Blau Syndrome With Delayed Cutaneous Manifestations: A Case Report.

ABSTRACT: Blau syndrome is a rare familial autoinflammatory disorder characterized by the triad of granulomatous dermatitis, polyarthritis, and uveitis. Blau syndrome exhibits an autosomal dominant inheritance pattern and can be caused by a gain-of-function mutation in nucleotide-binding oligomerization domain 2 (NOD2), a member of the NOD-like receptor family of pattern recognition receptors. Mutations in NOD2 cause upregulation of inflammatory cytokines and resultant autoinflammation. Because of the rarity of this condition and early onset of symptoms, Blau syndrome may be misdiagnosed as juvenile idiopathic arthritis. We present a case of a 37-year-old male patient with a long-documented history of juvenile idiopathic arthritis and uveitis, who developed an asymptomatic eruption of pink papules on the trunk and upper extremities. A biopsy demonstrated noncaseating, well-formed dermal granulomas with relatively sparse lymphocytic inflammation and Langerhans-type giant cells. Genetic testing confirmed a mutation in NOD2. Based on the patient's clinical history, histologic findings, genetic testing, the diagnosis of Blau syndrome was made.

Acute generalized exanthematous pustulosis associated with clindamycin in a patient with Hailey-Hailey disease.

A 61-year-old African-American female with moderately controlled Hailey-Hailey disease (HHD) presents to the emergency department with a rash and fever. One day prior to her presentation, she was started on oral clindamycin for a tooth extraction procedure. Her physical examination shows diffuse erythema on the trunk and extremities with multiple nonfollicular pustules. A punch biopsy of her upper extremity revealed intraepidermal acantholysis, neutrophilic spongiosis, and subcorneal pustules. The perivascular and interstitial superficial dermal infiltrate is mixed and composed of predominantly neutrophils, with lymphocytes and rare eosinophils. These findings suggest a superimposed acute generalized exanthematous pustulosis (AGEP) in the background of HHD. AGEP is a potentially severe cutaneous condition characterized by the abrupt onset of numerous nonfollicular pustules in a background of pruritic edematous erythroderma. To date, only two case reports have described AGEP in patients with HHD. Early diagnosis of AGEP is essential to initiate prompt and aggressive systemic therapy, prompt medication cessation, close monitoring for end-organ damage, and improve overall morbidity and mortality.

WITHDRAWN: JAAD Game Changers: Multivariate analysis of potential risk factors for lymph node metastasis in patients with cutaneous squamous cell carcinoma of the head and neck.

The Publisher regrets that this article is an accidental duplication of an article that has already been published in J Am Acad Dermatol, 80 (2019) 607 - 607, https://doi.org/10.1016/j.jaad.2018.10.049. The duplicate article has therefore been withdrawn. The full Elsevier Policy on Article Withdrawal can be found at https://www.elsevier.com/about/our-business/policies/article-withdrawal.

Unusually extensive scalp ulcerations manifested in pemphigus erythematosus.

Senear-Usher Syndrome, or pemphigus erythematosus, is an autoimmune skin blistering disorder with an overlapping clinical presentation of pemphigus foliaceus and lupus erythematosus. Lesions typically involve the scalp, face, and upper chest or back. This case study focuses on a patient who presentedwith progressive scalp ulcers, hyperpigmentation, and eroded plaques with overlying hemorrhagic crust. Pemphigus erythematosus was diagnosedwith direct immunofluorescence, demonstrating immunoglobulin G and complement deposition both intercellularly and at the dermoepidermal junction. The patient is continuing treatment with systemicsteroids and steroid-sparing immunosuppressants.

Successful treatment of two individual cases of generalized granuloma annulare with amoxicillin/clavulanic acid and a combination of doxycycline and pentoxifylline.

Granuloma annulare is a benign inflammatory skin disease potentially related to a delayed hypersensitivity reaction to the dermis. Generalized granuloma annulare (GGA) manifests as diffuse skin-colored to erythematous annular or nummular plaques affecting at least the trunk and either upper or lower extremities, or both. GGA is resistant to many therapeutic modalities, making it difficult to treat. Different therapeutic approaches to GGA have been attempted but definitive treatment for this disease remains elusive. This article focuses on the use of amoxicillin/clavulanic acid and a combination of doxycycline and pentoxifylline therapy as treatment options for GGA in two patients with histopathology-proven interstitial GGA. Both amoxicillin/clavulanic acid and doxycycline inhibit bacterial cell growth, raising the possibility that a bacterial pathogenesis may be of significance in GGA. This is the first reported case of successful treatment of GGA with these regimens.