A Typical Red Neck

Home , Keratosis pilaris, Melanosis, Pigmentation disorder, Poikiloderma, Poikiloderma of Civatte, Riehl melanosis

CLINICAL

A typical red neck

Diego Fernández-Nieto, telangiectasia, hyperpigmentation and/ pigmentation predominates, with Darío de Perosanz-Lobo, or hypopigmentation. It typically appears minimal or absent telangiectasia and Juan Jiménez-Cauhé, on the sides of the neck and the anterior smaller follicular papules.2 It also affects Daniel Ortega-Quijano, upper chest after years of repeated sun dark-skinned patients, and the face Sonia Bea-Ardebol exposure.1 It has an estimated prevalence is more commonly involved than in of 1.4%, occurring in approximately the poikiloderma of Civatte. fifth decade of life, and is more common EFFC has been suggested to be a CASE in women.2 However, prevalence is likely subtype of keratosis pilaris that usually A woman, aged 46 years, with no to be higher in countries with populations affects young males. It presents as notable medical history presented with of light skin phototypes and high sun hyperpigmentation, follicular plugging a one-year history of skin lesions on exposure.2 and erythema predominantly affecting the upper chest and neck. Occasional Although the exact causes of the lateral aspects of cheeks and rarely itching and burning sensation were poikiloderma of Civatte are unknown, the neck.1 noted, with worsening after sun exposure. solar radiation is recognised as the primary Other conditions that should be The patient, who had Fitzpatrick skin cause. Hormonal factors, fair skin types included in the differential diagnosis type III, had a history of chronic sun and photosensitising components present are chronic graft versus host disease, exposure and mild solar burns, with in some cosmetics are also thought to friction melanosis, melasma, localised only occasional sunscreen protection. play a role in the aetiology.3 Poikiloderma scleroderma, radiation-induced skin She was not taking any prescription of Civatte is strongly associated with changes and poikiloderma vasculare medication and did not use fragrances accumulated photodamage, so it is atrophicans (an unusual manifestation of or other topical products. Physical rarely seen in dark-skinned patients cutaneous T-cell lymphoma).4 examination revealed erythematous of Fitzpatrick skin type IV or higher. Rothmund–Thomson syndrome is a reticular patches with superficial atrophy Poikiloderma of Civatte is only a cosmetic genodermatosis characterised by facial and occasional hyperpigmentation concern, with a slowly progressive and poikiloderma in childhood, short stature, located in sun-exposed areas of the irreversible course. Poikiloderma of premature ageing and predisposition neck (Figures 1A, 1B). Civatte is classified into three clinical to certain cancers. Bloom syndrome types: erythemato-telangiectatic, is a chromosomal breakage syndrome pigmented and mixed, depending on characterised by sun-sensitive facial QUESTION 1 the predominating clinical feature.2 telangiectatic erythema, susceptibility to What is the diagnosis? infections and cancer. ANSWER 2 Phototoxic and photoallergic reactions QUESTION 2 The most common conditions to to medication should also be considered, What is the differential diagnosis? consider in the differential diagnosis and a thorough anamnesis including are pigmented contact dermatitis present medications and over-the- QUESTION 3 (also known as Riehl’s melanosis) and counter products is recommended. What are the treatment options for erythromelanosis follicularis faciei et Photosensitive connective tissue this disease? colli (EFFC). diseases such as dermatomyositis and Pigmented contact dermatitis occurs lupus erythematosus usually present ANSWER 1 as a result of phototoxic or photoallergic as erythematous macules or plaques This common condition is reactions. It has been associated predominantly affecting sun-exposed poikiloderma of Civatte and presents with many cosmetic compounds, areas, including the neck. Signs and as a combination of atrophy, typically bergamot oil. Spotted brown symptoms such as fever, fatigue, weight

loss, myalgias, arthritis and oral ulcers Sonia Bea-Ardebol Dermatology Department, Hospital Universitario Ramón y Cajal, Madrid, Spain CASE CONTINUED should be evaluated. Competing interests and funding: None. Furthermore, atypical forms of The patient was reassured of the benign Provenance and peer review: Not commissioned, poikiloderma of Civatte should nature of her condition. No atypical skin externally peer reviewed. be biopsied and/or referred to a lesions were present, and no further dermatologist. intervention was required. References 1. Griffiths CEM, Barker J, Chalmers R, Bleiker T, Chalmers R, Creamer D. Rook’s Textbook of ANSWER 3 Dermatology. 9th edn. New York, USA: John Wiley Sun protection is crucial to stop & Sons, 2016. 2. Katoulis AC, Stavrianeas NG, Georgala S, progression. High sun protection factor Key points et al. Poikiloderma of Civatte: A clinical and sunscreen is recommended, as well as • Poikiloderma of Civatte is a benign epidemiological study. J Eur Acad Dermatol Venereol 2005;19(4):444–48. doi: 10.1111/j.1468- physical barriers such as scarves, collared condition associated with chronic 3083.2005.01213.x. shirts and hats. Skin cancers should be sun exposure; it typically affects 3. Nofal A, Salah E. Acquired poikiloderma: Proposed monitored. Avoidance of perfumes is sun-exposed areas of the neck. classification and diagnostic approach. J Am Acad Dermatol 2013;69(3):e129–40. doi: 10.1016/j. recommended, and patch testing can • Sun protection and monitoring of jaad.2012.06.015. be useful if induction by allergen is skin cancers is recommended. 4. Lautenschlager S, Itin PH. Reticulate, patchy suspected.5 and mottled pigmentation of the neck. Acquired forms. Dermatology 1998;197(3):291–96. Treatment options include a variety Authors doi: 10.1159/000018016. of energy-based therapies. The most Diego Fernández-Nieto MD, Dermatologist, 5. Katoulis AC, Stavrianeas NG, Katsarou A, et al. common options are intense pulsed light, Dermatology Department, Hospital Universitario Evaluation of the role of contact sensitization Ramón y Cajal, Madrid, Spain. and photosensitivity in the pathogenesis pulsed dye laser and fractionated ablative [email protected] of poikiloderma of Civatte. Br J Dermatol and non-ablative lasers. Choice is based Darío de Perosanz-Lobo MD, Dermatologist, 2002;147(3):493–97. doi: 10.1046/j.1365- 2133.2002.04993.x. on the predominance of telangiectasias, Dermatology Department, Hospital Universitario Ramón y Cajal, Madrid, Spain 6. Goldman MP, Weiss RA. Treatment of atrophy or pigmentation disorder. Juan Jiménez-Cauhé MD, Dermatologist, poikiloderma of Civatte on the neck with an Intense pulsed light can improve both Dermatology Department, Hospital Universitario intense pulsed light source. Plast Reconstr Surg Ramón y Cajal, Madrid, Spain 2001;107(6):1376–381. doi: 10.1097/00006534- dyspigmentation and telangiectasias and 200105000-00009. Daniel Ortega-Quijano MD, Dermatologist, reports a 50–75% improvement with a Dermatology Department, Hospital Universitario low side-effect profile.6 Ramón y Cajal, Madrid, Spain correspondence [email protected]

A B

Figure 1. Clinical presentation of the patient a. Erythematous reticular patches with telangiectasias and prominent hair follicles located at the upper back; b. Same clinical findings in the upper chest and neck; sparing of the submental area is present