In 1963, Rowellet al. described a syndrome combining lupus erythematosus (LE) and erythema multiforme (EM)-like lesions. In this report, we present a 15-year-old female who presented with both systemic LE and EM-like skin lesions meeting all of the major and one of the minor criteria for a diagnosis of Rowell syndrome. Her condition improved with administration of systemic and topical corticosteroids, and hydroxychloroquine. Rowell Syndrome, a rare entity, is often debated as a coincidental overlap of other conditions according to Bonciolini,et al.[1]In light of Rowell Syndrome's infrequency and the paucity of available literature, we emphasize the clinical significance of recognizing this challenging condition.

Leukocytoclastic vasculitis (LCV) is a histopathologic descriptor for a prevalent type of small-vessel vasculitis (SVV) that affects arterioles, capillaries, and postcapillary venules. Although the association between vasculitis and malignancy only accounts for <5% of vasculitis cases, it has been recognized as a true paraneoplastic syndrome in several studies. A 57-year-old Filipino male presented with erythematous, nonblanching macules on his lower extremities, which rapidly progressed to violaceous lesions on his trunk, buttocks, and lower extremities. He also reported significant weight loss, decreased appetite, and vomiting. A skin biopsy confirmed LCV. Initially treated for meningococcemia, his condition did not improve. Abdominal imaging revealed an enlarged heterogeneous liver with retroperitoneal lymphadenopathy and a parenchymal nodule. He was eventually diagnosed with vasculitis secondary to an underlying hepatic malignancy and expired later from multiorgan failure.

Erythema elevatum diutinum (EED) is a rare, chronic leukocytoclastic vasculitis characterized by erythematous to violaceous plaques and nodules, typically on extensor surfaces like the hands, elbows, and knees. It results from immune complex deposition in blood vessels, leading to inflammation and fibrosis. EED is often associated with infections, autoimmune disorders, or hematologic malignancies, but can also occur idiopathically. Although globally documented, EED is extremely rare in the Philippines, particularly in adolescents, highlighting its significance in local literature.

A 14-year-old Filipino female presented with a 5-month history of asymptomatic, skin-colored papules on her right elbow, gradually spreading to both elbows and knees, with occasional mild pruritus and knee joint pain. Past medical and family history were unremarkable. After temporary relief from an unrecalled cream prescribed by a private dermatologist, she was referred for skin punch biopsy, which revealed spongiotic epidermis with papillary dermal edema, moderate inflammatory infiltrates, eosinophilic inclusion bodies, and nuclear dusts surrounding the blood vessels, consistent with EED. The patient was treated with dapsone 50 mg/day, clobetasol propionate ointment twice daily for two weeks, and cetirizine 10 mg as needed for pruritus, resulting in clinical improvement.

This case highlights the extreme rarity of EED in the Philippines, particularly in adolescents. It emphasizes the need to consider EED in chronic papular eruptions and demonstrates the effectiveness of dapsone and topical steroids in managing the condition. Early diagnosis and timely intervention are crucial for preventing disease progression and improving patient outcomes, as seen in this case.

Toxic epidermal necrolysis (TEN) is a rare, life-threatening dermatological emergency characterized by extensive epidermal necrosis and detachment, along with significant mucous membrane erosions and systemic symptoms. While conventional medications such as sulfonamides and anticonvulsants are known triggers, recent reports suggest that herbal medications can also induce TEN. This case report highlights the development of TEN in a patient with invasive cervical cancer following the use of an herbal supplement.

A 48-year-old Filipino woman with newly diagnosed invasive cervical cancer developed TEN after taking an herbal supplement containing 23 botanical extracts. Despite discontinuing the supplement, her condition rapidly worsened, with vesicles and bullae progressing to epidermal detachment over 90% of her body surface area. A histopathological examination confirmed the diagnosis of TEN. The patient was treated with systemic corticosteroids and supportive care, achieving full recovery within 14 days.

This case underscores the potential risks associated with herbal medications, particularly in vulnerable patients such as those with cancer. Although conventional pharmaceuticals are the primary culprits for TEN, this report highlights the need for greater awareness of herbal medicine-induced adverse drug reactions. Underreporting of such events, due to lack of awareness among patients and healthcare providers, further complicates the issue. This case emphasizes the need for strengthened regulatory policies and enhanced education to safeguard public health amidst the increasing use of herbal remedies worldwide.

