Human ; Male ; Female ; Dermoscopy ; Dermatology ; Melanoma

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

Morphea is a rare, chronic inflammatory condition that affects the skin and subcutaneous tissues with an unclear etiology. Genetic predisposition, autoimmune dysregulation, and environmental factors play a role in its pathogenesis. It affects both adults and children and presents as erythematous patches or plaques that develop sclerotic centers with a violaceous border. Early diagnosis and treatment are crucial to minimize damage and physical sequelae.

We present here a 30-year old female who presented with a solitary, violet-hue in color, indurated plaque on her left forearm after wearing a metal smartwatch for 4 months. She experienced on and off episodes of overheating from the watch but continued wearing it. There was no associated pruritus, tenderness, or loss of sensation. Anti-dsDNA showed a borderline positive result. Vitamin D levels were below the lower limit revealing a severe Vitamin D deficiency. Dermoscopy revealed fibrotic beams, branching vessels and an erythematous to pink background. Histopathologic analysis showed superficial and deep perivascular and periadnexal infiltrates of lymphocytes and plasma cells with compact collagen bundles and notable loss of periadnexal fat. The patient was started on topical halobetasol then shifted to tacrolimus 0.01% and started on targeted NB-UVB. Excellent response was seen after 9 sessions of phototherapy. There was a decrease in induration, size and no further progression.

Morphea is a rare inflammatory condition without a clear etiology and early diagnosis and treatment are important. This case highlights the relationship between gadget trauma and the development of Morphea.

Human ; Female ; Adult: 25-44 Yrs Old ; Morphea ; Scleroderma, Localized ; Trauma

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