[Downloaded free from http://www.e-ijd.org on Tuesday, April 08, 2014, IP: 111.93.251.154] || Click here to download free Android application for this journal CME Article Reticulate Dermatoses Keshavmurthy A Adya, Arun C Inamadar, Aparna Palit From the Department of Dermatology, Venereology and Leprosy, SBMP Medical College, Hospital and Research Center, BLDE University, Bijapur, Karnataka, India Abstract The term “reticulate” is used for clinical description of skin lesions that are configured in a net-like pattern. Many primary and secondary dermatoses present in such patterns involving specific body sites. Certain cutaneous manifestations of systemic diseases or genodermatoses also present in such manner. This review classifies and describes such conditions with reticulate lesions and briefly, their associated features. Key Words: Mottling, net-like, reticulate, retiform What was known? 3. Poikilodermatous Reticulate configuration of lesions is seen in many primary dermatoses and a. Inherited also as cutaneous reaction patterns consequent to internal pathology. • Rothmund–Thomson syndrome • Dyskeratosis congenita Reticulate Dermatoses • Xeroderma pigmentosum • Cockayne syndrome The term “reticulate” is commonly used for clinical • Fanconi anemia description of “net-like”, “sieve-like,” or “chicken wire” • Mendes da Costa syndrome configuration of the skin lesions. Various congenital • Kindler syndrome and acquired dermatoses present with this pattern of • Degos–Touraine syndrome skin lesions. Many systemic diseases also present with • Hereditary sclerosing poikiloderma of Weary such cutaneous manifestations providing useful clues to • Hereditary acrokeratotic poikiloderma of Weary diagnosis. • Werner’s syndrome (adult progeria) Classification • Chanarin–Dorfman syndrome • Diffuse and macular atrophic dermatosis 1. Vascular b. Acquired a. Cutis marmorata • Poikiloderma of Civatte b. Cutis marmorata telangiectatica congenita (CMTC) • Injury by cold, heat, or ionizing radiation c. Livedo reticularis (LR) • Chronic graft-versus-host disease (cGVHD) d. Livedo racemosa c. Others e. Livedoid vasculopathy • Dermatomyositis f. Reticulate purpura • Lupus erythematosus 2. Pigmentary • Parapsoriasis a. Dowling–Degos disease (DDD) • Mycosis fungoides b. Galli-Galli disease • Poikiloderma-like cutaneous amyloidosis c. Dermatopathia pigmentosa reticularis 4. Infectious d. Dyschromatosis universalis hereditaria (DUH) a. Confluent and reticulate papillomatosis (CRP) e. Reticulate acropigmentation b. Erythema infectiosum • Reticulate acropigmentation of Dohi c. Erythema marginatum (EM) • Reticulate acropigmentation of Kitamura d. Congenital rubella syndrome f. Syndromes associated with reticulate pigmentation 5. Metabolic • Naegeli–Franceschetti–Jadassohn syndrome a. Macular amyloidosis • Mendes da Costa–van der Valk syndrome b. Amyloidosis cutis dyschromica • Hoyeraal–Hreidarrson syndrome • Partington syndrome g. Atopic dirty neck Access this article online Quick Response Code: h. Epidermolysis bullosa with mottled pigmentation Website: www.e-ijd.org i. Systemic sclerosis Address for correspondence: Dr. Arun C Inamadar, Department of Dermatology, Venereology and Leprosy, SBMP Medical College, Hospital DOI: 10.4103/0019-5154.123480 and Research Center, BLDE University, Bijapur, Karnataka - 586 103, India. E-mail: [email protected] 3 Indian Journal of Dermatology 2014; 59(1) [Downloaded free from http://www.e-ijd.org on Tuesday, April 08, 2014, IP: 111.93.251.154] || Click here to download free Android application for this journal Adya, et al.: Reticulate dermatoses c. Reticular erythematous mucinosis Distribution may be localized, segmental, or widespread.[5] d. Hunter’s syndrome The mottling gets accentuated by cold temperature, crying, 6. Others or vigorous activity. More than half of the cases are a. Erythema ab igne associated with other anomalies. CMTC forms a part of b. Prurigo pigmentosa various syndromes also[6] [Table 2]. c. Mucosal lichen planus LR and livedo racemosa d. Leukoplakia e. Neonatal lupus erythematosus (LE) LR is perhaps the prototype among the reticulate f. Hereditary angioedema dermatoses. It is characterized by bluish-red discoloration g. Extensive congenital erosions and vesicles healing of the skin in a typical net-like pattern due to decreased with reticulate scarring blood flow through and decreased oxygen tension in h. Atrophodermia vermiculata the cutaneous venous plexuses.