Acute generalized exanthematous pustulosis (AGEP) and Stevens-Johnson Syndrome (SJS) are uncommon, severe cutaneous drug eruptions with distinct clinical and histopathological features. AGEP-SJS overlap is a rare and complicated cutaneous drug eruption. Neutrophilia, leukocytosis, and elevated liver enzymes can be seen in these patients. Currently, there are no available dermoscopic studies on AGEP overlapping SJS. The pathophysiology of overlapping drug reaction are mediated by T cells and delayed-type hypersensitivity. Management includes removal of offending drug and giving supportive measures like pain management, moist dressing and fluids.

Sweet syndrome, or acute febrile neutrophilic dermatosis, is a rare inflammatory condition characterized by painful erythematous plaques, fever, and neutrophilia. While it is often associated with malignancy or drug reactions, it may also occur following streptococcal infection. This case highlights the importance of early recognition and treatment of Sweet syndrome secondary to streptococcal pharyngitis.

A 44-year-old female presented with painful erythematous papules and nodules on her extremities, neck, and nape, associated with fever and chills. The lesions started as skin-colored papules that rapidly progressed to painful erythematous plaques. Her medical history included a sore throat two weeks prior. Initial management Clindamycin provided temporary relief, but the lesions persisted. Laboratory work revealed neutrophilic leukocytosis, elevated erythrocyte sedimentation rate (ESR), and an elevated anti-streptolysin O titer. A skin biopsy showed diffuse neutrophilic dermatitis with vasculitis, confirming the diagnosis of Sweet syndrome. Subsequent treatment with oral corticosteroids led to significant clinical improvement over a week.

This case illustrates the importance of considering infectious triggers, such as streptococcal infections, in the diagnosis of Sweet syndrome. Early diagnosis and appropriate treatment with corticosteroids are essential in preventing complications. Reporting this case contributes to the understanding of non-malignant triggers of Sweet syndrome and underscores the need for heightened awareness of this rare condition in patients presenting with acute febrile dermatosis.

Sweet syndrome (SS), or acute febrile neutrophilic dermatosis, is a rare inflammatory disorder characterized by fever, painful raised plaques, and dermal neutrophilic infiltration. Pediatric cases account for only 5% of SS globally, and no report exists of SS in infants in the Philippines. This report documents a unique case of SS in a one-month-old male, one of the first documented in the country. The rarity of this condition in infants and its successful management contribute valuable insights into the understanding and treatment of pediatric SS.

A one-month-old male presented with fever, erythematous plaques on the face and extremities, and a rapidly growing nodule on the left lateral neck, along with concurrent fever and cough. Skin biopsy showed dermal neutrophilic infiltration without leukocytoclastic vasculitis, confirming SS. A CT scan revealed a neck abscess and pneumonia. The abscess was treated with incision and drainage, and IV antibiotics. Due to the infection, systemic corticosteroids were contraindicated. Instead, topical hydrocortisone was applied, leading to rapid improvement of the skin lesions. This highlights a successful approach for managing SS when systemic therapy is unsuitable.

This case emphasizes the importance of early diagnosis and individualized treatment in rare pediatric cases of Sweet syndrome. The effective use of topical hydrocortisone in place of systemic steroids demonstrates the potential of alternative therapies, especially when infections preclude standard treatments. This case, one of the first in a neonate in the Philippines, contributes valuable insights to the limited literature on managing this rare condition in infancy.

Type 2 reversal reactions (erythema nodosum leprosum, ENL) are a significant cause of morbidity in multibacillary leprosy. These reactions are commonly managed with long-term corticosteroids, which pose many risks. This case report highlights the use of methotrexate as a steroid-sparing treatment for ENL.

A 35-year-old female diagnosed with lepromatous leprosy in March 2020 developed painful erythematous nodules two months into multi-drug therapy. Biopsy revealed lobular panniculitis with vasculitis, consistent with ENL. She was started on prednisone (50 mg/day), which was tapered over the next 3.5 years, fluctuating between 5-55 mg/day depending on lesion recurrence.

In August 2023, the patient was diagnosed with pulmonary tuberculosis (PTB), necessitating PTB treatment. At this point, the decision was made to augment the patient’s prednisone (25 mg/day) with methotrexate (5 mg/week). Over a year, Methotrexate was increased to 10 mg/week while Prednisone was reduced to 10 mg/day. The patient reported a 60% improvement in lesion number and erythema. The goal is to continue to taper her Prednisone until it is discontinued.

The Philippines is a global priority country for leprosy, with over 90% of cases being classified as multibacillary (MB). Given that ENL is seen in up to half of all MB cases, accessible management options are necessary. Long-term corticosteroid use for ENL is unsustainable due to side effects. Methotrexate, a cost-effective alternative, showed promise in this case by reducing steroid dependence and improving clinical outcomes. Further research is needed to establish Methotrexate’s long-term efficacy and safety in managing ENL.

