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

Discoid Lupus Erythematosus (DLE) is the most common form of chronic cutaneous Lupus Erythematosus (LE). Characteristic lesions cause disfigurement impacting quality of life, with 5% of progress into systemic LE (SLE). Prompt diagnosis avoids sequelae such as scarring, recurrence, and malignancy. Local therapy includes sun protection, steroids, and calcineurin inhibitors (CNIs). Antimalarials and surgical or cosmetic interventions are other options.

Human ; Female ; Adult: 25-44 Yrs Old ; Lupus Erythematosus, Cutaneous ; Lupus Erythematosus, Discoid

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

Alopecia areata with ophiasis has a worldwide prevalence of only 0.02%. In the last ten years, only 10 cases have been reported in the Philippines. This variant is often resistant to treatment. Novel therapeutic options are being explored, although these are frequently limited to case reports due to the rarity of the disease. Newer therapies, such as JAKSTAT inhibitors and monoclonal antibodies, show promise as effective options for ophiasis-type alopecia areata.

Human ; Female ; Adult: 25-44 Yrs Old ; Alopecia Areata ; Baricitinib

Bullous pemphigoid (BP) is an autoimmune blistering disorder characterized by tense vesicles and bullae, primarily affecting the elderly. It results from autoantibodies targeting hemidesmosomal proteins BP180 and BP230, leading to subepidermal blistering. BP often presents with widespread pruritic plaques and blisters on flexural surfaces, and mucosal involvement is rare. While BP typically occurs spontaneously, certain medications, such as linagliptin, have been implicated as triggers. Early diagnosis and treatment with systemic corticosteroids or immunosuppressive agents can significantly reduce morbidity.

Human ; Male ; Aged: 65-79 Yrs Old ; Pemphigoid, Bullous

Proteus syndrome (PS) is a mosaic disorder characterized by asymmetric overgrowth of a variety of tissues. Diagnostic criteria established in 1999 emphasized the mosaic distribution of lesions, progressive course, and disproportionate overgrowth. We present a case of proteus syndrome in a 12-year-old Filipino male with 9 year-history of enlargement of the left foot with soft, non-tender mass on the sole with a brain-like surface. Skin punch biopsy of the mass showed cerebriform connective tissue nevi which is pathognomonic of PS.

PS is a very rare disease with prevalence of less than 1 in 1,000,000 live births. Management of PS is extremely challenging, owing to the combination of the individuality of each case, the severity of the disease, and the risks of complications from procedures. A multidisciplinary clinical approach is strongly recommended to obtain the best possible management plans for individual patients.

Human ; Male ; Child: 6-12 Yrs Old ; Proteus Syndrome ; Mosaicism

Kaposi Sarcoma (KS) is a vascular tumor commonly associated with HIV/AIDS. There is unusual presentation of KS in a non-HIV patient, initially diagnosed as small vessel vasculitis. Early recognition and accurate diagnosis are important for the patient’s best management.

A 68-year-old male presented with spontaneous bluish-black patches on his extremities, swelling, pain, and bullae on his toes. Initial workup, including negative ANA and ANCA markers, pointed to small vessel vasculitis, with autoimmune, hematologic, and occlusive diseases considered. Peripheral vascular occlusion was ruled out, and a biopsy showed granulomatous vasculitis. Despite corticosteroid treatment, the lesions worsened. Five months later, the patient developed violaceous papules, plaques, and nodules. A second biopsy confirmed Kaposi Sarcoma (KS) with positive CD34 and HHV-8 stains. Restaining the initial biopsy also revealed KS. Treatment with Doxorubicin was initiated, but the disease progressed, affecting the gastrointestinal system. The patient’s condition deteriorated, and he died from complications of KS.

This case underscores the importance of considering Kaposi Sarcoma in HIV-negative patients with vascular lesions. The initial diagnosis of vasculitis, confirmed by granulomatous changes, delayed the KS diagnosis. Restaining the first biopsy later confirmed the presence of KS from the onset. The extensive skin and gastrointestinal involvement made management with Doxorubicin difficult, leading to a poor outcome.

Human ; Male ; Aged: 65-79 Yrs Old ; Sarcoma, Kaposi ; Tumors ; Neoplasms

For diagnosis of rare conditions, consistent follow-up on the part of the patient as well as a high index of suspicion on the part of the physician is needed. Evaluation of the management should be done in the event that patients do not respond to treatment.

