Hypocomplementemic urticarial vasculitis syndrome (HUVS) is a rare condition characterized by immune complex-mediated urticarial lesions with histological features of leukocytoclastic vasculitis, low serum complement levels, and is frequently associated with systemic manifestations. Its pathophysiology is poorly understood. We present a patient who presented with abdominal pain and skin rash. Extensive work-up was performed including skin biopsy, and the presence of angioedema, oral ulcers, low complement level, leukocytic vasculitis, and persistent eosinophilia ultimately led to the diagnosis of HUVS. This case highlights the importance of recognizing and differentiating HUVS from other cutaneous diseases, which in turn helps to optimally manage these patients.

Epidermolysis bullosa (EB) is a rare genetic condition characterized by fragile skin caused by impaired adhesion between the dermis and epidermis. EB is present at or near birth. There is no cure and treatments are supportive. Children with EB are at elevated risk of squamous cell cancer. Under ideal circumstances, EB patients benefit from interdisciplinary care teams who can offer state-of-the-art treatments. In reality and particularly in less-developed nations, care can be limited. In all cases, families dealing with a member with EB face great challenges in caregiving, much of which is managed at home, and incur great financial expenses for dressings, equipment, transportation, and out-of-pocket expenses. While research groups are working to find a cure for EB, clinicians working with EB patients around the world have found practical and relatively inexpensive tips to make life easier for people with EB. NoBabyBlisters.org, a nonprofit organization actively supplying monthly medical supplies for EB children on five continents and working on EB research, has innovated, developed, collected, and now offers here seven such practical and actionable items learned from its experience in the real world assisting children in less-developed nations, typically with hot climates. These are based on real-world clinical experience dealing with a complex disorder under challenging circumstances. The goal of this short paper is to provide advice to EB caregivers and their loved ones that may make things easier and enhance quality of life, including blister and pain reduction.

Stevens-Johnson Syndrome (SJS), a severe mucocutaneous hypersensitivity reaction primarily triggered by drugs, poses a low-incidence, high-mortality challenge. This report explores its clinical nuances and emphasizes supportive care as the mainstay of treatment. A 74-year-old female, burdened with a complex medical history, presented with a non-pruritic macular rash escalating to skin and oral mucosal involvement. A recent introduction of dipyrone (metamizole) implicated drug-induced SJS. Histopathological confirmation guided treatment involving supportive care, corticosteroids, and wound care, resulting in clinical improvement. The case underscores the significance of histopathological confirmation and thorough medication history in navigating SJS complexities, especially in patients with comorbidities like connective tissue disease. A successful multidisciplinary approach and the decision for post-discharge monitoring highlight the intricate management challenges. This case illuminates the intricate interplay of medication-induced hypersensitivity, comorbidities, and management challenges in SJS. Optimal outcomes require prompt diagnosis, trigger identification, and a multidisciplinary treatment approach, emphasizing ongoing research and clinical vigilance.

White fibrous papulosis of the neck (WFPN) manifests through the presence of numerous solid, persistent, and asymptomatic yellowish-white papules, displaying a distinctive asymmetrical distribution primarily localized on the neck and antecubital fossa. This case report describes the clinical presentation of a 70-year-old female diagnosed with WFPN, highlighting the significant finding of collagen fiber thickening upon histopathological analysis. Despite its predilection for specific anatomical sites, the elusive pathogenesis of WFPN adds diagnostic complexity, emphasizing the need for further research in this unique condition that generally follows a benign course.

Chanarin-Dorfman syndrome (CDS) is a rare medical condition that is inherited in an autosomal recessive pattern. In CDS, a comparative gene identification-58 gene mutation causes the accumulation of triglycerides in neutrophils, which can be observed as vacuoles on a peripheral smear. CDS patients present with a characteristic dermatological finding, ichthyosis, which is a non-bullous white scaling of the skin. Here, we describe a case report of a one-year-old boy who presented to the pediatric outpatient department (OPD) with chief complaints of peeling of the skin and ballooning of the abdomen since birth. Our patient had achieved all the developmental milestones pertaining to his age. Genetic testing was positive for heterozygous alleles in both parents.

