of multibacillary drug therapy and 24 blister packs of multibacillary drug therapy. METHOD: A review of records of smear positive Leprosy patients seen from 2002 to 2006 was done. Demographic, clinical and therapeutic data were collected. Bacteriologic index was determined from Leprosy Laboratory records. RESULT: A total of 391 patients were found to have complete records for review and analysis. Relapse rate was 11.9%(28) for patients who received 12 blister packs and 1.91%(3) for patients who received 24 blister packs and the difference was found to be statistically significant (p<0.01). Distribution of relapse was statistically significant according to age (p<0.01), bacteriologic index (p<0.01) and clinical spectrum (p<0.01). CONCLUSION: Relapse rates shown among smear positive leprosy patients receiving 12 blister packs vs. those receiving 24 blister packs was statistically higher which differs from previously published studies. Significant predictors were clinical spectrum, bacteriologic index of >3.5, and >4 and number of blister packs.

Molluscum contagiosum-like lesions: A telltale sign of disseminated cryptococcosis

Introduction@#Cryptococcosis is an opportunistic fungal infection caused by Cryptococcus neoformans. This systemic fungal infection affects 6 to 13% of patients with acquired immunodeficiency syndrome (AIDS). Only 10% of patients with this condition develop cutaneous manifestations.@*Case Summary@#A 20-year-old male, newly diagnosed case of HIV (Human Immunodeficiency Virus), presented with multiple skin-colored molluscum contagiosum-like umbilicated papules on the face, neck and both arms. Lesions increased in number rapidly and were associated with respiratory distress. Histopathologic examination revealed findings of opportunistic fungal infection suggestive of cryptococcosis. Serum Cryptococcal Antigen Latex Agglutination System (CALAS) test was also positive. Patient was then diagnosed as a case of cryptococcosis and was a candidate for intravenous amphotericin B. However, before the planned medication was given, the patient experienced severe respiratory distress and expired several hours later.@*Conclusion@#Molluscum-like skin lesions may be a telltale sign of a disseminated opportunistic fungal infection, including Cryptococcosis. Early diagnosis followed by prompt and aggresive treatment would improve outcome and survival of the patient.

Clinical, dermoscopy and histopathological findings in a case of lipoid proteinosis

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.

Cross-sectional survey on the knowledge, attitudes and practices of Philippine Dermatological Society members related to HIV/AIDS

Background@#Human Immunodeficiency Virus (HIV) infection/Acquired Immunodeficiency Syndrome (AIDS) remains a major challenge worldwide. Physicians have a crucial role in the struggle against HIV/AIDS and must be equipped with proper knowledge, attitude and practices (KAP). No study has been done to assess the KAP of dermatologists related to HIV-AIDS.@*Objective@#The study’s objective was to determine the KAP of Philippine Dermatological Society (PDS) members related to HIV/AIDS.@*Methods@#A cross-sectional study was done among PDS members from February to November 2017 utilizing a questionnaire consisting of socio-demographic questions and KAP questions related to HIV.@*Results@#Out of 210 participants, 71.43% show good working knowledge, 60.95% show negative attitudes, 55.71% show bad practices related to HIV/AIDS. Members aged 29 to 37 years old, having worked as dermatologists for 1 to 8 years, and having worked in the health care sector for 1 to 8 years have better working knowledge on HIV/AIDS. Although with lesser duration of work as dermatologist, these members have more positive attitudes towards HIV/AIDS. These findings may be attributed to greater exposure to HIV cases and better ability to access information by internet among younger dermatologists. Gender is not significantly associated with KAP related to HIV/AIDS. Practice related to HIV/AIDS is not significantly associated with any demographic variables. Bad working knowledge related to HIV/AIDS is associated with negative attitude and bad practices. This can be attributed to insufficient training in the field of HIV/AIDS.@*Conclusion@#Majority of PDS members show negative attitudes, bad practices, but good working knowledge related to HIV/AIDS.

Updates on sexually transmitted infections in the Philippines

@#Sexually transmitted infections (STIs) remain a public health problem worldwide, with more than 1 million people acquiring an STI every day. In the Philippines, the total number of people with STIs other than human immunodeficiency virus (HIV) infection is not known. Hence, the Philippine Dermatological Society (PDS) has taken the initiative to document STI cases in its institutions since 2011. Syphilis, human papillomavirus (HPV), herpes simplex virus (HSV) and HIV infections are some of the common STIs in our country. A total of 665 syphilis patients, 2,053 HPV-infected patients and 977 patients with herpes have been seen and treated in PDS institutions throughout the country from 2011 to 2018. The number of HIV cases on the other hand, have already reached 58,181 from January 1984 to August 2018. In August 2018 alone, 1,047 new cases of HIV have been reported to the HIV/AIDS & ART Registry of the Philip- pines (HARP). The rates of STI infections like HIV, syphilis, HPV and herpes may be on the rise but clinicians can have a significant impact on this trend. With effective counselling, early and accurate detection, diagnosis and treatment, the threat and burden of STIs can be greatly reduced.