This is a case of a 33 year old female who fifteen months prior, noticed erythematous scaly plaques on her cheeks with mild pruritus. She was treated for psoriasis with Halobetasol ointment and Petroleum Jelly, which had partial resolution. Twelve months prior, she was prescribed Methotrexate, again achieving partial resolution. Biopsy was done suggestive of Folliculotropic Mycosis Fungoides. Methotrexate was increased, and additional medications were prescribed, leading to lesion resolution. However, she was lost to follow-up and experienced worsening symptoms. One month prior, biopsy was repeated and again showed Diffuse Lymphocytic Dermatitis positive for CD3+, CD4+, CD5+, CD8-, CD20-, CD30-, and loss of staining for Pan-T cell markers, CD2- and CD7. She is currently managed with regular sessions of Narrowband UVB.

These rare cases are few but more often than not, they are easily missed and when caught are usually progressed and already difficult to treat. Physicians must be vigilant in treating patients, even if they initially diagnose it to be a commonly seen and easily managed disease. Skin Directed therapy is done with PUVA and NBUVB with complete response in 30-70%3. For prognosis, early stages are favorable with a 94% 5 year survival rate, decreasing to 69% after tumor development.

Human ; Female ; Adult: 25-44 Yrs Old ; Lymphoma, T-cell, Cutaneous ; Tumors ; Neoplasms

This is a case of a 74-year-old female who previously worked as a Metro Manila Aide and presented with a solitary erythematous, well-demarcated mass with hyperkeratosis on the right zygomatic area. It started as a pea-sized erythematous papule three years prior without associated symptoms. The patient self-medicated with Ketoconazole + Clobetasol Propionate cream for five months without improvement. Two months before consultation, the lesion enlarged and developed yellow hyperkeratotic crusts. A biopsy revealed invasive squamous cell carcinoma (SCC). Complete excision with adequate margins was recommended. The patient underwent Mohs Micrographic Surgery and reconstruction with a rotational flap repair. Histopathology of the excised tissue confirmed squamous cell carcinoma. No tumor necrosis or lymphovascular invasion was identified, and all resection margins were clear. Post-surgical management included wound care and medications. The case emphasizes early intervention and histopathological evaluation in managing growths especially in cases where patients have not consulted and self medicated instead.

Human ; Female ; Aged: 65-79 Yrs Old ; Mohs Surgery ; Carcinoma, Squamous Cell ; Tumors ; Neoplasms

Dyshidrotic eczema (DE) and palmoplantar psoriasis (ppPSO) are both conditions presenting with vesicopustules on the palms and soles. While they have differences, distinguishing them can be a challenge in cases complicated with allergic contact dermatitis (ACD).

A 24-year-old male clerk, presented with a one-year history of vesicopustules on the palms and soles, associated with onycholysis and subungual hyperkeratosis. He admits to frequent hand washing and using rubbing alcohol as well as wearing a new pair of rubber shoes. Patch testing (International Standard series) showed +++ Potassium dichromate 0.5%, Carba Mix 3.0%, Thuiram mix 1.0%, Textile dye 6.6% and ++ Formaldehyde 2%. Gram stain was negative and KOH from plantar area showed spores and long-septate hyphae. Dermoscopy showed a bright pink background with erosions, patchy dotted vessels atop with yellow crust, and scales. Histopathology revealed psoriasiform-spongiotic dermatitis with macrovesiculation and eosinophilic spongiosis which was consistent with subacute eczema. He was managed as a case of recalcitrant DE with secondary dermatophytosis and ACD to textile dye and rubber, but still keeping in mind the possibility of eczematized ppPSO. The patient was managed accordingly, with particular emphasis on avoiding allergens. The condition improved, and nail changes resolved with topical medications and a short course of oral corticosteroid and oral anti-fungal thus favoring our initial impression of recalcitrant DE complicated by dermatophytosis and ACD.

This highlights the diagnostic challenge in distinguishing between severe DE and ppPSO in the setting of concomitant ACD, underscoring the importance of a comprehensive evaluation.

Human ; Male ; Young Adult: 19-24 Yrs Old ; Dermatitis, Contact ; Eczema