The superficial fibramatoses are a rare group of mainly benign disorders that clinically manifest as slow progressive dermal hypertrophies. This bewildering group of dermal matrix proliferation is composed of fibrous tissue, fibroblastic cells or spindle stromal cells with varying degrees of cellularity. Skin lesions are usually solitary or localized to a specific site of predilection. This is a case of a 15-year-old Filipino boy, presenting with asymptomatic firm, skin colored to erythematous papules and nodules on his limbs and nose. The lesions slowly worsened, resulting in flexion deformity and limited range of motion of both hands.

Human ; Male ; Adolescent ; Administration, Cutaneous ; Fibroblasts ; Hypertrophy ; Nose ; Range Of Motion, Articular ; Skin ; Skin Diseases ; Stromal Cells ; ; Dupuytren Contracture

A 21 year old Filipino male presented with swelling of the second right hand digit unresponsive to antibiotics. Amputation revealed chronic inflammation and negative cultures. He developed sterile conjunctivitis and a generalized eruption of asymptomatic red papules and nodules. First skin biopsy revealed a diffuse infiltrate of epithelioid and foamy histiocytes, diagnosed as "juvenile xanthogranuloma." The second biopsy revealed large histiocytes with a "ground-glass" eosinophilic cytoplasm, multinucleated giant cells, and mixed cell infiltrate. Immunohistochemistry showed histiocytes staining with (+)S100 and (+)CD68, and (-)CD1a. Final diagnosis was "multicentric reticulohistocytosis." Despite treatment with oral prednisone, methotrexate and alendronate, lesions were progressive.
CONCLUSION: This fascinating case manifests with overlapping features of both juvenile xanthogranuloma and multicentric reticulohistiocytosis, and lead the authors to suggest considering the spectrum of diseases called the non-Langerhans cells histiocytosis when presented with a generalized nodular eruption.

Human ; Male ; Young Adult ; Antigens, Cd1 ; Biopsy ; Conjunctivitis ; Exanthema ; Giant Cells ; Histiocytes ; Histiocytosis, Non-langerhans-cell ; Inflammation ; Methotrexate

INTRODUCTION: Dermatomyositis is a rare idiopathic inflammatory myopathy with characteristic skin manifestations and proximal muscular weakness. In 30 percent of classic dermatomyositis, skin findings precede muscle weakness. Since the initial skin lesions are not always highly characteristic of dermatomyositis, there may be a delay in diagnosis and treatment. CASE REPORT: This is a case of a 36-year-old Filipino female who initially presented with erythematous patches and plaques on the face, arms, and thighs. One week later, she developed multiple, well-defined, erythematous to violaceous, edematous, tender patches and plaques on the face, V of the neck, upper back, proximal extremities and buttocks. Seven weeks later, she developed proximal muscle weakness described as difficulty in raising her arms and difficulty in standing up from a sitting position. Histopathology was consistent with dermatomyositis. SGPT, C3, ANA, and anti-ds-DNA were normal. SGOT and creatine kinase were 5 and 15 times the normal value, respectively. She was treated with prednisone from the first week of illness and hydroxychloroquine from the fifth week of illness. Her condition greatly improved with no progression of the disease for the succeeding 3 years. CONCLUSION Even in the absence of muscle weakness, there should be a high index of suspicion for dermatomyositis in patients with confluent, erythematous patches and plaques on the face, trunk and proximal extremities. Adequate work-up and clinical monitoring will pave the way for early diagnosis and consequently early treatment and a better patient outcome.
Dermatomyositis

INTRODUCTION: Cutaneous mastocytosis is the accumulation of mast cells in the skin. Diffuse cutaneous mastocytosis is a rare variant accounting for only 1.74% of al mastocytosis cases reported. Ninety percent of cases are seen in children presenting with multiple erythematous to yellow-tan papules and plaques with leathery texture. The pathogenesis is in the structure and activity of kit receptor expressed on mast cells, melanocytes and other cells. CASE SUMMARY: This is a female neonate born to an 18 year old mother, G1P1 via vaginal delivery at 37 weeks age of gestation. Patient presented with a generalized involvement of multiple, well defined, indurated, leathery, brown papules and confluent plaques. Darier sign was positive. Histopathological examination revealed diffuse involvement of the dermis with mast cells. Giemsa stain was positive. Patient was diagnosed both clinically and histopathologically with diffuse cutaneous mastocytosis without systemic involvement. She was treated with H1 antihistamines and topical glucocorticoids. CONCLUSION Diffuse cutaneous mastocytosis can be diagnosed both clinically and histopathologically. Treatment is mostly symptomatic. It is always prudent to inform co-managing physicians, the patient, and their families of potential mast cell degranulating stimuli and to watch out for signs and symptoms for systemic involvement.

