Radiation Induced Bullous Pemphigoid: When Radiation Dermatitis Is Not The Answer

History: A 78-year old black female with history of invasive ductal carcinoma in the left breast status post lumpectomy and cytotoxic chemotherapy presented with acute onset blistering of the left breast with onset during the 24th cycle of radiation therapy which was diagnosed as radiation dermatitis by her radiation oncologist. New bullae on the left breast continued to arise after cessation of radiation therapy (RT). Within 4 weeks, patient was hospitalized with dysphagia, odynophagia, and oral ulcerations. At 8 weeks, patient developed new tense bullae on extremities and presented to dermatology. Examination: The entirety of the left breast extending onto the left flank had multiple flaccid serosanguinous filled bullae and erosions interspersed by well-demarcated depigmented patches with perifollicular macules of re-pigmentation. Three tense serosanguinous bullae were present on the right medial thigh, right medial lower leg and left calcaneal area. Two linear erythematous erosions with mild fibrinous debris extended from the hard palate down the oropharynx. Nikolsky sign was negative. There was no involvement of ocular or genital mucosa.Course and therapy: Punch biopsy of a bulla on the right medial thigh revealed subepidermal blisters with sparse infiltrate of lymphocytes and eosinophils in the underlying dermis. Direct immunofluorescence revealed 3+ linear IgG and C3 staining at the basement membrane zone. Salt split skin analysis revealed localization of linear IgG and C3 to the epidermal side of the dermal-epidermal junction, confirming the diagnosis of bullous pemphigoid (BP). Patient was treated with oral prednisone taper, high potency topical corticosteroids, and doxycycline 100 mg BID with niacinamide 500 mg BID in order to avoid immunosuppression.Discussion: Radiation dermatitis is a common cutaneous side effect of RT; however, persistent bullous eruption with spreads outside the radiation field requires further workup. BP is a rare complication of RT most often seen in breast cancer patients, but may occur in carcinomas of the lung, vulva, or esophagus. Etiology is unclear but may involve RT altering the basal membrane and expression of a mixture of hemidesmosome phenotypes by malignant breast cells which would serve as an antigen for antibody production inducing BP. The majority of cases occur during or up to 6 months after RT and tend to remain localized to RT-treated areas; rarely is there involvement of the oral mucosa. RT-associated BP appears more indolent than traditional BP and may respond to topical and non-systemic corticosteroid use.https://scholarlycommons.henryford.com/merf2019caserpt/1022/thumbnail.jp

Acquired Acrodermatitis Enteropathica Secondary to Nutritional Deficiency from Alcoholism

History A 30-year-old white male with history of alcoholism complicated by cirrhosis who presented to an outside hospital with loss of consciousness after being found down in excrement and urine for an unknown amount of time. Patient’s course was complicated by pancreatitis, urosepsis, spontaneous bacterial peritonitis, and hepatic encephalopathy. Patient was transferred to Henry Ford Hospital for further management, and dermatology was consulted for a one-year history of pruritic rash on the arms and legs with concern for scabies. Examination: Patient appeared thin and malnourished. He was intubated with 2+ pitting edema to his mid-thighs. On his scalp, lateral neck, right abdomen, bilateral dorsal hands extending onto the dorsal forearms, dorsal feet, bilateral inner thighs and the entirety of his scrotum, the patient had well-demarcated pink plaques with cracked riverbed-appearing scale. There was no involvement of the axillae, interdigital finger or toe web spaces, mons pubis, or umbilicus. Course and therapy: Zinc level was found to be markedly decreased at 35 mcg/dL [normal: 70-150 mcg/dL], confirming a diagnosis of acrodermatitis enteropathica (AE). HIV 4th generation Ag/Ab was found to be nonreactive. Alkaline phosphatase was found to be elevated at 735 IU/L [normal: 44-147 IU/L]. Patient was treated with oral zinc replacement at 1.5 mg/kg/day as well as topical petrolatum as needed. Patient had improvement of his zinc level from 35 mcg/dL to within normal limits at 80 mcg/dL within three weeks. Discussion: Acrodermatitis enteropathica is a condition resulting from zinc deficiency characterized by sharply demarcated, symmetric erythematous patches and plaques with erosions and scale-crust in a peri-oral, genital, and acral distribution. Severe deficiency may be accompanied by alopecia, diarrhea, depression, hypogonadism, and immunosuppression. AE may be inherited as an autosomal recessive gene mutation in SLC39A4 leading to deficiency in an intestinal zinc transporter protein. This process often presents in neonates when weaning from breast milk to formula or in cases of low maternal breast milk zinc concentrations. AE, however, may also be acquired secondary to decreased nutritional intake or increased excretion/malabsorption leading to zinc deficiency. Risk factors for decreased nutritional zinc intake include limited resources, alcoholism, anorexia nervosa, vegan diets, and diets high in mineral-binding phytates. Risk factors for increased excretion include intestinal malabsorption, liver disease, renal disease, Crohn’s disease, cystic fibrosis, and sickle cell disease. Diagnosis is made based on clinical features and serum zinc level \u3c 60 mcg/dL. In our patient, his zinc level was found to be significantly lower at 35 mcg/dL. Typically in AE, given that serum alkaline phosphatase (ALP) is a zinc-dependent enzyme, a decreased ALP is expected. Our patient’s ALP was found to be elevated, likely secondary to concomitant pancreatitis. Appropriate treatment is with oral zinc replacement at 1-2 mg/kg/day of elemental zinc with cutaneous improvement expected in weeks to months. Extracutaneous improvement often shows within 24 hours of beginning treatment. Our patient was treated with 1.5 mg/kg/day of oral elemental zinc, with improvement in his cutaneous lesions seen within 3 weeks.https://scholarlycommons.henryford.com/merf2019caserpt/1086/thumbnail.jp

