Barrett Zlotoff

Primary cutaneous coccidioidomycosis: an incidental finding

A 77-year-old immunocompetent man residing in southern New Mexico presented for a routine skin cancer screening examination. His medical history was significant for several nonmelanoma skin cancers. During the examination, two lesions were found, which were suspected to be basal cell carcinomas (BCCs): a 2.5-mm pink papule on the right chin and a 6 · 8-mm thin pink papule on the left lower back (Fig. 1a). Histological examination of biopsy samples taken from the lesions confirmed the clinical diagnosis of BCC for the right chin papule, but the left lower back lesion had a granulomatous infiltrate in the dermis with spherules diagnostic of coccidioidomycosis (Fig. 1b,c). The patient had been unaware of the lesion on the left lower back until it was identified during the skinscreening examination. After discussing the diagnosis with the patient, he recalled that 5 months earlier he had been working in his yard pruning trees and had sustained a puncture wound from a mesquite branch to the area of concern on his left lower back. After the area had healed, it remained asymptomatic and he forgot about it . He reported that he was currently feeling well and did not recall any flu-like illness or respiratory symptoms at the time of the injury or subsequently. Physical examination found no evidence of local induration, regional adenopathy or evidence of lymphangitis. The patient was referred to a specialist in infectious disease for further evaluation, which revealed no evidence of systemic involvement. Test results included an unremarkable chest X-ray and normal complete blood count, serum electrolytes and liver function tests. Coccidioides antibody complement fixation titre was < 2 (normal range < 2) and antibody immunodiffusion CP D

Mycosis Fungoides with Focal CD 30 Transformation in an Adolescent

Abstract:  Mycosis fungoides is rare in children and adolescents. Large cell transformation in mycosis fungoides is typically seen in adult patients with advanced disease. We describe a 16‐year‐old girl with patch/plaque stage mycosis fungoides who developed a nodule within one of the plaques, which on histopathology showed large cell transformation, with positive labeling with the CD30 immunostain. To the best of our knowledge, this is the second reported case of mycosis fungoides with CD30+ large cell transformation in a child.

‘Spreading bumps’ on hands of a Native American

A 62-year-old Native American man was referred to our department in November 2006 for evaluation of spreading bumps on both arms. The patient first noticed the bumps on the backs of the hands, and the lesions had slowly spread up the forearms. They were not tender and did not itch, bleed or otherwise trouble the patient. His medical history was significant for type II diabetes mellitus. Notably, thyroid function was normal. The patient denied any recent exposure to or initiation of new medications, and his family and personal history were noncontributory. Clinical examination revealed multiple, diffuse, skincoloured to ivory, dome-shaped, firm papules, 2–5 mm in size, on the dorsa of both hands and on the wrists and extensor forearms extending to the antecubital fossae (Fig. 1). The papules were not indurated and showed no evidence of secondary changes. Urine and serum protein electrophoresis tests were negative for a monoclonal gammopathy. Histopathological findings

Hyperkeratosis and Discoloration of the Toenails in an 8-Year-Old

A n 8-year-old boy with no pertinent past medical history presented to the dermatology clinic with a complaint of an abnormal appearance and occasional pain in both great toenails. His mother reported that the nail changes had been present since the patient was born. The child had undergone surgical removal of both toenails 2 years prior, however, the nails grew back with a similar appearance. The patient denied any recent trauma to his toenails. No other family members were known to have a similar condition. On physical examination, the patient had yellowish-brown discoloration, hyperkeratosis, transverse ridging, and lateral deviation of his bilateral great toenails (Figure). The remainder of his nails (fingers and toes) were normal in appearance. There were no abnormalities of the scalp, hair, or teeth noted. Based upon his clinical presentation, this patient was diagnosed with congenital malalignment of the great toenails (CMGT). CMGT is characterized by the angular deviation of the longitudinal axis of the nail plate with respect to the corresponding axis of the distal phalanx. Almost all cases occur bilaterally, affect only the great toenails, and feature a lateral malalignment of the nail plate. Although subject to underreporting and misdiagnosis, the prevalence of CMGT has been estimated at 1%2% of the pediatric population. There does not seem to be a sex or racial predilection. In addition to its congenital onset and the bilateral foot involvement, other defining features of the condition include hyperkeratosis, onycholysis, brachyonychia, and discoloration of the nail plate. There may also be Beau lines, which are believed to be the result of repeated microtrauma to the nail matrix. The involved nail plates often appear dark yellow, however, a brownishblack color may also be seen as the result of either fungal infection or hemorrhage following microtrauma. In mild cases, the condition may actually go unnoticed until puberty, when greater involvement in sports and the use of tight shoes results in greater injury to the nail and subsequent discoloration. Diagnosis is typically made based on clinical appearance and a known persistence of the condition since birth. As part of an initial workup, bacterial and fungal cultures may also be obtained. Because of the similarities in appearance, the condition is often misdiagnosed as onychomycosis, which is a wellknown complication of the condition. Other common complications include onychocryptosis, onychogryphosis, and paronychia. Treatment of the condition varies based on severity and age. In pediatric cases involving severe malalignment, surgical realignment of the nail matrix can be performed. Elongation of the extensor tendon of the phalanx may also be considered during surgery. If completed before the age of 2 years, there is a high success rate to this approach. For less severe pediatric cases, a “watch and wait” approach is favored. It has been estimated that almost one-half of all children with the condition will experience spontaneous correction of the deviation by age 10 years. Management of patients who do not undergo surgery should involve treatment and education on the prevention of complications. ■

Cutaneous Clues: A Genetic Disorder Diagnosed by Dermatologic Findings

Figure 2. Two light-brown macules on the glans penis. A 2-year-old boy with a diagnosis of autism and developmental delay was brought to the clinic by his mother for evaluation of a mass on his back that had been growing for 5 months and did not appear to be painful. The patient had a history of speech delay, hypotonia, and macrocephaly. Evaluation in dysmorphology clinic included chromosomal microarray and fragile X testing. Results were normal, and no specific dysmorphologic condition was identified. On examination, the patient did not make eye contact with the examiner and demonstrated a limited vocabulary with pragmatic speech impairment. A large head circumference was noted. Cutaneous examination was significant for a soft, mobile, 3 5 cm subcutaneous nodule over the right scapula with no overlying skin changes (Figure 1). Two light-brown macules were noted on the glans penis (Figure 2). Bannayan-Riley-Ruvalcaba syndrome (BRRS) is a rare congenital disorder characterized by macrocephaly, lipomas, pigmented macules of the glans penis, and cognitive delay. Benign tumors, including lipomas and hemangiomas, are also frequently reported in BRRS. Hamartomatous polyps in the gastrointestinal tract occur in up to 40% of patients with BRRS and may present in childhood as chronic anemia, diarrhea, or intussusception of the small bowel. BRRS is one of several disorders, collectively known as PTEN hamartoma tumor syndromes, caused by a mutation