INTRODUCTION: The nails have many functions for daily life including protection of the digits against different kinds of trauma. The nail apparatus is composed of multiple kinds of keratins. Defects in keratin may produce several genodermatoses, which include Pachyonychia congenita. This is a rare genodermatosis that is either automosal dominant or sporadic. The usual involved keratins are KRT6a, KRT6b, KRT6C, Kl 6 and Kl 7. Keratin defects may manifest on the hair, mucosa, skin and nails. Different presentations of each patient depends on the involved mutated keratins, In rare cases, nail dystrophy may be the only manifestation of the keratin defect. CASE SUMMARY: A 23 -year-old female presented with a 13-year-history of nail thickening on both fingernails and toenails with no other associated symptoms. She denied oral and skin lesions. She had been unsuccessfully treated with both oral and topical antifungals by several dermatologists. No family members have any similar conditions. She admitted to having hoarseness. On physical examination, nails of both hands and feet marked subungual hyperkeratosis, yellowish to brown- gray discoloration and hypercurvature and rough surface of all 20 nails. The histopathology report revealed marked compact hyperkeratosis, hypergranulosis, papillomatosis and acanthosis. Fungal culture and PAS stain revealed no fungal elements. Patient responded well to nail avulsion and good nail regrowth was noted. She plans treatment of all nails with avulsion. CONCLUSION Thorough history, physical examination and histology may help confirm the diagnosis of Pachyonychia congenita, which at times may be mistaken for other nail problems. Treatment is based upon the clinical presentation of the patient. Prognosis for life is good but complications such as infections should be properly addressed.

INTRODUCTION Alopecia areata incognita is a rare form of alopecia areata which was first reported in 1987. The prevalence of this disease is unknown but it is more common in women. The usual presentation of alopecia areata incognita is acute, diffuse hair thinning. In most cases, it lacks the typical alopetic patches seen in alopecia areata. It may resemble telogen effluvium and androgenetic alopecia. The prognosis of this disease is favorable and recovery is rapid and spontaneous. Case: A 19- year-old Filipino female presents with a two-month history of alopecia areata incognita. She initially had a solitary round patch of hair loss on the scalp with proximally tapered hair, rapidly evolving into diffuse hair thinning. CBC, TFTS, FBS, HBA 1 c, ANA and VDRL were unremarkable. Histopathology demonstrated dense peribulbar lymphocytic infiltrate, miniaturized hair and increased catagen hair consistent with alopecia areata. There was gradual hair growth after treatment with minoxidil 5% lotion and topical betamethasone dipropionate 0.05% lotion.

BACKGROUND: The novel COVID-19 (Coronavirus Disease 19) predisposes the general population to a high risk of infection. The 106 million population of the Philippines would be considered an at-risk group due to the high density of the populace in cities. As the situation in each country differs during this era of the COVID-19 pandemic, this paper aims to give practical recommendations for safe procedural dermatology practice in the Philippine setting after the lifting of the government-mandated quarantine. METHODS: An online survey was conducted among Philippine Dermatological Society members. The survey was sent electronically on March 22, 2020. RESULTS: A total of 466 or 42% of the PDS’s 1100 current members replied to the survey. The top 10 procedures performed among the respondents are: 1. Electrocautery (N=437, 94.38%), 2. Chemical peeling (N=422, 91.13%), 3. Laser & energy based device treatment (N= 341, 73.65%), 4. Botulinum toxin injection (N=323, 69.76%), 5. Excision (N=263, 56.80%), 6. Acne surgery (N=176, 38.01%), 7. Injectable Filler (N=171, 36.93%), 8. Cryotherapy (N=145, 31.32%), 9. Platelet rich plasma injection (N=111, 23.97%) and 10. Scar revision (N=85, 18.36%). The majority of the respondents have access to personal protective equipment (PPE) such as surgical masks (N=457, 98.7%), face shields (57.67%), goggles (46.00%), protective gown (42.76%) and bonnets (32.83%). Before the government quarantine, the majority (N=375, 81.17%) of respondents see patients on a firstcome, first-serve system. Only 18.83% (N=87) see patients only by appointment. Regarding teledermatology, most respondents answered that they would advise patients to do digital consultation with only a minority responding they would not consider doing teledermatology. CONCLUSIONS: In the Philippine setting, the best ways to prevent COVID infection inthe procedural dermatology setting include: 1. Education of staff and patients on proper exposure prevention and sanitation measures. 2. Ensuring the correct usage of PPE. 3. Ensuring physical distancing and reducing patient wait times by scheduling visits on an appointment basis. 4. Sufficient protocols must be made for sanitation before and after each patient visit. 5. Teledermatology in pre-procedure consults and post-procedure follow-ups would reduce the risk of COVID-19 transmission for both patient and physician.
Leprostatic Agents ; Drug Therapy, Combination ; Leprosy ; Recurrence ; Chronic Disease

