Generalized Purpuric Lichen Nitidus in a 32-year-old Filipino male with Acute Myeloid Leukemia: Case report

@#A 32-year-old Filipino male managed as a case of acute myeloid leukemia presented with a 6-month history of generalized, red-brown papules. After chemotherapy, the patient was advised 30-minutes daily sun exposure as a form of natural phototherapy. On follow-up, most lesions flattened. This case demonstrates chemotherapy’s potential role in controlling the patient’s inflammatory state and using ultraviolet light therapy in the form of sunlight to manage generalized purpuric lichen nitidus.

Primary Anetoderma and Acquired Cutis Laxa associated with Glomerulonephritis in a 37-year-old Filipino male: A case report

@#A 37-year-old Filipino man presented with a 9-month history of sagging skin progressing cephalocaudally from the chin and neck to the axillae, side of the trunk, and pelvic area. This was followed by a 2-month history of increasing serum creatinine levels associated with periorbital and bipedal edema, generalized weakness, decreased appetite, vomiting, and headache. Subsequently, skin-colored, non-tender sac-like plaques appeared on the abdomen, inguinal, and intergluteal areas. Histopathology of the latter lesions showed increased spaces between collagen bundles in the dermis. Staining with Verhoeff-van Gieson revealed focal sparse elastic fibers in the papillary dermis compared to that of the reticular dermis consistent with anetoderma. Further work-up revealed normal ANA titer and low serum C3. Kidney biopsy showed IgG deposition in the tubular basement membrane and trace C3 deposition in the glomerular mesangium, giving a diagnosis of rapid progressive glomerulonephritis. On subsequent follow-up, the sac-like plaques became lax and presented as generalized wrinkling of the skin, raising the question whether cutis laxa and anetoderma are occurring in a spectrum instead as distinct entities. Based on the current review of literature, this is the first reported case of primary anetoderma co-occurring with cutis laxa in a patient with glomerulonephritis. Deposition of immunoglobulins along the elastic fibers could have activated the complement system, mediating the destruction of the elastic fibers, resulting to cutis laxa and anetoderma. This case also considers the possibility of anetoderma and type I acquired cutis laxa occurring either in a spectrum or as distinct diseases in a single patient. Further investigations may identify an ultrastructural pattern that can help differentiate the two entities.

Traversing the spectrum of non-Langerhans Cell Histiocytosis: A case of Rosai-Dorfman Disease with features of Necrobiotic Xanthogranuloma

Introduction@#Non-Langerhans cell histiocytoses (non-LCH) are a group of rare diseases with varied clinical manifestations and overlapping features seen among the subtypes. Here, we present a case of Rosai-Dorfman disease with features of necrobiotic xanthogranuloma. @*Case@#A 45-year-old female presented with a 10-year history of an enlarging neck mass with normal overlying skin accompanied by dysphagia and multiple asymptomatic pink to yellowish-brown papules, nodules, and plaques on the face, trunk and extremities. Biopsies of a skin nodule and plaque revealed granulomatous dermal infiltrates (lymphocytes, foamy histiocytes, and Touton giant cells), emperipolesis and areas of necrosis. CD1A and Fite-Faraco staining showed negative results while CD68 and S100 positively stained the tissues of interest. Histopathology of the neck mass paralleled these findings in addition to being negative for lymphoid markers. Patient had monoclonal gammopathy and thyromegaly with enlarged cervical lymph nodes on further tests and imaging. Intralesional and systemic steroids were given which led to flattening of skin lesions and improvement in dysphagia, respectively. @*Conclusion@#Diagnosis and classification of a particular type of non-LCH may be difficult due to similarities across its subtypes. Hence, it is our belief that these diseases may occur on a spectrum. Treatment involves a multidisciplinary approach for the best possible care.

Trichotillomania masked by diffuse Alopecia Areata: A case report

@#An 11-year-old girl previously treated for tinea capitis presented a 3-month history of continuous decrease in hair density on the vertex, frontal, and parieto-temporal areas of the scalp. Hair pull test was negative. Trichoscopic findings showed black dots, micro-exclamation point hairs, regrowing vellus hair, and zigzag hairs. Histopathology showed CD3+ peribulbar lymphocytic infiltrates and occasional eosinophils around the anagen hair follicle consistent with a non-scarring alopecia. A diagnosis of diffuse alopecia areata was made. Patient was given methylprednisolone (0.5 mg/kg/day) for 2 weeks and noted marked increase in hair density except on focal areas of the scalp. Patient eventually admitted to occasional hair pulling. Trichoscopy revealed trichoptilosis, V-sign, tulip hairs, and multiple broken hairs of varying length while a second biopsy showed trichomalacia and pigment casts consistent with trichotillomania. In this case, where co-existence of alopecia areata and trichotillomania is considered to be uncommon, trichoscopy proved to be an important tool in differentiating hair disorders with similar presentation. Knowing key features of hair diseases can help elucidate the diagnosis when presented with an atypical case.

Azathiopine for the treatment of extensive forms of alopecia areata: A systematic review

@#<p style="text-align: justify;"><strong>BACKGROUND:</strong> Alopecia areata (AA) is an autoimmune hair disorder, with the clinical variants ophiasis and extensive variants AA totalis and universalis, having poor response to therapy. Oral steroids are used to treat the severe variants, requiring prolonged therapy, which leads to side effects while discontinuation leads to high relapse rate. Azathioprine is a steroid-sparing agent for the severe AA forms.</p><p style="text-align: justify;"><strong>OBJECTIVE:</strong> To review the current evidence on the therapeutic efficacy and adverse effects of azathioprine for severe forms of alopecia areata</p><p style="text-align: justify;"><strong>METHODS:</strong> Published articles utilizing azathioprine for alopecia areata were obtained until July 2018 from PubMed, MEDLINE, Cochrane Library, TRIP database, HERDIN, and Google Scholar.</p><p style="text-align: justify;"><strong>RESULTS:</strong> Seven articles underwent a full-length review. Clinical variants include patchy, diffuse, steroid-resistant, reticulate, totalis, universalis, ophiasis, and sisaipho. Doses ranged from 2 to 2.5 mg/kg/day or weekly 5 mg/kg pulse therapy. Initial response ranged from 6 to 12 weeks, with almost complete resolution by 32 weeks. Response was sustained for 6 months upon discontinuation, with only 14% relapsing at 2.5 months. Adverse effects were gastrointestinal discomfort, elevated liver function tests, and myelosuppression.</p><p style="text-align: justify;"><strong>CONCLUSION:</strong> There is emerging evidence on the efficacy and safety of azathioprine for the treatment of extensive forms of alopecia areata. Randomized-controlled trials are needed to evaluate its efficacy.</p>