ABSTRACT
We present a case of cyclical periorbital angio-oedema, and highlight the appropriate investigations to support the clinical diagnosis of this rare dermatosis.
Subject(s)
Angioedema , Dermatitis, Atopic , Angioedema/diagnosis , HumansABSTRACT
A 23-year-old woman presented with a hyperpigmented intertriginous eruption unresponsive to topical steroids and to topical and systemic antifungals. Histopathological assessment demonstrated patchy inflammatory infiltrate at the demoepidermal junction with pigment incontinence. Click here for the corresponding questions to this CME article.
Subject(s)
Exanthema , Hyperpigmentation , Female , Humans , Young Adult , Adult , Hyperpigmentation/diagnosisABSTRACT
Omenn syndrome is a rare combined immunodeficiency mostly associated with RAG1 and RAG2 mutations; the clinical manifestations are well-described and include neonatal erythroderma. Mortality due to opportunistic infections is a serious risk, and a timely diagnosis with a skin biopsy is an important part of the diagnostic workup. We wish to highlight key clinical features of Omenn syndrome and discuss the relevance of a skin biopsy.
Subject(s)
Dermatitis, Exfoliative , Severe Combined Immunodeficiency , Homeodomain Proteins/genetics , Humans , Infant, Newborn , Mutation , Severe Combined Immunodeficiency/diagnosis , SyndromeSubject(s)
Scabies , Humans , Scabies/diagnosis , Immunosuppression Therapy , Diagnosis, Differential , Ivermectin/therapeutic useABSTRACT
A neonatal boy presented with a persistent urticarial rash. Initial investigations showed raised inflammatory markers and evidence of systemic inflammation. A working diagnosis of cryopyrin-associated periodic syndrome (CAPS) was made, and the patient responded extremely well to Anakinra. Molecular genetic testing revealed a somatic mutation (affecting 12.5% of cells) in the NLRP3 gene, accounting for the persistent inflammatory state but milder phenotype as seen in our patient.
Subject(s)
Antirheumatic Agents/therapeutic use , Cryopyrin-Associated Periodic Syndromes/diagnosis , Interleukin 1 Receptor Antagonist Protein/therapeutic use , Urticaria/etiology , Cryopyrin-Associated Periodic Syndromes/drug therapy , Humans , Infant, Newborn , Male , Mutation , NLR Family, Pyrin Domain-Containing 3 Protein/genetics , Skin/pathologyABSTRACT
BACKGROUND: Phototherapy is a well-recognized treatment in adults and children. Previous articles have reported success in treating recalcitrant skin disorders such as atopic dermatitis (AD), psoriasis, pityriasis lichenoides chronica, and vitiligo in children. METHODS: This was a retrospective review over an 18-month period from June 2012 to December 2013 of all children receiving phototherapy in a tertiary pediatric dermatology center. RESULTS: Seventy-five patients 3 to 17 years of age (mean 10.6 years; 35 male, 40 female) were included. Forty-eight (64%) patients had AD and 21 (28%) had psoriasis. Seventy received narrowband ultraviolet B (NBUVB) treatment and five received hand and foot psoralen and ultraviolet A (PUVA) treatment. All patients with AD were treated with NBUVB and four (8.3%) were also treated with hand PUVA. After phototherapy, 76% had documented clear to almost clear skin. At the 12-month follow-up, 52% of the patients with AD remained clear. All 21 patients with psoriasis underwent NBUVB phototherapy. The clearance rate after phototherapy was 86%. At the 12-month follow-up, 43% of the patients with psoriasis remained clear. CONCLUSION: Phototherapy can reduce disease burden in individuals with severe AD and psoriasis and should be considered as a second-line therapy if standard topical regimens are unsuccessful.
Subject(s)
Dermatitis, Atopic/therapy , Phototherapy , Pityriasis Lichenoides/therapy , Psoriasis/therapy , Vitiligo/therapy , Adolescent , Child , Child, Preschool , Female , Humans , Male , Retrospective Studies , Treatment OutcomeSubject(s)
Dapsone/therapeutic use , Vasculitis, Leukocytoclastic, Cutaneous/drug therapy , Vasculitis, Leukocytoclastic, Cutaneous/pathology , Biopsy, Needle , Humans , Immunohistochemistry , Leg Dermatoses/diagnosis , Leg Dermatoses/etiology , Male , Middle Aged , Rare Diseases , Severity of Illness Index , Treatment Outcome , Vasculitis, Leukocytoclastic, Cutaneous/diagnosisSubject(s)
Blister/etiology , Churg-Strauss Syndrome/complications , Churg-Strauss Syndrome/diagnosis , Purpura/etiology , Aged , Humans , MaleABSTRACT
Intravenous immunoglobulin (IVIg) is increasingly used across multiple specialties for the treatment of inflammatory and autoimmune diseases. Cutaneous reactions to IVIg are generally minor. Pompholyx is a common eruption of small vesicles on the palms, soles, and/or lateral aspects of the fingers. It has a multifactorial aetiology but is rarely attributed to being a drug-related side effect. We describe a 43-year-old woman presenting with peripheral sensory neuropathy who developed pompholyx eczema on both palms following treatment with IVIg.
Subject(s)
Autoimmune Diseases , Drug-Related Side Effects and Adverse Reactions , Eczema, Dyshidrotic , Peripheral Nervous System Diseases , Adult , Autoimmune Diseases/drug therapy , Eczema, Dyshidrotic/chemically induced , Eczema, Dyshidrotic/drug therapy , Female , Humans , Immunoglobulins, Intravenous/adverse effects , Peripheral Nervous System Diseases/chemically inducedABSTRACT
Clear cell acanthoma (CCA) is a rare, benign epidermal lesion of clear glycogen-containing keratinocytes. The exact etiology is unknown. Although CCA usually appear as solitary lesions, cases of multiple CCAs have also been described. The rare eruptive CCA variant describes cases with more than 30 lesions.Current therapeutic evidence for the management of multiple CCAs is limited. CO2 laser remains the most widely used laser in clinical practice. However, literature describing its effectiveness on CCA is sparse, and previous reports have described its use for a maximum of three lesions. To our knowledge, this is the first report of successful CO2 laser treatment of the multiple eruptive CCA variant with an excellent cosmetic outcome.Our case adds to the evidence that CO2 laser is an effective treatment for this condition. Particularly in those patients with refractory or multiple eruptive CCAs, or for those who may not tolerate repeated courses of cryotherapy, or who are concerned about scarring and cosmetic outcome.