Hypertrophic lichen planus (HLP) is a papulosquamous eruption presenting with extremely pruritic hyperkeratotic flat-topped papules, plaques, and nodules. This is a case of 38-year-old male who presented with a 2-month history of generalized erythematous-to-hyperpigmented papules, patches, and plaques topped with white-to-gray oyster shell-like scales on a background of hyperpigmented macules and patches. There was no involvement of the conjunctival, otic, oral, and genital mucosae, and palmar and plantar aspects of the hands and feet. Dermoscopy showed reticular pearly white structures corresponding to the Wickham striae, comedo-like openings, blue-gray dots, brownish-black dots, and scales. Histopathologic examination revealed marked compact hyperkeratosis, wedge-shaped hypergranulosis, irregular saw-toothed epidermal acanthosis, scattered dyskeratotic keratinocytes, and superficial perivascular lichenoid infiltrate of lymphocytes, histiocytes, and melanophages. The patient was managed as a case of HLP. He was started on methotrexate 10 mg per week, bath psoralen photochemotherapy (PUVA) three times a week, betamethasone valerate 1mg/g cream twice a day for 2 weeks alternating with tacrolimus 0.1% ointment twice a day for another 2 weeks, 10% lactic acid, emollients, and sunscreen. After 6 months of treatment, there was almost 80% improvement of lesions and relief of pruritus.
Methotrexate

INTRODUCTION: lgA pemphigus is a rare, chronic, relapsing, benign group of autoimmune intraepidermal blistering dermatosis with an unknown etiology. It is characterized by significantly pruritic, vesiculopustular lesions that occur mainly on the trunk and proximal extremities. Histopathologic and immunofluorescence studies show intraepidermal blisters and deposition of immunoglobulin A in the intercellular spaces of the epidermis, respectively. CASE REPORT To our knowledge, we present the first reported pediatric case of lgA pemphigus, intraepidermal neutrophilic type, in an 8-year old Filipino female with a 2-year history of generalized papules and flaccid pustules, some forming an annular pattern. Diagnosis was confirmed by histopathology and direct immunofluorescence. Enzyme-linked immunosorbent assay for Desmoglein 1 was negative. Complete clearance of lesions was achieved with dapsone, colchicine and prednisone.

Introduction: Chronic bullous disease of childhood (CBDC) is a rare immune-mediated subepidermal vesiculobullous eruption, characterized by linear IgA deposition along the basement membrane zone of the skin. Although mostly idiopathic, CBDC may be triggered by factors such as infection, and drugs. Clinical and immunohistopathological features of drug-induced cases are heterogeneous and indistinguishable from the idiopathic form. Case report: A two-year-old Filipino male presented with pruritic vesicles and bullae on the back several days after finishing a course of cefuroxime, and cefaclor. Examination revealed multiple tense vesicles and bullae, some coalescing into a rosette pattern with central crusts on the perioral, scalp, neck, back, perineal, and perianal areas. Histopathology showed a subepidermal split with neutrophilic and eosinophilic infiltrates. Direct immunofluorescence revealed strong linear deposition of IgA, and granular deposits of C3 and IgM at the basement membrane zone, thus confirming the di- agnosis of CBDC. Dapsone at 2mg/kg/day was started, with oral prednisolone (1.3mg/kg/day), and cloxacillin syrup (40mg/kg/day). Topical care with betamethasone dipropionate and mupirocin ointment was included. After eight weeks, patient showed significant im- provement with few vesicles and resolved lesions healing with post-inflammatory hyperpigmentation. Conclusion We report a case of a two-year-old male presenting with vesiculobullous lesions after a course of cefuroxime, and cefaclor. As both were given and withdrawn in a period of close proximity, it is difficult to determine the probable culprit drug. Spontaneous resolution upon withdrawal of the suspected drug is variable. Systemic therapy such as dapsone may be necessary for treatment.
Linear IgA Bullous Dermatosis ; Cefaclor ; Cefuroxime