UNASSIGNED: Epidermolysis bullosa (EB) is a rare, incurable genodermatosis causing blisters that can result in multisystemic complications and death. Limited data exists on EB in South Africa. Research indicates that the majority of African patients consult traditional health practitioners (THPs) before seeking allopathic healthcare.
UNASSIGNED: This study aims to understand THPs belief systems, experiences, perceptions and management of EB patients and their families in the social and cultural context to improve the healthcare of EB patients.
UNASSIGNED: The study setting is Nelson Mandela School of Medicine, Durban, and Grey\'s hospital, Pietermaritzburg, KwaZulu-Natal.
UNASSIGNED: Qualitative in-depth interviews were conducted with 10 THPs. A non-probability, purposive sampling method was used. A two-site qualitative study was guided by interpretative phenomenological analysis. Guba\'s trustworthiness framework was used to ensure rigour.
UNASSIGNED: Three male and seven female THPs were interviewed, including sangoma, inyanga and umthandazi. The integration presented five global themes: (1) THP practices, (2) perceptions of THP, (3) experiences of THP with patients with EB, (4) diagnosis and management plans of THP and (5) vision and role of THPs. There were multiple divergent perspectives among the THPs with the shared African worldview.
UNASSIGNED: Understanding THPs belief systems and therapeutic options is crucial for holistic patient management. Knowledge exchange can promote safe healthcare practices and facilitate collaboration between traditional and allopathic health practitioners.
UNASSIGNED: This is the first study to explore THPs perceptions and practices regarding EB, a rare disease.

Lichen planus pigmentosus (LPP) is a rare form of lichen planus that typically affects middle-aged people with darker-pigmented skin. LPP is associated with a longer clinical course than classical lichen planus, which distinguishes it clinically. Its occurrence in children is uncommon, with few reported cases in this population in the literature. We report a rare presentation of unilateral blaschkoid LPP in a seven-year-old Saudi Arabian female patient.

Erdheim Chester disease (ECD) is a rare and complex non-Langerhans histiocytic systemic disease that affects multiple organ systems, including the bones, heart, lungs, and central nervous system. Fewer than 1,000 cases have been reported in the medical literature and dermatological manifestations of the disease are rare but can provide valuable diagnostic clues for this challenging disease. The cutaneous manifestations of ECD can take many forms, including nodules, plaques, papules, and xanthomas. These lesions can occur on any part of the body and may be solitary or multiple. Cutaneous manifestations of ECD have been reported to occur in up to 20% of cases, but the true prevalence may be higher, as many cases may go undiagnosed. We present the case of a 62-year-old gentleman with a history of ECD currently on vemurafenib who presented with multiple painless subcutaneous nodules on his back after an excision biopsy under local anesthetic revealed histological features of ECD. The objective of this case report is to raise awareness of ECD and its dermatological manifestations. Further research is warranted to better understand the pathogenesis and morphology of cutaneous involvement in ECD.

Malignant eccrine spiradenoma is a rare cutaneous adnexal neoplasm and is often a result of the malignant transformation of a benign eccrine spiradenoma. A woman without a history of skin cancer presented with a mass on her posterior scalp. An excisional biopsy was obtained, and histology was consistent with eccrine spiradenocarcinoma with the lesion extending to all margins of the excision specimen. Physical exam and imaging did not reveal lymph node involvement or distant spread of disease. It was recommended that the patient undergo wide local excision.

Langerhans cell histiocytosis (LCH) is a rare neoplastic disease of myeloid dendritic cells with a widely variable presentation of organ system involvement and severity. In this case report, we share the details of a rare case of cutaneous LCH resembling hidradenitis suppurativa (HS).