Congenital Midline Nodules on the Chin and Sternum

History: A 5-day old black male full-term neonate born via vacuum-assisted delivery for non-reassuring fetal heart rate presented with congenital presentation of two asymptomatic midline lesions which appeared asymptomatic. There was no history of seizures, ophthalmologic findings, abnormalities in head circumference, height, weight or limb size. Newborn screening examination was unremarkable. Examination: On the midline submental chin there was a soft, brown dome-shaped plaque measuring 0.8-centimeters with a circumferential ring of light brown pigmentation; on the midline upper chest there was a light brown 2-millimeter dome-shaped papule. Course and Therapy: Ultrasound of the submental chin lesion revealed a 0.5 x 0.8 x 0.4-centimeter heterogeneously hypoechoic structure with a peripheral soft tissue rind. Punch biopsies of the submental chin and the midline upper chest revealed haphazardly arranged striated muscle fibers in the dermis, some of which inserted directly into the epidermis. The muscle fibers were highlighted by Masson’s trichrome and myogenin. Alcian blue revealed increased dermal mucin. Discussion: Striated muscle hamartomas (SMH) are rare, benign congenital skin tumors characterized by haphazard arrangement of mature striated skeletal muscle, collagen, nerve bundles, and adipose tissue in the dermal and subcutaneous tissue. Although a rare entity, it is important to recognize this benign hamartoma as a congenital midline defect. Conservative management with clinical monitoring is recommended if cosmetically acceptable, as spontaneous regression over a period of years has been reported. Surgical excision may be pursued; however, the hamartoma may recur.https://scholarlycommons.henryford.com/merf2020caserpt/1133/thumbnail.jp

Sleep-related rhythmic movement disorder: A case report of head banging alopecia

We present a case of alopecia associated with sleep-related rhythmic movement disorder (RMD) in an otherwise healthy 2-year-old boy. The alopecic patch he presented with on his scalp coincided with the location of repeated head banging in a video taken by the patient\u27s mother. Alopecia secondary to RMD is an under recognized entity and should be included in the differential diagnosis of pediatric alopecia

Seeing a Difference: A Visual Aid Tool for Topical Medication Selection

Topical medications are the mainstay of many common dermatologic conditions. There are many factors that contribute to patient adherence to topical regimens with medication vehicle playing a role in usage. Due to lack of familiarity with topical vehicle nomenclature, patients often have difficulty selecting the appropriate vehicle when discussing their therapy plan, leading to unsatisfactory results and potentially medication discontinuation. To assist the patient in topical medication vehicle selection, we created a visual aid that was conveniently placed in examination rooms to assist in the selection of both new and refilled medications. Following medication selection, a three-question survey was provided to patients seen at one dermatology clinic assessing patient perception of the visual aid. Over a three-month period, 50 patients were surveyed and the results were examined via chi-square analysis. Most medications prescribed were new (80%), with refill (8%), and new plus refilled medications (12%) making up the minority. A clear majority of those surveyed (98%) found the visual aid helpful in selection of their medication. Additionally, 80% of patients responded that they would be more likely to use their topical medication after selection with assistance of the visual aid. These results demonstrate that the visual aid is a statistically effective tool assisting in medication selection as well as having the potential to improve patient adherence to topical medications (p\u3c 0.001). Anecdotally the authors also thought it expedited the medication selection process. Although additional studies are likely needed, this data supports that use of a visual aid in selection of topical medications.https://scholarlycommons.henryford.com/merf2019qi/1001/thumbnail.jp