Systemic lupus erythematosus (SLE) associated macrophage activation syndrome (MAS) is clinically severe, with a high mortality rate and rare neuropsychiatric symptoms. In the course of diagnosis and treatment, it is necessary to actively determine whether the neuropsychiatric symptoms in patients are caused by neuropsychiatric systemic lupus erythematosus (NPSLE) or macrophage activation syndrome. This paper retrospectively analyzed the clinical data of 2 cases of SLE associated MAS with neuropsychiatric lesions, Case 1: A 30-year-old female had obvious alopecia in 2019, accompanied by emaciation, fatigue and dry mouth. In March 2021, she felt weak legs and fell down, followed by fever and chills without obvious causes. After completing relevant examinations, she was diagnosed with SLE and given symptomatic treatments such as hormones and anti-infection, but the patient still had fever. The relevant examinations showed moderate anemia, elevated ferritin, elevated triglycerides, decreased NK cell activity, and a perforin positivity rate of 4.27%, which led to the diagnosis of "pre-hemophagocytic syndrome (HPS)". In May 2021, the patient showed mental trance and babble, and was diagnosed with "SLE-associated MAS"after completing relevant examinations. After treatment with methylprednisolone, anti-infection and psychotropic drugs, the patient's temperature was normal and mental symptoms improved. Case 2: A 30-year-old female patient developed butterfly erythema on both sides of the nose on her face and several erythema on her neck in June 2019, accompanied by alopecia, oral ulcers, and fever. She was diagnosed with "SLE" after completing relevant examinations, and her condition was relieved after treatment with methylprednisolone and human immunoglobulin. In October 2019, the patient showed apathy, no lethargy, and fever again, accompanied by dizziness and vomiting. The relevant examination indicated moderate anemia, decreased NK cell activity, elevated triglycerides, and elevated ferritin. The patient was considered to be diagnosed with "SLE, NPSLE, and SLE-associated MAS". After treatment with hormones, human immunoglobulin, anti-infection, rituximab (Mabthera), the patient's condition improved and was discharged from the hospital. After discharge, the patient regularly took methylprednisolone tablets (Medrol), and her psychiatric symptoms were still intermittent. In November 2019, she developed symptoms of fever, mania, and delirium, and later turned to an apathetic state, and was given methylprednisolone intravenous drip and olanzapine tablets (Zyprexa) orally. After the mental symptoms improved, she was treated with rituximab (Mabthera). Later, due to repeated infections, she was replaced with Belizumab (Benlysta), and she was recovered from her psychiatric anomalies in March 2021. Through the analysis of clinical symptoms, imaging examination, laboratory examination, treatment course and effect, it is speculated that the neuropsychiatric symptoms of case 1 are more likely to be caused by MAS, and that of case 2 is more likely to be caused by SLE. At present, there is no direct laboratory basis for the identification of the two neuropsychiatric symptoms. The etiology of neuropsychiatric symptoms can be determined by clinical manifestations, imaging manifestations, cerebrospinal fluid detection, and the patient's response to treatment. Early diagnosis is of great significance for guiding clinical treatment, monitoring the condition and judging the prognosis. The good prognosis of the two cases in this paper is closely related to the early diagnosis, treatment and intervention of the disease.
Humans ; Female ; Adult ; Rituximab/therapeutic use* ; Macrophage Activation Syndrome/etiology* ; Retrospective Studies ; Lupus Erythematosus, Systemic/drug therapy* ; Methylprednisolone/therapeutic use* ; Lupus Vasculitis, Central Nervous System ; Fever/drug therapy* ; Erythema/drug therapy* ; Hormones/therapeutic use* ; Anemia ; Alopecia/drug therapy* ; Triglycerides/therapeutic use* ; Ferritins/therapeutic use*

The acquired immunodeficiency syndrome patients with compromised immunity are prone to hemophagocytic syndrome secondary to opportunistic infections.This paper reports a rare case of hemophagocytic syndrome secondary to human parvovirus B19 infection in an acquired immunodeficiency syndrome patient,and analyzes the clinical characteristics,aiming to improve the diagnosis and treatment of the disease and prevent missed diagnosis and misdiagnosis.
Humans ; Lymphohistiocytosis, Hemophagocytic/drug therapy* ; Erythema Infectiosum/complications* ; Acquired Immunodeficiency Syndrome/complications* ; Parvoviridae Infections/diagnosis* ; Parvovirus B19, Human