[12] Lower temperature i. Nekam’s disease accentuate the mottling. The anatomical organization of the cutaneous vasculature is the basis of this particular Vascular Causes of Reticulate Lesions pattern of mottling. Lower extremities are predominantly involved. The net-like pattern is usually complete Cutis marmorata in LR as opposed to livedo racemosa which has a Cutis marmorata is a physiological response to cold exposure seen in neonates that readily disappears on Table 1: Syndromes associated with persistent cutis re-warming. It is characterized by marbling of the skin marmorata in the form of a reticulate bluish vascular pattern due to Syndromes Other features dilatation of capillaries and small venules.[1,2] It involves Cornelia de Lange Facies: Expressionless face, the limbs and trunk in a symmetrical manner [Figure 1]. syndrome arched eyebrows, long eyelashes, This physiological mottling disappears by 6 months of long philtrum, thin upper lip, age.[3] Persistent cutis marmorata, or the one which recurs down-turned angles of mouth until early childhood, is associated with cretinism and Low-set ears and posterior hair line forms a part of various inherited syndromes [Table 1]. Cardiac septal defects Feeding In some infants, due to transient constriction of the difficulties deep vasculature, a white negative pattern of cutis Cognitive impairment marmorata (cutis marmorata alba) may occur.[2] Reduced life expectancy Adams-Oliver Cranial defects, limb defects, CMTC (congenital phlebectasia, van Lohuizen syndrome scalp defects, cardiac, and central syndrome) nervous system anomalies CMTC is a distinct condition characterized by reticular Edward syndrome Facies: Prominent occiput, small vascular mottling, predominantly involving the trunk and (trisomy 18) chin, low-set ears Clenched hands, rocker-bottom feet limbs, that resembles the physiological cutis marmorata. Single palmar crease Cardiac and However, it is distinguished from the latter by the persistent renal abnormalities Cutaneous nature (even after re-warming), deep livid-purple color, features: Redundant neck folds, and association with cutaneous atrophy or ulceration.[4] hypertrichosis of the forehead and back, and capillary hemangiomas Down syndrome Facies: Microcephaly, flat face, (trisomy 21) short nose, misshapen ears, slanting palpabral fissures, thick eyelids Lax joints, curved little fingers Cataracts, hypothyroidism, endocardial cushion defect Mental retardation Homocystinuria Marfanoid habitus, seizures, developmental delay, ectopia lentis, platyspondylia, kyphoscoliosis, pectus carinatum, arachnodactyly, rocker-bottom feet, Charlie Chaplin-like gait Divry-Van Bogaert Seizures, visual field defects, syndrome mental disturbances, and Figure 1: Cutis marmorata progressive dementia Indian Journal of Dermatology 2014; 59(1) 4 [Downloaded free from http://www.e-ijd.org on Tuesday, April 08, 2014, IP: 111.93.251.154] || Click here to download free Android application for this journal Adya, et al.: Reticulate dermatoses branching pattern (broken livedo). Other differences symmetrical reticulate hyperpigmentation involving the between LR and livedo racemosa[12-14] are listed in neck, axillae [Figure 2], groins, and other flexures like Table 3. the inframammary folds in females.[36,37] It occurs due to LR may be physiological (cutis marmorata), idiopathic (usually benign), or may be associated with other Table 2: Associations of CMTC [9,15-17] conditions [Table 4]. If the mottling is associated Phenotypic[4,7,8] Limb hypoplasia (less commonly with necrosis or purpura, the terms necrotizing livedo or hyperplasia), aplasia cutis, cleft palate, retiform purpura (see below), respectively, are used. Livedo macrocephaly racemosa and livedo with purpura or necrosis are almost Vascular[9] Nevus flammeus, varicosities, infantile always associated with systemic diseases.[12] hemangioma Ophthalmologic[9,10] Glaucoma, retinal pigmentation, retinal Livedoid vasculopathy (livedoid vasculitis, detachment segmental hyalinizing vasculitis, LR with summer/ Neurologic[9] Seizures, hydrocephalus, psychomotor winter ulceration) retardation [6] One of the synonyms for this condition is “painful Syndromes Adams-Oliver syndrome, phacomatosis pigmentovascularis (type V), purpuric ulcers with reticular pattern of the lower macrocephaly-CMTC syndrome extremities (PURPLE),” which precisely describes Less common Generalized congenital fibromatosis, the clinical features of this entity. Livedoid vasculitis, associations[6,7,8,11] premature ovarian failure, Chiari [18] usually a disease of middle aged women, presents in I malformation, rectal and genital two stages; initial manifestations are painful purpuric anomalies, hypothyroidism, scoliosis lesions involving predominantly the gaiter region of CMTC: Cutis marmorata telangiectatica congenita the lower extremities (usually bilateral) that evolve into painful shallow ulcers. Later the ulcers heal with stellate ivory-white scars, which have a reticulate pattern (atrophie Table 3: Differences between LR and livedo racemosa blanche). Livedo reticularis